Materiały źródłowe

• #1 Desmoid tumors: Epidemiology, molecular pathogenesis, clinical presentation, and diagnosis – UpToDate

  https://www.uptodate.com/contents/desmoid-tumors-epidemiology-molecular-pathogenesis-clinical-presentation-and-diagnosis/print

  Desmoid tumors are rare and most arise sporadically. They account for approximately 0.03 percent of all neoplasms and fewer than 3 percent of all soft tissue tumors. The estimated incidence in the general population is two to four per million people per year. […] Most patients diagnosed with a desmoid tumor are between the ages of 15 and 60. They are slightly more common in females than in males. There is no significant racial or ethnic predilection.

• #2 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7764110/

  Desmoid tumors (DTs) are common in patients with familial adenomatous polyposis (FAP). An active surveillance approach has been recently proposed as a valuable alternative to immediate treatment in some patients. However, no clear indication exists on which patients are suitable for active surveillance, how to establish the cut-off for an active treatment, and which imaging technique or predictive factors should be used during the surveillance period. […] In the general population, DTs are rare, accounting for 0.03% of all neoplasms and 3% of soft tissue tumors. They have an estimated incidence of 25 cases per 1 million of inhabitants in European countries, with a median age at diagnosis of 35 years. […] It is estimated that patients with FAP have an approximate 800-1000-fold increased risk of developing DTs, compared with the general population. DTs occur in approximately 7.5-15% of patients with FAP, although their prevalence could reach up to 21-35% given their pleomorphic presentation, which may lead to underestimated predictions.

• #3 Desmoid Tumor: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1060887-overview

  Desmoid tumors are reported to account for 0.03% of all neoplasms. […] When present in patients with familial polyposis of the colon, the prevalence of desmoid tumors is as high as 13%. […] Desmoid tumors most commonly occur in women after childbirth. Desmoid tumors are twice as common in females than in males; however, 60 patients were described, and the female-to-male ratio was 1.2:1. In children, the sex incidence is equal. […] Although desmoid tumors are more common in persons aged 10-40 years than in others, they do occur in young children and older adults. Sixty patients were described by Lee et al in 2006, with an average age at diagnosis of 41.3 years.

• #4 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7764110/

  Desmoid tumors (DTs) are common in patients with familial adenomatous polyposis (FAP). An active surveillance approach has been recently proposed as a valuable alternative to immediate treatment in some patients. However, no clear indication exists on which patients are suitable for active surveillance, how to establish the cut-off for an active treatment, and which imaging technique or predictive factors should be used during the surveillance period. […] In the general population, DTs are rare, accounting for 0.03% of all neoplasms and 3% of soft tissue tumors. They have an estimated incidence of 25 cases per 1 million of inhabitants in European countries, with a median age at diagnosis of 35 years. […] It is estimated that patients with FAP have an approximate 800-1000-fold increased risk of developing DTs, compared with the general population. DTs occur in approximately 7.5-15% of patients with FAP, although their prevalence could reach up to 21-35% given their pleomorphic presentation, which may lead to underestimated predictions.

• #5

  https://link.springer.com/article/10.1007/s12325-023-02592-0

  Desmoid tumors (DT) are rare, locally aggressive, fibroblastic soft-tissue tumors that are characterized by infiltrative growth and can affect organs and adjacent structures, resulting in substantial clinical burden impacting patients health-related quality of life. […] The low incidence of DT (estimated 35 cases per million person-years) limits disease awareness. […] There is a high unmet need for treatments that specifically target DT and improve quality of life. […] Five European sources providing estimates on the incidence of DT were identified in the literature. […] The most recently published study, Anneberg et al. estimated 2.2 cases per million person-years in 2011 and 4.3 cases per million person-years in 2017 in Denmark, using the Danish Sarcoma Database and excluding patients aged 18 years and younger.

• #6 Desmoid tumours (extra-abdominal), a surgeon’s nightmare | Bone & Joint

  https://boneandjoint.org.uk/Article/10.1302/0301-620X.105B7.BJJ-2023-0117

  Desmoid tumours can occur in any anatomical site, but commonly arise in the limbs. […] They affect between five and six people per million worldwide per year, accounting for 3% of all soft-tissue tumours. […] The peak incidence is in patients aged between 30 and 40 years, with a female predominance. […] The most common site is the abdominal wall, followed by the chest wall, paraspinal trunk, mesentery, breast, limbs and girdles. […] The most recent guidelines published by the Desmoid Tumour Working Group in 2020 advise active surveillance, defined as the continuous monitoring of patients with an initial MRI or CT scan, performed within one or two months, and then at three- to six-monthly intervals. […] Surveillance should begin after a histological diagnosis and radiological evaluation and should be discontinued in favour of active treatment only in patients with persistent progression and/or increasing symptoms.

• #7 Desmoid tumor epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Desmoid_tumor_epidemiology_and_demographics

  Desmoid tumor accounts for 0.03% of all tumors and less than 3% of all the soft tissue tumors. It affects 1 to 2 per 500,000 people worldwide. Incidence of desmoid tumor is approximately two to four per million population per year in general population. Familial desmoids are present in 10% to 25% of FAP patients. Gender ratio is 2:1 for females to males and commonly affects individuals between 20 to 60 years of age. […] The incidence of desmoid tumor is approximately 0/2-0/4 per 100,000 population per year in general population. […] Desmoid tumor accounts for approximately 0.03 percent of all neoplasms. It accounts for less than 3% of all the soft tissue tumors. Affects 1 to 2 per 500,000 people worldwide. In the United States, 900 to 1,500 new cases are diagnosed per year. Sporadic desmoid tumors are more common than familial desmoids associated with familial adenomatous polyposis. Familial desmoid tumor (germline APC mutation) occurs in 10% to 25% of FAP patients. People with germline APC mutation have 850 times greater risk of developing desmoids than the general population. Sporadic desmoids (APC or the beta-catenin gene mutations) are relatively rare, affect 2 to 5 individuals per million per year in the general population.

• #8 The Facts About Desmoid Tumors –

  https://www.desmoidtumors.com/hcp/facts-about-desmoid-tumors/

  There are approximately 1000 to 1650 annual cases in the United States. […] Most patients are diagnosed between 20-44 years of age. […] Female-to-male ratio is ~2-3:1. […] Recent pregnancy, injury, or surgery may increase risk. […] Patients with familial adenomatous polyposis (FAP) have ~850-fold higher risk of developing desmoid tumors than the general population. […] FAP is associated with desmoid tumors. […] Desmoid tumors have been reported to occur in 10% to 12% of patients with FAP. […] Patients with FAP are at much higher risk of developing desmoid tumors than the general population. […] FAP-associated desmoid tumors are predominantly intra-abdominal (80%), usually within the mesentery. […] In addition, prior colectomy is associated with subsequent development of desmoid tumors, which represent a significant cause of morbidity in patients with FAP.

• #9 Desmoid tumors: Epidemiology, molecular pathogenesis, clinical presentation, and diagnosis – UpToDate

  https://www.uptodate.com/contents/desmoid-tumors-epidemiology-molecular-pathogenesis-clinical-presentation-and-diagnosis/print

  Desmoid tumors are rare and most arise sporadically. They account for approximately 0.03 percent of all neoplasms and fewer than 3 percent of all soft tissue tumors. The estimated incidence in the general population is two to four per million people per year. […] Most patients diagnosed with a desmoid tumor are between the ages of 15 and 60. They are slightly more common in females than in males. There is no significant racial or ethnic predilection.

• #10 Economic evaluation of percutaneous cryoablation vs conventional surgery in extra-abdominal desmoid tumours in the Spanish healthcare system | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-023-01580-w

  Desmoid tumours (DTs) or deep fibromatosis are benign soft-tissue tumours, sometimes locally aggressive, requiring intervention on some cases. […] DTs represent less than 3% of soft tissue neoplasms and less than 0.03% of all tumours, with an annual incidence between 2 and 4 cases/million patients/year. […] The management of DTs has evolved in recent years, but there is still no clear consensus about the best treatment option for DTs. […] In the management and treatment guidelines recently published by the Desmoid Tumour Working Group (DTWG), active tumour surveillance is recommended as the first option, although conventional surgery continues to be strategy most used in clinical practice, specifically in the case of extra-abdominal DTs. […] The intervention chosen to treat DTs depends on the location of the tumour.

• #11 Desmoid tumor epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Desmoid_tumor_epidemiology_and_demographics

  Female are more commonly affected by desmoid tumor than male. The female of fertile age to male ratio is approximately 2:1. Incidence is the same in both genders in children. […] Desmoid tumor commonly affects individuals between 20 to 60 years of age. Desmoid tumor commonly affects 30 to 40 years.

• #12

  https://link.springer.com/article/10.1007/s12325-023-02592-0

  The epidemiological studies reveal that most DT cases appear in the age range of 20-44 years, with a female predominance, and female cases 2.2-3.9 times the number of male cases. […] Few epidemiological studies providing incidence estimates of DT were identified in the literature; all these studies were conducted in European countries, and no study provided prevalence estimates. […] Because all the epidemiological data are from European populations, a US population-based study will be necessary to compare incidence estimates between Europe and the US.

• #13 Desmoid tumor | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/desmoid-tumour?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjgh&lang=us

  Desmoid tumors are rare tumors, thought to account for only ~0.03% of all neoplasms. Desmoid tumors are found in all age groups but are most frequently encountered between 20 and 40 years of age. They are seen more in women (2:1). They are rare lesions with an estimated incidence of 3.7 new cases per million population per year. […] Some cases have been associated with pregnancy and estrogen therapy. […] In the case of mesenteric desmoid they are seen either sporadically or in association with familial polyposis coli syndrome (FAP): 9-18% of FAP cases may have a desmoid tumor.

• #14 Desmoid tumor epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Desmoid_tumor_epidemiology_and_demographics

  Female are more commonly affected by desmoid tumor than male. The female of fertile age to male ratio is approximately 2:1. Incidence is the same in both genders in children. […] Desmoid tumor commonly affects individuals between 20 to 60 years of age. Desmoid tumor commonly affects 30 to 40 years.

• #15 Desmoid Tumor: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1060887-overview

  Desmoid tumors are reported to account for 0.03% of all neoplasms. […] When present in patients with familial polyposis of the colon, the prevalence of desmoid tumors is as high as 13%. […] Desmoid tumors most commonly occur in women after childbirth. Desmoid tumors are twice as common in females than in males; however, 60 patients were described, and the female-to-male ratio was 1.2:1. In children, the sex incidence is equal. […] Although desmoid tumors are more common in persons aged 10-40 years than in others, they do occur in young children and older adults. Sixty patients were described by Lee et al in 2006, with an average age at diagnosis of 41.3 years.

• #16 Desmoid tumor epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Desmoid_tumor_epidemiology_and_demographics

  Female are more commonly affected by desmoid tumor than male. The female of fertile age to male ratio is approximately 2:1. Incidence is the same in both genders in children. […] Desmoid tumor commonly affects individuals between 20 to 60 years of age. Desmoid tumor commonly affects 30 to 40 years.

• #17 Desmoid tumors: Epidemiology, molecular pathogenesis, clinical presentation, and diagnosis – UpToDate

  https://www.uptodate.com/contents/desmoid-tumors-epidemiology-molecular-pathogenesis-clinical-presentation-and-diagnosis/print

  Desmoid tumors are rare and most arise sporadically. They account for approximately 0.03 percent of all neoplasms and fewer than 3 percent of all soft tissue tumors. The estimated incidence in the general population is two to four per million people per year. […] Most patients diagnosed with a desmoid tumor are between the ages of 15 and 60. They are slightly more common in females than in males. There is no significant racial or ethnic predilection.

• #18

  https://link.springer.com/article/10.1007/s12325-023-02592-0

  The epidemiological studies reveal that most DT cases appear in the age range of 20-44 years, with a female predominance, and female cases 2.2-3.9 times the number of male cases. […] Few epidemiological studies providing incidence estimates of DT were identified in the literature; all these studies were conducted in European countries, and no study provided prevalence estimates. […] Because all the epidemiological data are from European populations, a US population-based study will be necessary to compare incidence estimates between Europe and the US.

• #19 Molecular pathogenesis of desmoid tumor and the role of γ-secretase inhibition | npj Precision Oncology

  https://www.nature.com/articles/s41698-022-00308-1

  Desmoid tumor (DT) is a rare, soft tissue neoplasm associated with an unpredictable clinical course. […] DT occurs rarely (accounting for fewer than 3% of soft tissue tumors), with a reported incidence of 35 cases per million population per year. […] Onset of DT has been reported from infancy through adulthood, although it occurs most commonly between the ages of 15 and 60 years (with peak incidence between 30-40 years of age) and 2-3 times more frequently in women than men. […] Approximately 85-90% of DT cases are sporadic, associated with mutations in the CTNNB1 gene that encodes β-catenin, while 5-10% of cases arise in the context of familial adenomatous polyposis (FAP) in which there is a germline mutation in the adenomatous polyposis coli gene (APC). […] The natural history of DT is variable and ranges from an asymptomatic, indolent course to aggressive infiltration of neurovascular structures and vital organs resulting in pain, disfigurement, organ dysfunction, and rarely death.

• #20 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://www.mdpi.com/2075-4426/13/12/1653

  Desmoid tumors (DTs), otherwise known as desmoid fibromatosis or aggressive fibromatosis, are a locally aggressive, non-metastasizing mesenchymal tumor with an invasive growth pattern, no metastatic potential, and a high propensity for locoregional recurrence after surgery. Desmoid tumors account for less than 3% of soft tissue tumors, frequently affect women of childbearing age, and are linked to pregnancy in a subset of cases. Most cases are sporadic and, in these cases, mutations in the beta-catenin gene (CTNNB1) are usually found. About 5–10% of cases occur in patients with familial adenomatous polyposis (FAP), due to an APC (adenomatous polyposis coli) germ line mutation, with the two mutations being mutually exclusive. Patients suffering from these heredo-familial syndromes usually discover that they have DTs during routine examinations performed for screening purposes. DTs can occur in both somatic and visceral sites. Extra-abdominal, abdominal wall, and intra-abdominal are common locations, and different prognoses have been associated with different locations.

• #21 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://www.mdpi.com/2075-4426/13/12/1653

  Desmoid tumors (DTs), otherwise known as desmoid fibromatosis or aggressive fibromatosis, are a locally aggressive, non-metastasizing mesenchymal tumor with an invasive growth pattern, no metastatic potential, and a high propensity for locoregional recurrence after surgery. Desmoid tumors account for less than 3% of soft tissue tumors, frequently affect women of childbearing age, and are linked to pregnancy in a subset of cases. Most cases are sporadic and, in these cases, mutations in the beta-catenin gene (CTNNB1) are usually found. About 5–10% of cases occur in patients with familial adenomatous polyposis (FAP), due to an APC (adenomatous polyposis coli) germ line mutation, with the two mutations being mutually exclusive. Patients suffering from these heredo-familial syndromes usually discover that they have DTs during routine examinations performed for screening purposes. DTs can occur in both somatic and visceral sites. Extra-abdominal, abdominal wall, and intra-abdominal are common locations, and different prognoses have been associated with different locations.

• #22 Molecular pathogenesis of desmoid tumor and the role of γ-secretase inhibition | npj Precision Oncology

  https://www.nature.com/articles/s41698-022-00308-1

  Desmoid tumor (DT) is a rare, soft tissue neoplasm associated with an unpredictable clinical course. […] DT occurs rarely (accounting for fewer than 3% of soft tissue tumors), with a reported incidence of 35 cases per million population per year. […] Onset of DT has been reported from infancy through adulthood, although it occurs most commonly between the ages of 15 and 60 years (with peak incidence between 30-40 years of age) and 2-3 times more frequently in women than men. […] Approximately 85-90% of DT cases are sporadic, associated with mutations in the CTNNB1 gene that encodes β-catenin, while 5-10% of cases arise in the context of familial adenomatous polyposis (FAP) in which there is a germline mutation in the adenomatous polyposis coli gene (APC). […] The natural history of DT is variable and ranges from an asymptomatic, indolent course to aggressive infiltration of neurovascular structures and vital organs resulting in pain, disfigurement, organ dysfunction, and rarely death.

• #23 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7764110/

  Desmoid tumors (DTs) are common in patients with familial adenomatous polyposis (FAP). An active surveillance approach has been recently proposed as a valuable alternative to immediate treatment in some patients. However, no clear indication exists on which patients are suitable for active surveillance, how to establish the cut-off for an active treatment, and which imaging technique or predictive factors should be used during the surveillance period. […] In the general population, DTs are rare, accounting for 0.03% of all neoplasms and 3% of soft tissue tumors. They have an estimated incidence of 25 cases per 1 million of inhabitants in European countries, with a median age at diagnosis of 35 years. […] It is estimated that patients with FAP have an approximate 800-1000-fold increased risk of developing DTs, compared with the general population. DTs occur in approximately 7.5-15% of patients with FAP, although their prevalence could reach up to 21-35% given their pleomorphic presentation, which may lead to underestimated predictions.

• #24 Desmoid Tumors in Familial Adenomatous Polyposis | Anticancer Research

  https://ar.iiarjournals.org/content/37/7/3357

  The incidence of DTs in the general population is 2-4 new patients per million, corresponding to 0.03% of all newly diagnosed cases and 3% of all soft-tissue neoplasms. […] The incidence of DTs in FAP is 800- to 1,000-fold higher than in the general population: DTs occur in between 10% and 15% of patients with FAP. […] A family history of DTs arising in first-degree relatives is associated with a 25% risk of developing DTs. […] A peculiar risk factor for the development of DTs is surgical trauma: most DTs develop within 5 years after surgery, in particular 68-83% DTs after abdominal operations, mostly within 24 months. […] The syndrome is characterized by an increased risk of extra-intestinal neoplasias: gastric and duodenal polyps, thyroid and pancreatic cancer, adrenal cortical adenoma, hepatoblastomas, CNS medulloblastoma or glioblastoma (Turcot syndrome). […] DTs are a typical finding in FAP syndrome, where they affect 10-15% of patients. […] In particular, individuals with mutations of the APC gene beyond codon 1,444 are at a 12-fold higher risk of developing DTs.

• #25 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7764110/

  Desmoid tumors (DTs) are common in patients with familial adenomatous polyposis (FAP). An active surveillance approach has been recently proposed as a valuable alternative to immediate treatment in some patients. However, no clear indication exists on which patients are suitable for active surveillance, how to establish the cut-off for an active treatment, and which imaging technique or predictive factors should be used during the surveillance period. […] In the general population, DTs are rare, accounting for 0.03% of all neoplasms and 3% of soft tissue tumors. They have an estimated incidence of 25 cases per 1 million of inhabitants in European countries, with a median age at diagnosis of 35 years. […] It is estimated that patients with FAP have an approximate 800-1000-fold increased risk of developing DTs, compared with the general population. DTs occur in approximately 7.5-15% of patients with FAP, although their prevalence could reach up to 21-35% given their pleomorphic presentation, which may lead to underestimated predictions.

• #26 The Facts About Desmoid Tumors –

  https://www.desmoidtumors.com/hcp/facts-about-desmoid-tumors/

  There are approximately 1000 to 1650 annual cases in the United States. […] Most patients are diagnosed between 20-44 years of age. […] Female-to-male ratio is ~2-3:1. […] Recent pregnancy, injury, or surgery may increase risk. […] Patients with familial adenomatous polyposis (FAP) have ~850-fold higher risk of developing desmoid tumors than the general population. […] FAP is associated with desmoid tumors. […] Desmoid tumors have been reported to occur in 10% to 12% of patients with FAP. […] Patients with FAP are at much higher risk of developing desmoid tumors than the general population. […] FAP-associated desmoid tumors are predominantly intra-abdominal (80%), usually within the mesentery. […] In addition, prior colectomy is associated with subsequent development of desmoid tumors, which represent a significant cause of morbidity in patients with FAP.

• #27 Desmoid Tumors in Familial Adenomatous Polyposis | Anticancer Research

  https://ar.iiarjournals.org/content/37/7/3357

  The incidence of DTs in the general population is 2-4 new patients per million, corresponding to 0.03% of all newly diagnosed cases and 3% of all soft-tissue neoplasms. […] The incidence of DTs in FAP is 800- to 1,000-fold higher than in the general population: DTs occur in between 10% and 15% of patients with FAP. […] A family history of DTs arising in first-degree relatives is associated with a 25% risk of developing DTs. […] A peculiar risk factor for the development of DTs is surgical trauma: most DTs develop within 5 years after surgery, in particular 68-83% DTs after abdominal operations, mostly within 24 months. […] The syndrome is characterized by an increased risk of extra-intestinal neoplasias: gastric and duodenal polyps, thyroid and pancreatic cancer, adrenal cortical adenoma, hepatoblastomas, CNS medulloblastoma or glioblastoma (Turcot syndrome). […] DTs are a typical finding in FAP syndrome, where they affect 10-15% of patients. […] In particular, individuals with mutations of the APC gene beyond codon 1,444 are at a 12-fold higher risk of developing DTs.

• #28 Desmoid tumor | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/desmoid-tumour?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjgh&lang=us

  Desmoid tumors are rare tumors, thought to account for only ~0.03% of all neoplasms. Desmoid tumors are found in all age groups but are most frequently encountered between 20 and 40 years of age. They are seen more in women (2:1). They are rare lesions with an estimated incidence of 3.7 new cases per million population per year. […] Some cases have been associated with pregnancy and estrogen therapy. […] In the case of mesenteric desmoid they are seen either sporadically or in association with familial polyposis coli syndrome (FAP): 9-18% of FAP cases may have a desmoid tumor.

• #29 Desmoid Tumors and Pregnancy: What’s the Connection? | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/desmoid-tumors-and-pregnancy-whats-the-connection

  Desmoid tumors are very rare. Very few pregnant or postpartum individuals will develop desmoid tumors. […] According to the National Cancer Institute, desmoid tumors are more common in women between the ages of 15 to 60. […] Active surveillance is usually the preferred approach for managing desmoid tumors associated with pregnancy. This approach is also recommended for the majority of desmoid tumors that are not related to pregnancy. […] Some peoples tumors shrink on their own without treatment. Your doctor may recommend active surveillance if your tumor is stable (not growing) and isnt causing any symptoms.

• #30 The Facts About Desmoid Tumors –

  https://www.desmoidtumors.com/hcp/facts-about-desmoid-tumors/

  There are approximately 1000 to 1650 annual cases in the United States. […] Most patients are diagnosed between 20-44 years of age. […] Female-to-male ratio is ~2-3:1. […] Recent pregnancy, injury, or surgery may increase risk. […] Patients with familial adenomatous polyposis (FAP) have ~850-fold higher risk of developing desmoid tumors than the general population. […] FAP is associated with desmoid tumors. […] Desmoid tumors have been reported to occur in 10% to 12% of patients with FAP. […] Patients with FAP are at much higher risk of developing desmoid tumors than the general population. […] FAP-associated desmoid tumors are predominantly intra-abdominal (80%), usually within the mesentery. […] In addition, prior colectomy is associated with subsequent development of desmoid tumors, which represent a significant cause of morbidity in patients with FAP.

• #31 Desmoid Tumors in Familial Adenomatous Polyposis | Anticancer Research

  https://ar.iiarjournals.org/content/37/7/3357

  The incidence of DTs in the general population is 2-4 new patients per million, corresponding to 0.03% of all newly diagnosed cases and 3% of all soft-tissue neoplasms. […] The incidence of DTs in FAP is 800- to 1,000-fold higher than in the general population: DTs occur in between 10% and 15% of patients with FAP. […] A family history of DTs arising in first-degree relatives is associated with a 25% risk of developing DTs. […] A peculiar risk factor for the development of DTs is surgical trauma: most DTs develop within 5 years after surgery, in particular 68-83% DTs after abdominal operations, mostly within 24 months. […] The syndrome is characterized by an increased risk of extra-intestinal neoplasias: gastric and duodenal polyps, thyroid and pancreatic cancer, adrenal cortical adenoma, hepatoblastomas, CNS medulloblastoma or glioblastoma (Turcot syndrome). […] DTs are a typical finding in FAP syndrome, where they affect 10-15% of patients. […] In particular, individuals with mutations of the APC gene beyond codon 1,444 are at a 12-fold higher risk of developing DTs.

• #32 Desmoid Tumors in Familial Adenomatous Polyposis | Anticancer Research

  https://ar.iiarjournals.org/content/37/7/3357

  The incidence of DTs in the general population is 2-4 new patients per million, corresponding to 0.03% of all newly diagnosed cases and 3% of all soft-tissue neoplasms. […] The incidence of DTs in FAP is 800- to 1,000-fold higher than in the general population: DTs occur in between 10% and 15% of patients with FAP. […] A family history of DTs arising in first-degree relatives is associated with a 25% risk of developing DTs. […] A peculiar risk factor for the development of DTs is surgical trauma: most DTs develop within 5 years after surgery, in particular 68-83% DTs after abdominal operations, mostly within 24 months. […] The syndrome is characterized by an increased risk of extra-intestinal neoplasias: gastric and duodenal polyps, thyroid and pancreatic cancer, adrenal cortical adenoma, hepatoblastomas, CNS medulloblastoma or glioblastoma (Turcot syndrome). […] DTs are a typical finding in FAP syndrome, where they affect 10-15% of patients. […] In particular, individuals with mutations of the APC gene beyond codon 1,444 are at a 12-fold higher risk of developing DTs.

• #33 The Facts About Desmoid Tumors –

  https://www.desmoidtumors.com/hcp/facts-about-desmoid-tumors/

  There are approximately 1000 to 1650 annual cases in the United States. […] Most patients are diagnosed between 20-44 years of age. […] Female-to-male ratio is ~2-3:1. […] Recent pregnancy, injury, or surgery may increase risk. […] Patients with familial adenomatous polyposis (FAP) have ~850-fold higher risk of developing desmoid tumors than the general population. […] FAP is associated with desmoid tumors. […] Desmoid tumors have been reported to occur in 10% to 12% of patients with FAP. […] Patients with FAP are at much higher risk of developing desmoid tumors than the general population. […] FAP-associated desmoid tumors are predominantly intra-abdominal (80%), usually within the mesentery. […] In addition, prior colectomy is associated with subsequent development of desmoid tumors, which represent a significant cause of morbidity in patients with FAP.

• #34 Large Desmoid Tumor in the Setting of Prior Cosmetic Breast Augmentation – European Journal of Breast Health

  https://eurjbreasthealth.com/articles/large-desmoid-tumor-in-the-setting-of-prior-cosmetic-breast-augmentation/doi/ejbh.galenos.2022.2022-7-3

  Desmoid tumors of the breast are rare, comprising 0.2% of all breast tumors. […] The rate of local recurrence is high at 2465% in 10 years. […] Breast implants are a potential risk factor for desmoid tumors of the breast. […] Initial treatment could be surveillance depending on presentation. […] If there is enlargement of the tumor or complications related to local invasion, surgery (wide excision) with negative margins is the treatment of choice. […] The recurrence rate of breast fibromatosis is lower than that of other sites of fibromatosis, reported at 2127% compared to 3065%. […] The initial treatment recommended is surveillance. […] Following definitive resection, surveillance is essential in detecting early recurrence. […] The standardized incidence ratio (SIR) was calculated to examine if there was a connection between breast implants and breast desmoid tumors. The SIR was 482 to 823, correlating to a 482823 times higher risk of developing a breast desmoid tumor after breast implant placement compared to the general population.

• #35 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7764110/

  In the last few decades, the routine use of aggressive first-line treatments for DTs, such as surgery and RT, has been progressively challenged by many authors. Considering the unpredictable behavior of DTs and the high recurrence rates registered after surgery, close observation is now considered an acceptable approach, especially for asymptomatic patients; whereas treatment is recommended in symptomatic patients, in case of tumors adjacent to critical structures or for cosmetic concerns. […] Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #36 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10744644/

  In patients with desmoid tumors (DTs), active surveillance has been increasingly preferred over surgery, while treatment (including pharmacological therapy, radiotherapy, and/or surgery) is performed in cases with confirmed disease progression. […] Our findings support the role of active surveillance as initial management, as demonstrated by the fact that about half the patients did not experience any progression, while surgery can be reserved as a first-line approach for selected patients. […] Currently, a wait-and-see approach based on active surveillance is preferred, especially for asymptomatic patients, and treatment, including pharmacological therapy, radiotherapy, and/or surgery, should be postponed until the disease has demonstrated progression. […] Active surveillance is generally the first approach for most people with a new diagnosis of DT and consists of active monitoring or active observation by check-up visits with the oncologist every three to four months.

• #37 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://www.mdpi.com/2075-4426/13/12/1653

  Currently, a “wait-and-see” approach based on active surveillance is preferred, especially for asymptomatic patients, and treatment, including pharmacological therapy, radiotherapy, and/or surgery, should be postponed until the disease has demonstrated progression. Non-invasive treatments are generally preferred over surgery to avoid unnecessary complications associated with tissue destruction. Spontaneous disease regression has been reported to occur in approximately 30% of DT cases. […] Active surveillance is generally the first approach for most people with a new diagnosis of DT and consists of active monitoring or active observation by check-up visits with the oncologist every three to four months. At each visit, the decision to stop active surveillance and start specific therapy is based on clinical and radiological examinations (such as ultrasound or magnetic resonance imaging), and may be driven by the worsening of symptoms that may compromise quality of life (e.g., pain that cannot be managed with medication); rapid increase in tumor size; tumor growth leading to the proximity or compression of important structures, such as organs, large vessels, or nerves; or continued progression over time. In such cases, a multidisciplinary team (including oncologists, surgeons, pathologists, radiologists, and pain therapists) with expertise in DT treatment evaluates both the clinical situation and patient characteristics to choose the best treatment for each patient. […] This study aimed to evaluate event-free survival (where the event was either a recurrence after initial surgical treatment or a change in the therapeutic management) and pain management according to different treatment strategies.

• #38 Molecular pathogenesis of desmoid tumor and the role of γ-secretase inhibition | npj Precision Oncology

  https://www.nature.com/articles/s41698-022-00308-1

  Historically, the primary treatment for DT was surgery; however, high rates of local recurrence and poor functional outcomes following surgery have led to a shift toward nonsurgical approaches, either active surveillance or medical management. […] Spontaneous regression is well reported in DT and may occur in up to 10-20% of patients. […] Several studies have demonstrated that a period of surveillance (also called watchful waiting) can result in long-term avoidance of local and systemic interventions. […] Thus, nearly all patients with asymptomatic DT should receive a period of active surveillance. […] Locoregional treatments that have shown some benefit in managing DT include radiation therapy, cryoablation, and high-intensity focused ultrasound. […] Although no medications have as yet received regulatory approval for the treatment of DT, a number of alternatives have been investigated, including hormonal therapy, non-steroidal anti-inflammatory drugs (NSAIDs), chemotherapy, and small molecule therapies such as tyrosine kinase inhibitors (TKIs).

• #39 Desmoid Tumors Market Size & Share | Forecast – 2034

  https://www.imarcgroup.com/desmoid-tumors-market

  The desmoid tumors market has been comprehensively analyzed in IMARC’s new report titled „Desmoid Tumors Market: Epidemiology, Industry Trends, Share, Size, Growth, Opportunity, and Forecast 2024-2034”. […] Desmoid tumors are uncommon tumors, with a reported frequency of 2-4 per million people, accounting for 0.03% of all neoplasms. […] Every year, between 900 and 1,500 people in the United States are diagnosed with a desmoid tumor. […] According to epidemiological research, the majority of desmoid tumor cases occur between the ages of 20 and 44, with females exceeding males by 2.2-3.9 times. […] In patients with desmoid tumors, familial adenomatous polyposis can be diagnosed in up to 5% of patients. […] Spontaneous regressions of the condition are noted in as many as 20%-30% of cases.

• #40 Approaches to Patient Management of Desmoid Tumors –

  https://www.desmoidtumors.com/hcp/management/

  A course of ongoing observation is an appropriate option even for patients with disease progression, if the patient is minimally symptomatic and the anatomical location of the tumor is not critical. […] For progressive, morbid, or symptomatic desmoid tumors, systemic therapies are recommended as a first-line treatment option according to the NCCN Guidelines and DTWG Guideline. […] According to the NCCN Guidelines: In general, surgery is not considered a first-line treatment option for desmoid tumors, except in certain situations if agreed upon by a multidisciplinary tumor board. […] Desmoid tumors are invasive and clear margins are challenging to achieve. […] Although they do not metastasize, desmoid tumors are associated with local recurrence rates ranging from 24% to 77% after surgical resection, regardless of margin status.

• #41 Active Surveillance Approach Can Avoid Treatment for Desmoid Tumors

  https://www.targetedonc.com/view/active-surveillance-approach-can-avoid-treatment-for-desmoid-tumors

  The Desmoid Tumor Working Group updated the guidelines and the first thing that you’ll notice is that after you make a diagnosis, you watch [with active surveillance]. […] The guidelines say that you watch for 1 to 2 years. […] Because the tumor can get better on its own, and the recurrence rates with surgery approach 75%. […] The NCCN guidelines comment on the Working Group for desmoids, number 1 being [a recommendation to be treated at] centers with expertise, considering [evaluation for] familial adenomatous polyposis [FAP] for young women, and appropriate colorectal screening and then imaging for young individuals with family history of colorectal cancer. […] If it progresses, you [might] still watch. Location is key. […] The NCCN guidelines say every 3 to 6 months for 2 to 3 years, and every 6 to 12 months [thereafter].

• #42 Desmoid tumours (extra-abdominal), a surgeon’s nightmare | Bone & Joint

  https://boneandjoint.org.uk/Article/10.1302/0301-620X.105B7.BJJ-2023-0117

  Progressive symptoms and/or enlargement of the tumour are the main indications for discontinuing surveillance in favour of active treatment. […] According to the Joint Global Consensus-Based Guideline Approach, the site of the tumour is the main factor which guides decisions about the type of active intervention, while also considering the risk of complications and the patients age. […] For extra-abdominal desmoid tumours, systemic treatment should be the first option. […] There is currently insufficient comparative information to define a unique sequence of systemic medical treatments or a preference towards one or another. […] Patients with more symptoms or an aggressive tumour are eligible for low-dose methotrexate with vinblastine or vinorelbine, which usually achieves long-term control with a favourable profile of side effects and acceptable toxicity.

• #43 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10744644/

  In patients with desmoid tumors (DTs), active surveillance has been increasingly preferred over surgery, while treatment (including pharmacological therapy, radiotherapy, and/or surgery) is performed in cases with confirmed disease progression. […] Our findings support the role of active surveillance as initial management, as demonstrated by the fact that about half the patients did not experience any progression, while surgery can be reserved as a first-line approach for selected patients. […] Currently, a wait-and-see approach based on active surveillance is preferred, especially for asymptomatic patients, and treatment, including pharmacological therapy, radiotherapy, and/or surgery, should be postponed until the disease has demonstrated progression. […] Active surveillance is generally the first approach for most people with a new diagnosis of DT and consists of active monitoring or active observation by check-up visits with the oncologist every three to four months.

• #44 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://www.mdpi.com/2075-4426/13/12/1653

  Currently, a “wait-and-see” approach based on active surveillance is preferred, especially for asymptomatic patients, and treatment, including pharmacological therapy, radiotherapy, and/or surgery, should be postponed until the disease has demonstrated progression. Non-invasive treatments are generally preferred over surgery to avoid unnecessary complications associated with tissue destruction. Spontaneous disease regression has been reported to occur in approximately 30% of DT cases. […] Active surveillance is generally the first approach for most people with a new diagnosis of DT and consists of active monitoring or active observation by check-up visits with the oncologist every three to four months. At each visit, the decision to stop active surveillance and start specific therapy is based on clinical and radiological examinations (such as ultrasound or magnetic resonance imaging), and may be driven by the worsening of symptoms that may compromise quality of life (e.g., pain that cannot be managed with medication); rapid increase in tumor size; tumor growth leading to the proximity or compression of important structures, such as organs, large vessels, or nerves; or continued progression over time. In such cases, a multidisciplinary team (including oncologists, surgeons, pathologists, radiologists, and pain therapists) with expertise in DT treatment evaluates both the clinical situation and patient characteristics to choose the best treatment for each patient. […] This study aimed to evaluate event-free survival (where the event was either a recurrence after initial surgical treatment or a change in the therapeutic management) and pain management according to different treatment strategies.

• #45 What is a Desmoid Tumor? – The Desmoid Tumor Research Foundation

  https://dtrf.org/about-desmoid-tumors/what-is-a-desmoid-tumor/

  Given the rarity of this disease and lack of awareness outside of sarcoma specialists, desmoid tumors are often misdiagnosed, which can lead to improper clinical treatment. We highly recommend that you seek out a sarcoma specialist at a sarcoma center if you have been diagnosed with a desmoid tumor to ensure that you receive knowledgeable and appropriate care. […] Active surveillance is the accepted first-line treatment in most cases. […] Surgery is the accepted second-line treatment only for sporadic desmoid tumors located in the abdominal wall failing observation.

• #46 Active Surveillance Approach Can Avoid Treatment for Desmoid Tumors

  https://www.targetedonc.com/view/active-surveillance-approach-can-avoid-treatment-for-desmoid-tumors

  The Desmoid Tumor Working Group updated the guidelines and the first thing that you’ll notice is that after you make a diagnosis, you watch [with active surveillance]. […] The guidelines say that you watch for 1 to 2 years. […] Because the tumor can get better on its own, and the recurrence rates with surgery approach 75%. […] The NCCN guidelines comment on the Working Group for desmoids, number 1 being [a recommendation to be treated at] centers with expertise, considering [evaluation for] familial adenomatous polyposis [FAP] for young women, and appropriate colorectal screening and then imaging for young individuals with family history of colorectal cancer. […] If it progresses, you [might] still watch. Location is key. […] The NCCN guidelines say every 3 to 6 months for 2 to 3 years, and every 6 to 12 months [thereafter].

• #47 Desmoid-type fibromatosis | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/desmoid-type-fibromatosis/

  DF is rare. In every million people, around 5-6 could be expected to develop DF. […] Active surveillance is usually the first approach to treat people who are newly diagnosed with DF. This is sometimes known as a watch and wait policy, active monitoring or active observation. […] People diagnosed with DF are usually on active surveillance for 1-2 years. You may be on active surveillance for a shorter or longer time depending on your individual circumstances. If your condition stabilises or regresses, active surveillance will continue. If your condition progresses, then other treatment options will be considered for you.

• #48 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://www.mdpi.com/2075-4426/13/12/1653

  Currently, a “wait-and-see” approach based on active surveillance is preferred, especially for asymptomatic patients, and treatment, including pharmacological therapy, radiotherapy, and/or surgery, should be postponed until the disease has demonstrated progression. Non-invasive treatments are generally preferred over surgery to avoid unnecessary complications associated with tissue destruction. Spontaneous disease regression has been reported to occur in approximately 30% of DT cases. […] Active surveillance is generally the first approach for most people with a new diagnosis of DT and consists of active monitoring or active observation by check-up visits with the oncologist every three to four months. At each visit, the decision to stop active surveillance and start specific therapy is based on clinical and radiological examinations (such as ultrasound or magnetic resonance imaging), and may be driven by the worsening of symptoms that may compromise quality of life (e.g., pain that cannot be managed with medication); rapid increase in tumor size; tumor growth leading to the proximity or compression of important structures, such as organs, large vessels, or nerves; or continued progression over time. In such cases, a multidisciplinary team (including oncologists, surgeons, pathologists, radiologists, and pain therapists) with expertise in DT treatment evaluates both the clinical situation and patient characteristics to choose the best treatment for each patient. […] This study aimed to evaluate event-free survival (where the event was either a recurrence after initial surgical treatment or a change in the therapeutic management) and pain management according to different treatment strategies.

• #49 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://www.mdpi.com/2077-0383/9/12/4012

  Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Taking into account the unpredictable course of this benign, but locally aggressive tumor, recent position papers by the European Desmoid Working Group and of the European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)/Sarcoma Patients EuroNet (SPAEN) consider active surveillance with a regular instrumental monitoring (MRI or CT scan, depending on the anatomical site of origin of DT) from 1 to 3 months from baseline, and every 3–6 months thereafter, the “first-line” approach to asymptomatic DTs, independently from the sites of origin and from dimensions. The decision to start a treatment should rely only upon the documentation of a confirmed progression of disease on more than one instrumental reevaluation, with the only exceptions being worsening of symptoms or specific anatomic sites, as mesentery/abdominal cavity or head–neck district. In these latter cases, an active treatment (local or systemic) should be considered at the first documentation of progression of disease. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #50 Active Surveillance Approach Can Avoid Treatment for Desmoid Tumors

  https://www.targetedonc.com/view/active-surveillance-approach-can-avoid-treatment-for-desmoid-tumors

  The Desmoid Tumor Working Group updated the guidelines and the first thing that you’ll notice is that after you make a diagnosis, you watch [with active surveillance]. […] The guidelines say that you watch for 1 to 2 years. […] Because the tumor can get better on its own, and the recurrence rates with surgery approach 75%. […] The NCCN guidelines comment on the Working Group for desmoids, number 1 being [a recommendation to be treated at] centers with expertise, considering [evaluation for] familial adenomatous polyposis [FAP] for young women, and appropriate colorectal screening and then imaging for young individuals with family history of colorectal cancer. […] If it progresses, you [might] still watch. Location is key. […] The NCCN guidelines say every 3 to 6 months for 2 to 3 years, and every 6 to 12 months [thereafter].

• #51 Desmoid tumors Sarcoma – GMKA – Global Medical Knowledge Alliance

  https://gmka.org/en/articles/desmoyidni-puhlyny/

  Desmoid Tumors (DTs) are locally aggressive but non-metastasizing, deep-seated, monoclonal fibroblastic neoplasms with an annual incidence of 5 to 6 cases per million. Recognition of their unpredictable biological behavior that may lead to spontaneous regression or asymptomatic stability has led to major shifts in management strategies away from upfront treatment toward initial front-line active surveillance. […] Currently, ESMO and DTWG guidelines recommend active surveillance when the DT size does not pose a vital risk to the patient and otherwise propose medical treatment as the first-line strategy in select patients who truly require aggressive local treatment to avoid over-treating a large proportion of patients with DTs. […] Monitoring with computed tomography (CT) or MRI imaging according to the tumor location should be performed closely after the diagnosis to avoid missing a significant progression. The first surveillance exam is typically performed one or two months after the initial imaging.

• #52 Desmoid-type fibromatosis | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/desmoid-type-fibromatosis/

  DF is rare. In every million people, around 5-6 could be expected to develop DF. […] Active surveillance is usually the first approach to treat people who are newly diagnosed with DF. This is sometimes known as a watch and wait policy, active monitoring or active observation. […] People diagnosed with DF are usually on active surveillance for 1-2 years. You may be on active surveillance for a shorter or longer time depending on your individual circumstances. If your condition stabilises or regresses, active surveillance will continue. If your condition progresses, then other treatment options will be considered for you.

• #53 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10744644/

  This study aimed to evaluate event-free survival (where the event was either a recurrence after initial surgical treatment or a change in the therapeutic management) and pain management according to different treatment strategies. […] Event-free survival (where the event was recurrence after initial surgical treatment or a change in therapeutic management after initial non-surgical treatment) at 1, 3, and 5 years was: 85%, 70%, and 62% in the SGPre12 group; 76%, 58%, and 49% in the SGPost12 group; 49%, 31%, and 31% in the MG group; and 45%, 45%, and 45% in the ASG group. […] Our study supports the role of active surveillance as initial management, provided that about half of the patients did not experience any progression, while surgery or other treatment approaches may be considered as first-line treatment for selected patients.

• #54 Evaluation of outcomes in the management of primary sporadic desmoid-type fibromatosis at a specialist soft tissue sarcoma unit | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-022-00751-7

  Desmoids are rare fibroblastic tumours whose treatment in any individual case presents a persistent challenge. We endeavoured to evaluate various clinicopathological factors contributing to tumour behaviour. This is a retrospective review of 95 primary truncal sporadic fibromatosis managed between 2011 and 2020. We studied progression rate for wait and watch (WW) and recurrence rate for the surgically treated group as adverse events. Relevant event-free survivals and potential risk factors were analysed over a median follow-up of 27 months. 66 patients (69.5%) received watchful surveillance and 28 upfront surgery. 2-year progression-free survival in WW group (88.9%) was higher than RFS in the surgical group (77.1%) p=0.02. Adverse event rate compared favourably, 28.8% in WW and 28.6% in surgical group. At final follow-up, rate of stable disease for WW was 47%, and the regression rate was 24.2%. On Cox regression analysis, meantime to progress was 142.0 months, with larger tumour size as a significant prognostic indicator (p=0.05). Surgical group’s mean time to recurrence was 13.82.76 months, with tumour location a significant contributing factor (p=0.05). This study confirms to the safety of both treatment approaches. Adverse event rates remained comparable, but event-free survival was longer for the watchful surveillance group. Sporadic tumours have a recognised unpredictable natural history. The spectrum ranges from those having an indolent clinical course or even spontaneous regression on a wait and watch strategy, to rapidly growing locally aggressive tumours. Some of these will develop local recurrence after complete surgical resection or require adjuvant treatment modalities for control of the disease. Although there has been a paradigm shift towards watchful waiting in past decades with commendable results, it is this variable course of the disease combined with its relative rarity that still necessitates individualisation of management approach. The primary endpoints were rate of progression and rate of regression for patients managed with the Wait and watch approach, and rate of recurrence for the surgical resection group. The active surveillance group included subjects observed with a wait and watch strategy (WW). Progression was defined as a failure of wait and watch approach by documentation of an increase in tumour size necessitating further intervention. Throughout the study, surgical resections were performed by a set of trained sarcoma specialists. The disease course was analysed clinically as well as radiologically with all significant events including progression, stable disease, regression as well as recurrence documented in accordance with the RECIST. The rate of dimensionally stable disease from this population group over a median follow-up of 26 months was 47% (n=31), regression was achieved in 24.2% (n=16) at final follow-up, while the rate of progression was 28.8% (n=19). The total number of cases who had a surgical resection during their management were 35 (36.8%). The rate of recurrence in the surgical group over a median follow-up of 30 months was 28.6% (n=10), with a mean time to recurrence of 13.82.76 SE of mean (95% CI 10.2819.38). The adverse event rate in the present study remained comparable for both treatment approaches and identical to the reported data. This confirms the safety of both treatment approaches in sporadic primary desmoids. However, the event-free survival in our study was better for the watchful surveillance group, which shall be given primary consideration. […] The objectives of this study were to evaluate and report upon the management of abdominal desmoid tumours at our centre and compare it with the national and international standards.

• #55 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://www.mdpi.com/2075-4426/13/12/1653

  Currently, a “wait-and-see” approach based on active surveillance is preferred, especially for asymptomatic patients, and treatment, including pharmacological therapy, radiotherapy, and/or surgery, should be postponed until the disease has demonstrated progression. Non-invasive treatments are generally preferred over surgery to avoid unnecessary complications associated with tissue destruction. Spontaneous disease regression has been reported to occur in approximately 30% of DT cases. […] Active surveillance is generally the first approach for most people with a new diagnosis of DT and consists of active monitoring or active observation by check-up visits with the oncologist every three to four months. At each visit, the decision to stop active surveillance and start specific therapy is based on clinical and radiological examinations (such as ultrasound or magnetic resonance imaging), and may be driven by the worsening of symptoms that may compromise quality of life (e.g., pain that cannot be managed with medication); rapid increase in tumor size; tumor growth leading to the proximity or compression of important structures, such as organs, large vessels, or nerves; or continued progression over time. In such cases, a multidisciplinary team (including oncologists, surgeons, pathologists, radiologists, and pain therapists) with expertise in DT treatment evaluates both the clinical situation and patient characteristics to choose the best treatment for each patient. […] This study aimed to evaluate event-free survival (where the event was either a recurrence after initial surgical treatment or a change in the therapeutic management) and pain management according to different treatment strategies.

• #56 Desmoid tumours (extra-abdominal), a surgeon’s nightmare | Bone & Joint

  https://boneandjoint.org.uk/Article/10.1302/0301-620X.105B7.BJJ-2023-0117

  Progressive symptoms and/or enlargement of the tumour are the main indications for discontinuing surveillance in favour of active treatment. […] According to the Joint Global Consensus-Based Guideline Approach, the site of the tumour is the main factor which guides decisions about the type of active intervention, while also considering the risk of complications and the patients age. […] For extra-abdominal desmoid tumours, systemic treatment should be the first option. […] There is currently insufficient comparative information to define a unique sequence of systemic medical treatments or a preference towards one or another. […] Patients with more symptoms or an aggressive tumour are eligible for low-dose methotrexate with vinblastine or vinorelbine, which usually achieves long-term control with a favourable profile of side effects and acceptable toxicity.

• #57 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://www.mdpi.com/2077-0383/9/12/4012

  Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Taking into account the unpredictable course of this benign, but locally aggressive tumor, recent position papers by the European Desmoid Working Group and of the European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)/Sarcoma Patients EuroNet (SPAEN) consider active surveillance with a regular instrumental monitoring (MRI or CT scan, depending on the anatomical site of origin of DT) from 1 to 3 months from baseline, and every 3–6 months thereafter, the “first-line” approach to asymptomatic DTs, independently from the sites of origin and from dimensions. The decision to start a treatment should rely only upon the documentation of a confirmed progression of disease on more than one instrumental reevaluation, with the only exceptions being worsening of symptoms or specific anatomic sites, as mesentery/abdominal cavity or head–neck district. In these latter cases, an active treatment (local or systemic) should be considered at the first documentation of progression of disease. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #58 Evaluation of outcomes in the management of primary sporadic desmoid-type fibromatosis at a specialist soft tissue sarcoma unit | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-022-00751-7

  Desmoids are rare fibroblastic tumours whose treatment in any individual case presents a persistent challenge. We endeavoured to evaluate various clinicopathological factors contributing to tumour behaviour. This is a retrospective review of 95 primary truncal sporadic fibromatosis managed between 2011 and 2020. We studied progression rate for wait and watch (WW) and recurrence rate for the surgically treated group as adverse events. Relevant event-free survivals and potential risk factors were analysed over a median follow-up of 27 months. 66 patients (69.5%) received watchful surveillance and 28 upfront surgery. 2-year progression-free survival in WW group (88.9%) was higher than RFS in the surgical group (77.1%) p=0.02. Adverse event rate compared favourably, 28.8% in WW and 28.6% in surgical group. At final follow-up, rate of stable disease for WW was 47%, and the regression rate was 24.2%. On Cox regression analysis, meantime to progress was 142.0 months, with larger tumour size as a significant prognostic indicator (p=0.05). Surgical group’s mean time to recurrence was 13.82.76 months, with tumour location a significant contributing factor (p=0.05). This study confirms to the safety of both treatment approaches. Adverse event rates remained comparable, but event-free survival was longer for the watchful surveillance group. Sporadic tumours have a recognised unpredictable natural history. The spectrum ranges from those having an indolent clinical course or even spontaneous regression on a wait and watch strategy, to rapidly growing locally aggressive tumours. Some of these will develop local recurrence after complete surgical resection or require adjuvant treatment modalities for control of the disease. Although there has been a paradigm shift towards watchful waiting in past decades with commendable results, it is this variable course of the disease combined with its relative rarity that still necessitates individualisation of management approach. The primary endpoints were rate of progression and rate of regression for patients managed with the Wait and watch approach, and rate of recurrence for the surgical resection group. The active surveillance group included subjects observed with a wait and watch strategy (WW). Progression was defined as a failure of wait and watch approach by documentation of an increase in tumour size necessitating further intervention. Throughout the study, surgical resections were performed by a set of trained sarcoma specialists. The disease course was analysed clinically as well as radiologically with all significant events including progression, stable disease, regression as well as recurrence documented in accordance with the RECIST. The rate of dimensionally stable disease from this population group over a median follow-up of 26 months was 47% (n=31), regression was achieved in 24.2% (n=16) at final follow-up, while the rate of progression was 28.8% (n=19). The total number of cases who had a surgical resection during their management were 35 (36.8%). The rate of recurrence in the surgical group over a median follow-up of 30 months was 28.6% (n=10), with a mean time to recurrence of 13.82.76 SE of mean (95% CI 10.2819.38). The adverse event rate in the present study remained comparable for both treatment approaches and identical to the reported data. This confirms the safety of both treatment approaches in sporadic primary desmoids. However, the event-free survival in our study was better for the watchful surveillance group, which shall be given primary consideration. […] The objectives of this study were to evaluate and report upon the management of abdominal desmoid tumours at our centre and compare it with the national and international standards.

• #59

  https://link.springer.com/article/10.1245/s10434-019-07827-5

  In the management of DT patients, understanding in whom to intervene and when are seminal questions that are not yet resolved. […] Desmoid tumors (also known as desmoid-type fibromatosis) are rare mesenchymal neoplasms of fibroblastic derivation that can be locally invasive, but have no metastatic potential. They occur with an annual incidence of 24 per 1,000,000 individuals and tend to develop in younger patients. […] Furthermore, active surveillance defined as establishing the diagnosis of DT, performing serial imaging (MR, CT, and /or US) with patient evaluation every 3-6 months, to determine if the tumor may require intervention, has been adopted as primary standard of care by the majority of expert centers over the past 5-10 years. […] Nonetheless, this seminal study from the Royal Marsden provides further credence that active surveillance should be the frontline approach in sporadic DT as only 46% of the patients progressed and/or symptomatically required intervention.

• #60 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://www.mdpi.com/2075-4426/13/12/1653

  Currently, a “wait-and-see” approach based on active surveillance is preferred, especially for asymptomatic patients, and treatment, including pharmacological therapy, radiotherapy, and/or surgery, should be postponed until the disease has demonstrated progression. Non-invasive treatments are generally preferred over surgery to avoid unnecessary complications associated with tissue destruction. Spontaneous disease regression has been reported to occur in approximately 30% of DT cases. […] Active surveillance is generally the first approach for most people with a new diagnosis of DT and consists of active monitoring or active observation by check-up visits with the oncologist every three to four months. At each visit, the decision to stop active surveillance and start specific therapy is based on clinical and radiological examinations (such as ultrasound or magnetic resonance imaging), and may be driven by the worsening of symptoms that may compromise quality of life (e.g., pain that cannot be managed with medication); rapid increase in tumor size; tumor growth leading to the proximity or compression of important structures, such as organs, large vessels, or nerves; or continued progression over time. In such cases, a multidisciplinary team (including oncologists, surgeons, pathologists, radiologists, and pain therapists) with expertise in DT treatment evaluates both the clinical situation and patient characteristics to choose the best treatment for each patient. […] This study aimed to evaluate event-free survival (where the event was either a recurrence after initial surgical treatment or a change in the therapeutic management) and pain management according to different treatment strategies.

• #61 Approaches to Patient Management of Desmoid Tumors –

  https://www.desmoidtumors.com/hcp/management/

  Patients with desmoid tumors seek both disease control and symptom improvement. […] For progressive, morbid, or symptomatic desmoid tumors, systemic therapies are recommended as a first-line treatment option according to the NCCN Guidelines and DTWG Guideline. […] Although they do not metastasize, desmoid tumors are associated with local recurrence rates ranging from 24% to 77% after surgical resection, regardless of margin status. […] The NCCN Guidelines and DTWG Guideline recommend engaging a multidisciplinary care team with experience in desmoid tumors. […] Active surveillance may be an appropriate option for patients who have desmoid tumors that are asymptomatic and not progressing or morbid. […] NCCN Guidelines recommend continued observation with MRI or CT scan. […] According to the DTWG Guideline, active surveillance means that patients need to be continuously monitored with the next MRI within 1-2 months, then in 3-6 months intervals.

• #62 Approaches to Patient Management of Desmoid Tumors –

  https://www.desmoidtumors.com/hcp/management/

  Patients with desmoid tumors seek both disease control and symptom improvement. […] For progressive, morbid, or symptomatic desmoid tumors, systemic therapies are recommended as a first-line treatment option according to the NCCN Guidelines and DTWG Guideline. […] Although they do not metastasize, desmoid tumors are associated with local recurrence rates ranging from 24% to 77% after surgical resection, regardless of margin status. […] The NCCN Guidelines and DTWG Guideline recommend engaging a multidisciplinary care team with experience in desmoid tumors. […] Active surveillance may be an appropriate option for patients who have desmoid tumors that are asymptomatic and not progressing or morbid. […] NCCN Guidelines recommend continued observation with MRI or CT scan. […] According to the DTWG Guideline, active surveillance means that patients need to be continuously monitored with the next MRI within 1-2 months, then in 3-6 months intervals.

• #63 What is a Desmoid Tumor? – The Desmoid Tumor Research Foundation

  https://dtrf.org/about-desmoid-tumors/what-is-a-desmoid-tumor/

  Given the rarity of this disease and lack of awareness outside of sarcoma specialists, desmoid tumors are often misdiagnosed, which can lead to improper clinical treatment. We highly recommend that you seek out a sarcoma specialist at a sarcoma center if you have been diagnosed with a desmoid tumor to ensure that you receive knowledgeable and appropriate care. […] Active surveillance is the accepted first-line treatment in most cases. […] Surgery is the accepted second-line treatment only for sporadic desmoid tumors located in the abdominal wall failing observation.

• #64 Desmoid Tumors in the Active Surveillance Era: Evaluation of Treatment Options and Pain Relief in a Single-Center Retrospective Analysis

  https://www.mdpi.com/2075-4426/13/12/1653

  Currently, a “wait-and-see” approach based on active surveillance is preferred, especially for asymptomatic patients, and treatment, including pharmacological therapy, radiotherapy, and/or surgery, should be postponed until the disease has demonstrated progression. Non-invasive treatments are generally preferred over surgery to avoid unnecessary complications associated with tissue destruction. Spontaneous disease regression has been reported to occur in approximately 30% of DT cases. […] Active surveillance is generally the first approach for most people with a new diagnosis of DT and consists of active monitoring or active observation by check-up visits with the oncologist every three to four months. At each visit, the decision to stop active surveillance and start specific therapy is based on clinical and radiological examinations (such as ultrasound or magnetic resonance imaging), and may be driven by the worsening of symptoms that may compromise quality of life (e.g., pain that cannot be managed with medication); rapid increase in tumor size; tumor growth leading to the proximity or compression of important structures, such as organs, large vessels, or nerves; or continued progression over time. In such cases, a multidisciplinary team (including oncologists, surgeons, pathologists, radiologists, and pain therapists) with expertise in DT treatment evaluates both the clinical situation and patient characteristics to choose the best treatment for each patient. […] This study aimed to evaluate event-free survival (where the event was either a recurrence after initial surgical treatment or a change in the therapeutic management) and pain management according to different treatment strategies.

• #65 What is a Desmoid Tumor? – The Desmoid Tumor Research Foundation

  https://dtrf.org/about-desmoid-tumors/what-is-a-desmoid-tumor/

  Given the rarity of this disease and lack of awareness outside of sarcoma specialists, desmoid tumors are often misdiagnosed, which can lead to improper clinical treatment. We highly recommend that you seek out a sarcoma specialist at a sarcoma center if you have been diagnosed with a desmoid tumor to ensure that you receive knowledgeable and appropriate care. […] Active surveillance is the accepted first-line treatment in most cases. […] Surgery is the accepted second-line treatment only for sporadic desmoid tumors located in the abdominal wall failing observation.

• #66 Desmoid Tumors: An In-Depth Review

  https://www.scitechnol.com/peer-review/desmoid-tumors-an-indepth-review-Zz6O.php?article_id=26572

  Desmoid tumors can occur sporadically or as part of genetic syndromes such as Familial Adenomatous Polyposis (FAP). […] The clinical management of desmoid tumors requires a multidisciplinary approach, involving surgery, radiation therapy, and systemic treatment, often guided by tumor localization, size, and patient-specific factors. […] Regular follow-up is crucial for monitoring disease recurrence and managing any long-term effects of treatment. […] Follow-up typically involves: Clinical examination: Periodic physical examinations to detect any signs of recurrence or complications. […] Imaging studies: Routine imaging (e.g., MRI or CT scans) to monitor for residual or recurrent disease, especially in the first few years after treatment. […] Patient education: Educating patients about signs of recurrence and the importance of adherence to follow-up schedules.

• #67 Desmoid tumors Sarcoma – GMKA – Global Medical Knowledge Alliance

  https://gmka.org/en/articles/desmoyidni-puhlyny/

  Desmoid Tumors (DTs) are locally aggressive but non-metastasizing, deep-seated, monoclonal fibroblastic neoplasms with an annual incidence of 5 to 6 cases per million. Recognition of their unpredictable biological behavior that may lead to spontaneous regression or asymptomatic stability has led to major shifts in management strategies away from upfront treatment toward initial front-line active surveillance. […] Currently, ESMO and DTWG guidelines recommend active surveillance when the DT size does not pose a vital risk to the patient and otherwise propose medical treatment as the first-line strategy in select patients who truly require aggressive local treatment to avoid over-treating a large proportion of patients with DTs. […] Monitoring with computed tomography (CT) or MRI imaging according to the tumor location should be performed closely after the diagnosis to avoid missing a significant progression. The first surveillance exam is typically performed one or two months after the initial imaging.

• #68 Diagnosis and Management of Desmoid Fibromatosis of the Breast | Kangas-Dick | World Journal of Oncology

  https://www.wjon.org/index.php/wjon/article/view/1844/1588

  After definitive therapy, patients should continue to be followed for surveillance every 3 to 6 months for the first 2 years and yearly with MRI afterwards, as well as continuing age and patient appropriate screening for breast cancer. […] Desmoid fibromatosis of the breast is a rare locally invasive tumor malignancy that rarely leads to metastatic disease.

• #69 ICD-10 Impact: Desmoid Tumor Advances

  https://www.ajmc.com/view/icd-10-impact-desmoid-tumor-advances

  New location-specific International Statistical Classification of Diseases, Tenth Revision (ICD-10) codes for desmoid tumors became available in October 2022 after advocacy from the Desmoid Tumor Research Foundation. […] These codes will enable better understanding of desmoid tumor epidemiology, facilitating research and appropriate treatments. […] The new codes provide needed specificity on incidence, prevalence, geographic distribution, and outcomes. […] Overall, the new ICD-10 codes though requiring diligent application should improve desmoid tumor diagnosis, treatment, research, reimbursement, and, ultimately, patient outcomes.

• #70 ICD-10 Impact: Desmoid Tumor Advances

  https://www.ajmc.com/view/icd-10-impact-desmoid-tumor-advances

  New location-specific International Statistical Classification of Diseases, Tenth Revision (ICD-10) codes for desmoid tumors became available in October 2022 after advocacy from the Desmoid Tumor Research Foundation. […] These codes will enable better understanding of desmoid tumor epidemiology, facilitating research and appropriate treatments. […] The new codes provide needed specificity on incidence, prevalence, geographic distribution, and outcomes. […] Overall, the new ICD-10 codes though requiring diligent application should improve desmoid tumor diagnosis, treatment, research, reimbursement, and, ultimately, patient outcomes.

• #71 Desmoid tumours (extra-abdominal), a surgeon’s nightmare | Bone & Joint

  https://boneandjoint.org.uk/Article/10.1302/0301-620X.105B7.BJJ-2023-0117

  The guidelines describe a stepwise approach and advocate surveillance as the first line of management for most, if not all, patients. […] Several retrospective series have supported the use of surveillance, documenting the natural history of this condition, for which a natural arrest of growth is not uncommon. […] Three prospective European studies have recently assessed the role of surveillance in the management of patients with a desmoid tumour. […] A significant correlation between tumours of larger size and the presence of the S45F mutation was reported, and this specific mutation was an independent predictor for switching to active treatment. […] The site of the tumour was identified as a prognostic factor for local recurrence in several studies, and tumours in the limbs, especially in distal sites, had the worst prognosis.

• #72 Evaluation of outcomes in the management of primary sporadic desmoid-type fibromatosis at a specialist soft tissue sarcoma unit | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-022-00751-7

  Desmoids are rare fibroblastic tumours whose treatment in any individual case presents a persistent challenge. We endeavoured to evaluate various clinicopathological factors contributing to tumour behaviour. This is a retrospective review of 95 primary truncal sporadic fibromatosis managed between 2011 and 2020. We studied progression rate for wait and watch (WW) and recurrence rate for the surgically treated group as adverse events. Relevant event-free survivals and potential risk factors were analysed over a median follow-up of 27 months. 66 patients (69.5%) received watchful surveillance and 28 upfront surgery. 2-year progression-free survival in WW group (88.9%) was higher than RFS in the surgical group (77.1%) p=0.02. Adverse event rate compared favourably, 28.8% in WW and 28.6% in surgical group. At final follow-up, rate of stable disease for WW was 47%, and the regression rate was 24.2%. On Cox regression analysis, meantime to progress was 142.0 months, with larger tumour size as a significant prognostic indicator (p=0.05). Surgical group’s mean time to recurrence was 13.82.76 months, with tumour location a significant contributing factor (p=0.05). This study confirms to the safety of both treatment approaches. Adverse event rates remained comparable, but event-free survival was longer for the watchful surveillance group. Sporadic tumours have a recognised unpredictable natural history. The spectrum ranges from those having an indolent clinical course or even spontaneous regression on a wait and watch strategy, to rapidly growing locally aggressive tumours. Some of these will develop local recurrence after complete surgical resection or require adjuvant treatment modalities for control of the disease. Although there has been a paradigm shift towards watchful waiting in past decades with commendable results, it is this variable course of the disease combined with its relative rarity that still necessitates individualisation of management approach. The primary endpoints were rate of progression and rate of regression for patients managed with the Wait and watch approach, and rate of recurrence for the surgical resection group. The active surveillance group included subjects observed with a wait and watch strategy (WW). Progression was defined as a failure of wait and watch approach by documentation of an increase in tumour size necessitating further intervention. Throughout the study, surgical resections were performed by a set of trained sarcoma specialists. The disease course was analysed clinically as well as radiologically with all significant events including progression, stable disease, regression as well as recurrence documented in accordance with the RECIST. The rate of dimensionally stable disease from this population group over a median follow-up of 26 months was 47% (n=31), regression was achieved in 24.2% (n=16) at final follow-up, while the rate of progression was 28.8% (n=19). The total number of cases who had a surgical resection during their management were 35 (36.8%). The rate of recurrence in the surgical group over a median follow-up of 30 months was 28.6% (n=10), with a mean time to recurrence of 13.82.76 SE of mean (95% CI 10.2819.38). The adverse event rate in the present study remained comparable for both treatment approaches and identical to the reported data. This confirms the safety of both treatment approaches in sporadic primary desmoids. However, the event-free survival in our study was better for the watchful surveillance group, which shall be given primary consideration. […] The objectives of this study were to evaluate and report upon the management of abdominal desmoid tumours at our centre and compare it with the national and international standards.

• #73 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7764110/

  In the last few decades, the routine use of aggressive first-line treatments for DTs, such as surgery and RT, has been progressively challenged by many authors. Considering the unpredictable behavior of DTs and the high recurrence rates registered after surgery, close observation is now considered an acceptable approach, especially for asymptomatic patients; whereas treatment is recommended in symptomatic patients, in case of tumors adjacent to critical structures or for cosmetic concerns. […] Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #74 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://www.mdpi.com/2077-0383/9/12/4012

  Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Taking into account the unpredictable course of this benign, but locally aggressive tumor, recent position papers by the European Desmoid Working Group and of the European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)/Sarcoma Patients EuroNet (SPAEN) consider active surveillance with a regular instrumental monitoring (MRI or CT scan, depending on the anatomical site of origin of DT) from 1 to 3 months from baseline, and every 3–6 months thereafter, the “first-line” approach to asymptomatic DTs, independently from the sites of origin and from dimensions. The decision to start a treatment should rely only upon the documentation of a confirmed progression of disease on more than one instrumental reevaluation, with the only exceptions being worsening of symptoms or specific anatomic sites, as mesentery/abdominal cavity or head–neck district. In these latter cases, an active treatment (local or systemic) should be considered at the first documentation of progression of disease. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #75 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7764110/

  In the last few decades, the routine use of aggressive first-line treatments for DTs, such as surgery and RT, has been progressively challenged by many authors. Considering the unpredictable behavior of DTs and the high recurrence rates registered after surgery, close observation is now considered an acceptable approach, especially for asymptomatic patients; whereas treatment is recommended in symptomatic patients, in case of tumors adjacent to critical structures or for cosmetic concerns. […] Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #76

  https://link.springer.com/article/10.1245/s10434-019-07827-5

  Although first-line active surveillance is definitely more globally accepted, decision making related to the timing of intervention for DT patients undergoing observation is not as well defined. […] The type of treatment used after the decision to treat DT patients evolved during the period of this study as initially, surgical intervention dominated. However, this decreased to 20% of all interventions over time as indications were refined to keep abdominal wall disease or emergency intervention for symptomatic abdominal patients. […] The community should continue to understand the impact of surveillance on patients and coordinate prospective data collection to clarify the triggers for intervention and identify biologic factors prognostic for tumor progression.

• #77 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7764110/

  In the last few decades, the routine use of aggressive first-line treatments for DTs, such as surgery and RT, has been progressively challenged by many authors. Considering the unpredictable behavior of DTs and the high recurrence rates registered after surgery, close observation is now considered an acceptable approach, especially for asymptomatic patients; whereas treatment is recommended in symptomatic patients, in case of tumors adjacent to critical structures or for cosmetic concerns. […] Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #78 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://www.mdpi.com/2077-0383/9/12/4012

  Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Taking into account the unpredictable course of this benign, but locally aggressive tumor, recent position papers by the European Desmoid Working Group and of the European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)/Sarcoma Patients EuroNet (SPAEN) consider active surveillance with a regular instrumental monitoring (MRI or CT scan, depending on the anatomical site of origin of DT) from 1 to 3 months from baseline, and every 3–6 months thereafter, the “first-line” approach to asymptomatic DTs, independently from the sites of origin and from dimensions. The decision to start a treatment should rely only upon the documentation of a confirmed progression of disease on more than one instrumental reevaluation, with the only exceptions being worsening of symptoms or specific anatomic sites, as mesentery/abdominal cavity or head–neck district. In these latter cases, an active treatment (local or systemic) should be considered at the first documentation of progression of disease. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #79 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://www.mdpi.com/2077-0383/9/12/4012

  Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Taking into account the unpredictable course of this benign, but locally aggressive tumor, recent position papers by the European Desmoid Working Group and of the European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)/Sarcoma Patients EuroNet (SPAEN) consider active surveillance with a regular instrumental monitoring (MRI or CT scan, depending on the anatomical site of origin of DT) from 1 to 3 months from baseline, and every 3–6 months thereafter, the “first-line” approach to asymptomatic DTs, independently from the sites of origin and from dimensions. The decision to start a treatment should rely only upon the documentation of a confirmed progression of disease on more than one instrumental reevaluation, with the only exceptions being worsening of symptoms or specific anatomic sites, as mesentery/abdominal cavity or head–neck district. In these latter cases, an active treatment (local or systemic) should be considered at the first documentation of progression of disease. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #80 Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

  https://www.mdpi.com/2077-0383/9/12/4012

  Despite the increasing interest on surveillance protocols in DTs, a standardized approach is still lacking, no clear indication exists on which imaging technique (MRI or CT scan) should be preferred, and no significant prognostic or predictive factors have been identified to distinguish between patients with indolent tumors and those who would need active therapy. […] Taking into account the unpredictable course of this benign, but locally aggressive tumor, recent position papers by the European Desmoid Working Group and of the European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)/Sarcoma Patients EuroNet (SPAEN) consider active surveillance with a regular instrumental monitoring (MRI or CT scan, depending on the anatomical site of origin of DT) from 1 to 3 months from baseline, and every 3–6 months thereafter, the “first-line” approach to asymptomatic DTs, independently from the sites of origin and from dimensions. The decision to start a treatment should rely only upon the documentation of a confirmed progression of disease on more than one instrumental reevaluation, with the only exceptions being worsening of symptoms or specific anatomic sites, as mesentery/abdominal cavity or head–neck district. In these latter cases, an active treatment (local or systemic) should be considered at the first documentation of progression of disease. […] Active surveillance protocols are an important tool in the management of patients with DTs, although clear guidelines and possibly predictive markers to choose the most appropriate therapeutic path for each patient are still needed.

• #81

  https://link.springer.com/article/10.1245/s10434-019-07827-5

  Although first-line active surveillance is definitely more globally accepted, decision making related to the timing of intervention for DT patients undergoing observation is not as well defined. […] The type of treatment used after the decision to treat DT patients evolved during the period of this study as initially, surgical intervention dominated. However, this decreased to 20% of all interventions over time as indications were refined to keep abdominal wall disease or emergency intervention for symptomatic abdominal patients. […] The community should continue to understand the impact of surveillance on patients and coordinate prospective data collection to clarify the triggers for intervention and identify biologic factors prognostic for tumor progression.

• #82 Soft Tissue Sarcoma: Desmoid Tumors: What They Are, Causes, Symptoms, and Treatment | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/soft-tissue-sarcoma/types/desmoid-tumor

  Desmoid tumors are related to a group of cancers called soft tissue sarcoma and are treated by cancer doctors. […] In some cases, desmoid tumors can be monitored (watched) for a period of time. However, some desmoid tumors may need treatment sooner. They include tumors that cause symptoms, such as pain or loss of function, or that can harm vital organs. Desmoid tumors should be treated by doctors with special training in caring for people with soft tissue sarcoma. […] At MSK, we have a team of experts in: […] Quickly diagnosing desmoid tumors with MSKs Rapid Diagnosis program. […] Research studies, called clinical trials, that lead to new treatments for these tumors. […] MSK has deep experience with desmoid tumors, and developed a prediction tool to better understand that risk.

• #83 First FDA-approved Treatment for Desmoid Tumors | AACR

  https://www.aacr.org/patients-caregivers/progress-against-cancer/first-fda-approved-treatment-for-desmoid-tumors/

  The FDA has approved nirogacestat for the treatment of noncancerous but painful growths called desmoid tumors. […] According to the National Cancer Institute, each year, desmoid tumors occur in every two to four people per million worldwide.

• #84 Comparing surveillance and surgery for the management of desmoid tumors – VJOncology

  https://www.vjoncology.com/video/pii8xzbutoq-comparing-surveillance-and-surgery-for-the-management-of-desmoid-tumors/

  Joanna Szkandera, MD, Medical University of Graz, Graz, Austria, discusses the evolving treatment approach for desmoid tumors, highlighting the recent FDA approval of nirogacestat. Prioritizing active surveillance for asymptomatic patients, especially when the tumor is located outside the abdomen, has been agreed on as the best course of action in these patients. […] This approach underscores a shift towards less invasive management unless symptoms or tumor location necessitate surgical intervention.

• #85 Molecular pathogenesis of desmoid tumor and the role of γ-secretase inhibition | npj Precision Oncology

  https://www.nature.com/articles/s41698-022-00308-1

  The choice and sequence of therapy, including treatment with systemic agents, depends on individual patient characteristics such as age and comorbidities, as well as risk factors for recurrence such as location. […] More recently, γ-secretase inhibitors (GSIs) have been investigated for the treatment of DT, based on their mechanism of action to inhibit the Notch signaling pathway. […] Inhibiting the proteolytic activity of γ-secretase prevents the release of the NICD and its translocation to the nucleus—the key step for activation of all downstream effects—leading to decreased expression of several Notch target genes, including those in the HES family. […] To date, nirogacestat (formerly PF-03084014) has been the most extensively studied GSI for the treatment of DT. […] In a phase 2 study, 17 patients with unresectable DT received nirogacestat 150mg orally BID in 3-week cycles. […] The phase 2/3 RINGSIDE trial of AL102 in ~192 patients with progressive DT was initiated in March 2021.

• #86 Molecular pathogenesis of desmoid tumor and the role of γ-secretase inhibition | npj Precision Oncology

  https://www.nature.com/articles/s41698-022-00308-1

  The choice and sequence of therapy, including treatment with systemic agents, depends on individual patient characteristics such as age and comorbidities, as well as risk factors for recurrence such as location. […] More recently, γ-secretase inhibitors (GSIs) have been investigated for the treatment of DT, based on their mechanism of action to inhibit the Notch signaling pathway. […] Inhibiting the proteolytic activity of γ-secretase prevents the release of the NICD and its translocation to the nucleus—the key step for activation of all downstream effects—leading to decreased expression of several Notch target genes, including those in the HES family. […] To date, nirogacestat (formerly PF-03084014) has been the most extensively studied GSI for the treatment of DT. […] In a phase 2 study, 17 patients with unresectable DT received nirogacestat 150mg orally BID in 3-week cycles. […] The phase 2/3 RINGSIDE trial of AL102 in ~192 patients with progressive DT was initiated in March 2021.

• #87 ESMO E-Learning: Desmoid Fibromatosis: from Biology to Management

  https://oncologypro.esmo.org/education-library/esmo-e-learning-and-v-learning/desmoid-fibromatosis-from-biology-to-management

  To provide an update on epidemiology, biology, clinical presentation and natural course of disease in patients with desmoid tumours […] In the first part of the module, the author elaborates on the epidemiology of desmoid tumours with 5 cases diagnosed per one million of the population per year. In terms of incidence, the author underlines that 5-10% of desmoid tumours are associated with Gardner syndrome, while 90-95% are sporadic fibromatosis/desmoid tumours. […] The author also provides a summary of ongoing trials for the treatment of patients with desmoid tumours.

• #88 Economic evaluation of percutaneous cryoablation vs conventional surgery in extra-abdominal desmoid tumours in the Spanish healthcare system | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-023-01580-w

  The DTWG recommendations mention that local ablative treatments such as cryotherapy or radiotherapy are options that can be considered as an alternative to medical therapies on an individual basis. […] In this complex clinical scenario, the use of percutaneous cryoablation in progressed extra-abdominal DTs has recently increased and displayed promising results. […] Percutaneous cryoablation is a minimally invasive technique that causes cell death by freezing the affected tissue. […] In primary or locally recurrent extra-abdominal DTs, percutaneous cryoablation prevents primary local recurrence and achieves long-term disease control comparable to surgery. […] The analysis yields valuable findings for informed health decision-making. […] The estimation of the target population of the analysis was carried out based on the epidemiological data described.

• #89 Burden of illness of desmoid tumors | RTI Health Solutions

  https://www.rtihs.org/publications/burden-illness-desmoid-tumors

  BACKGROUND AND OBJECTIVE: Aggressive fibromatosis, or desmoid tumor (DT), is a rare, locally aggressive, and invasive soft tissue tumor. […] The low global incidence of DTs (estimated 3 to 5 cases per year per million population) limits disease awareness. […] Although no treatment approved by the US Food and Drug Administration currently exists for DTs, some of these tumors can be initially managed through active surveillance. […] The substantial burden of illness of DTs is related to the clinical and chronic symptoms (pain, physical, and functional limitations), decreased quality of life (due to anxiety and depression) and increased financial burden.

• #90

  https://link.springer.com/article/10.1007/s12325-023-02592-0

  Desmoid tumors (DT) are rare, locally aggressive, fibroblastic soft-tissue tumors that are characterized by infiltrative growth and can affect organs and adjacent structures, resulting in substantial clinical burden impacting patients health-related quality of life. […] The low incidence of DT (estimated 35 cases per million person-years) limits disease awareness. […] There is a high unmet need for treatments that specifically target DT and improve quality of life. […] Five European sources providing estimates on the incidence of DT were identified in the literature. […] The most recently published study, Anneberg et al. estimated 2.2 cases per million person-years in 2011 and 4.3 cases per million person-years in 2017 in Denmark, using the Danish Sarcoma Database and excluding patients aged 18 years and younger.

• #91 Burden of illness of desmoid tumors | RTI Health Solutions

  https://www.rtihs.org/publications/burden-illness-desmoid-tumors

  BACKGROUND AND OBJECTIVE: Aggressive fibromatosis, or desmoid tumor (DT), is a rare, locally aggressive, and invasive soft tissue tumor. […] The low global incidence of DTs (estimated 3 to 5 cases per year per million population) limits disease awareness. […] Although no treatment approved by the US Food and Drug Administration currently exists for DTs, some of these tumors can be initially managed through active surveillance. […] The substantial burden of illness of DTs is related to the clinical and chronic symptoms (pain, physical, and functional limitations), decreased quality of life (due to anxiety and depression) and increased financial burden.

• #92 Desmoid tumours (extra-abdominal), a surgeon’s nightmare | Bone & Joint

  https://boneandjoint.org.uk/Article/10.1302/0301-620X.105B7.BJJ-2023-0117

  If a more rapid response is needed because of an aggressively growing tumour in an unfavourable site, conventional dose chemotherapy using an anthracycline-based regimen should be considered. […] Surgery for desmoid tumours in the limbs or girdles has a higher rate of morbidity, potentially causing varying degrees of loss of function and cosmesis. […] The rate of recurrence after surgery is also reported to be unacceptably high, exceeding 40%. […] Using an initial period of surveillance may allow surgery to be avoided in 90% of patients with sporadic extra-abdominal desmoid tumours. […] The role of surgery for regressing and residual tumours is still much debated. […] Quality of life should be the main goal of treatment, both by optimizing the control of symptoms and by limiting as much as possible the side effects of treatment, in the short and long term. […] Surveillance should be considered as a first step at the time of diagnosis, whenever possible.

• #93 Desmoid Tumors Market: Epidemiology, Industry Trends, Share, Size, Growth, Opportunity, and Forecast 2024-2034

  https://marketpublishers.com/report/life_sciences/healthcare/desmoid-tumors-market-epidemiology-industry-trends-share-size-growth-opportunity-n-forecast-2024-2034.html

  The 7 major desmoid tumors markets reached a value of US$ 1.7 Billion in 2023. Looking forward, IMARC Group expects the 7MM to reach US$ 3.2 Billion by 2034, exhibiting a growth rate (CAGR) of 5.61% during 2024-2034. Desmoid tumors are uncommon tumors, with a reported frequency of 2-4 per million people, accounting for 0.03% of all neoplasms. Every year, between 900 and 1,500 people in the United States are diagnosed with a desmoid tumor. According to epidemiological research, the majority of desmoid tumor cases occur between the ages of 20 and 44, with females exceeding males by 2.2-3.9 times. In patients with desmoid tumors, familial adenomatous polyposis can be diagnosed in up to 5% of patients. Spontaneous regressions of the condition are noted in as many as 20%-30% of cases. […] The report also provides the current and future patient pool across the seven major markets. According to the report, the United States has the largest patient pool for desmoid tumors and also represents the largest market for its treatment.

• #94 Desmoid Tumors Market: Epidemiology, Industry Trends, Share, Size, Growth, Opportunity, and Forecast 2024-2034

  https://marketpublishers.com/report/life_sciences/healthcare/desmoid-tumors-market-epidemiology-industry-trends-share-size-growth-opportunity-n-forecast-2024-2034.html

  The 7 major desmoid tumors markets reached a value of US$ 1.7 Billion in 2023. Looking forward, IMARC Group expects the 7MM to reach US$ 3.2 Billion by 2034, exhibiting a growth rate (CAGR) of 5.61% during 2024-2034. Desmoid tumors are uncommon tumors, with a reported frequency of 2-4 per million people, accounting for 0.03% of all neoplasms. Every year, between 900 and 1,500 people in the United States are diagnosed with a desmoid tumor. According to epidemiological research, the majority of desmoid tumor cases occur between the ages of 20 and 44, with females exceeding males by 2.2-3.9 times. In patients with desmoid tumors, familial adenomatous polyposis can be diagnosed in up to 5% of patients. Spontaneous regressions of the condition are noted in as many as 20%-30% of cases. […] The report also provides the current and future patient pool across the seven major markets. According to the report, the United States has the largest patient pool for desmoid tumors and also represents the largest market for its treatment.

• #95 Desmoid Tumors Market: Epidemiology, Industry Trends, Share, Size, Growth, Opportunity, and Forecast 2024-2034

  https://www.researchandmarkets.com/reports/5970374/desmoid-tumors-market-epidemiology-industry?srsltid=AfmBOooWJzfNl-2oA70HfMsZFTnlG3z8QLkyfatUWQopx4clFU4XwWIh

  According to the report, the United States has the largest patient pool for desmoid tumors and also represents the largest market for its treatment. […] Furthermore, the current treatment practice/algorithm, market drivers, challenges, opportunities, reimbursement scenario, unmet medical needs, etc., have also been provided in the report.

• #96 Desmoid Tumors Market Size & Share | Forecast – 2034

  https://www.imarcgroup.com/desmoid-tumors-market

  What is the number of prevalent cases (2018-2034) of desmoid tumors across the seven major markets? […] How many patients are diagnosed (2018-2034) with desmoid tumors across the seven major markets? […] What is the size of the desmoid tumors patient pool (2018-2023) across the seven major markets? […] What would be the forecasted patient pool (2024-2034) across the seven major markets? […] What are the key factors driving the epidemiological trend of desmoid tumors? […] What will be the growth rate of patients across the seven major markets?

• #97 Desmoid Tumors – Epidemiology Forecast – 2032

  https://www.researchandmarkets.com/reports/5525293/desmoid-tumors-epidemiology-forecast-2032?srsltid=AfmBOooWwjQnfvfnrL5kTPOrZsK4BCq6rd6u05EBUU0VGCDc2MOXxHIM

  The Desmoid Tumors epidemiology covered in the report provides historical as well as forecasted Desmoid Tumors epidemiology scenario in the 7MM covering the United States, EU5 countries (Germany, Spain, Italy, France, and the United Kingdom), and Japan from 2019 to 2032. The Desmoid Tumors report also provides the epidemiology trends observed in the 7MM during the study period, along with the assumptions undertaken. The calculated data are presented with relevant tables and graphs to give a clear view of the epidemiology at first sight. […] The report provides insight into the historical and forecasted patient pool of Desmoid Tumors in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan. […] The report assesses the disease risk and burden and highlights the unmet needs of Desmoid Tumors.

• #98

  https://scholars.duke.edu/individual/pub1524181

  Desmoid tumors (DTs) are rare soft tissue mesenchymal neoplasms that may be associated with impairments, disfigurement, morbidity, and (rarely) mortality. […] Consequently, active surveillance in conjunction with pain management is now recommended for most patients. […] As management strategies continue to evolve, further studies will help clarify these issues and optimize outcomes for patients.

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