Materiały źródłowe

• #1 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. […] Both sporadic DT and familial adenomatous polyposis (FAP)-associated DT are linked to constitutive activation of the Wnt signaling pathway with mutations in the -catenin oncogene CTNNB1 or the tumor suppressor gene APC, respectively. […] Due to the role -secretase plays in Notch signaling through cleavage of the Notch intracellular domain (with subsequent translocation to the nucleus to activate gene transcription), -secretase inhibitors (GSIs) have emerged as a potential treatment for DT. […] More recently, -secretase inhibitors (GSIs) have been investigated for the treatment of DT, based on their mechanism of action to inhibit the Notch signaling pathway. […] Dysregulation of the Wnt signaling pathway plays a key role in DT pathogenesis, however, as the result of crosstalk, Wnt dysregulation may lead to activation of the Notch pathway.

• #2 Desmoid Tumor – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459231/

  Desmoid tumor, a rare malignancy of mesenchymal origin, affects a small proportion of the population with an incidence of 2 to 4 patients per million. Also known as „aggressive fibromatosis” or „desmoid-type fibromatosis,” these tumors primarily impact the younger age group. Despite the absence of metastasis, desmoid tumors exhibit local invasion, leading to severe morbidity. The association with the APC gene mutation can result in multifocal DTs, occasionally leading to mortality. […] The pathophysiology of desmoid tumor lies in the dysregulation of the Wnt pathway. Mutation in the -catenin gene or germline mutation in the adenomatous polyposis coli (APC) gene leads to the accumulation of the -catenin protein; in turn, -catenin is translocated to the nucleus and activates the expression of the Wnt gene. Subsequently, several proteins, including cyclo-oxygenase 1 (COX-2) and vascular endothelial growth factor, are produced, leading to tumor growth. These proteins are also the target of some medicines used in treating desmoid tumor.

• #3 Desmoid tumor pathophysiology – wikidoc

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

  Desmoid tumors arises from monoclonal proliferation of well-differentiated fibroblasts. […] The exact etiology remains uncertain, however, they are seem to be associated with antecedent surgical or accidental trauma at the tumor site and various mutations at the molecular level including beta-catenin gene, CTNNB1 or APC gene involved in Wnt/beta-cateninsignaling pathway. […] Their exact etiology remains uncertain, although they are frequently associated with previous trauma or surgical incision. On the molecular level, desmoids are characterised by mutations in the -catenin gene, CTNNB1, or the adenomatous polyposis coli gene, APC. […] The key factor in pathogenesis of desmoids at genetic level is beta-catenin stabilization implicated by mutations in two mediators of Wnt-APC-beta-catenin pathway.

• #4 Desmoid Tumors: A Clear Perspective or a Persisting Enigma? A Case Report and Review of Literature | Mishra | World Journal of Oncology

  https://www.wjon.org/index.php/wjon/article/view/961/718

  Desmoid tumors are benign but locally aggressive tumors of mesenchymal origin which are poorly circumscribed, infiltrate the surrounding tissue, lack a true capsule and are composed of abundant collagen. […] Desmoid tumors are cytologically bland fibrous neoplasms originating from the musculoaponeurotic structures throughout the body. The term desmoid, coined by Muller in 1838, is derived from the Greek word desmos, which means tendon-like. Desmoid tumors are monoclonal fibroblastic proliferations arising in musculoaponeurotic structures. They are benign but aggressive tumors of mesenchymal origin, forming a heterogenous group of pathologic entities resulting from the proliferation of well-differentiated fibroblasts. […] Although desmoid tumors most commonly arise from the rectus abdominis muscle in postpartum women and in scars due to abdominal surgery, they may arise in any skeletal muscle. Desmoid tumors tend to infiltrate adjacent muscle bundles, frequently entrapping them and causing their degeneration.

• #5 Molecular pathogenesis of desmoid tumor and the role of γ-secretase inhibition

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9448813/

  Desmoid tumor (DT) is a rare, soft tissue neoplasm associated with an unpredictable clinical course. […] Both sporadic DT and familial adenomatous polyposis (FAP)-associated DT are linked to constitutive activation of the Wnt signaling pathway with mutations in the -catenin oncogene CTNNB1 or the tumor suppressor gene APC, respectively. […] Due to the role -secretase plays in Notch signaling through cleavage of the Notch intracellular domain (with subsequent translocation to the nucleus to activate gene transcription), -secretase inhibitors (GSIs) have emerged as a potential treatment for DT. […] Dysregulation of the Wnt signaling pathway has been reported in a number of cancers, including breast cancer, colorectal carcinoma, hepatocellular carcinoma, pancreatic ductal adenocarcinoma, and non-small cell lung cancer.

• #5 Molecular pathogenesis of desmoid tumor and the role of γ-secretase inhibition

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9448813/

  As previously noted, the Wnt pathway plays a key role in DT pathogenesis, with constitutive activation caused by mutations in the -catenin oncogene CTNNB1 in most sporadic cases of DT, or a germline mutation in APC (which regulates -catenin degradation) in cases associated with FAP. […] Dysregulation of the Wnt pathway in DT leads to overexpression of Wnt genes involved in proliferation and fibrosis, such as ADAM12, Fap-1, WISP1, and SOX11, as well as genes such as VEGF, which is involved in the regulation of angiogenesis, and COX2, which initiates activation of growth factor receptors, such as the PDGFRs and (PDGF- and PDGF-). […] The overexpression of Notch pathway components in DT provides a rationale for development of pharmacologic agents targeting Notch. […] Inhibiting the proteolytic activity of -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. […] The GSIs are a drug class currently being evaluated in patients with progressive DT requiring systemic treatment. […] Dysregulation of the Wnt signaling pathway plays a key role in DT pathogenesis, however, as the result of crosstalk, Wnt dysregulation may lead to activation of the Notch pathway.

• #6 Desmoid Tumor – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459231/

  The etiology of desmoid tumor is unknown. Most desmoid tumors occur sporadically, and 85% have a mutation in the CTNNB1 encoding -catenin pathway. The 3 distinct mutations identified are 41A, 45F, and 45. Mutation 45F is associated with a high risk of recurrence. Desmoid tumor is seen at increased frequency in familial adenomatous polyposis (FAP), mostly abdominal FAP caused by a mutation in the APC gene. Desmoid tumor arising in FAP has a predilection for the prior surgical site, and previous surgery is a risk factor. In patients who are treated with prophylactic colectomy, desmoid tumor is the more important cause of morbidity and mortality than colon cancer. Desmoid tumor occurs with increased frequency in females during or after pregnancy, and anecdotal evidence suggests abdominal wall trauma and high estrogen states are the possible reasons. Pregnancy-associated desmoid tumor has overall better outcomes.

• #7 UNDERSTANDING DESMOID TUMORS: CAUSES AND TREATMENTS | Mya Care

  https://myacare.com/blog/understanding-desmoid-tumors-causes-and-treatments

  Desmoid tumors, also known as desmoid fibromatosis or aggressive fibromatosis, are rare, non-cancerous tumors that can occur in any part of the body. These tumors are characterized by their aggressive growth and tendency to invade surrounding tissues, making them difficult to treat. […] The exact cause of desmoid tumors is still unknown. Research suggests that they may be caused by mutations in the beta-catenin (-catenin) gene, also known as CTNNB1. This gene regulates cell growth and division. Mutations in this gene can raise the risk of acquiring a desmoid tumor. Mutation 45F is one of the most aggressive. This type maintains an average recurrence-free survival rate of 25% after 5 years. […] While the exact cause remains unknown, research demonstrates that mutations in the beta-catenin gene may result in their growth.

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

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

  These mutations prevented phosphorylation of threonine and serine residues, promoting -catenin accumulation at the nuclear level, with subsequent activation of the T-cell factor and transcription of target genes. […] Trisomy 8, in particular, is one of the most frequent events (about 30% of cases) and different studies have suggested that this kind of aberration may define a subgroup of patients with a high risk of recurrence.

• #9 Desmoid tumor pathophysiology – wikidoc

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

  Familial cases of desmoid (aka hereditary desmoid disease) are associated with germline mutations in the APC gene. […] Generally, desmoid tumors occur more frequently when mutations are in the 3′ end of the APC gene, specifically between codons 1445 and 1580. […] This leads to accumulation of beta catenin which binds to, and activate the transcription factor tcf-4. […] Sex hormones seem to play a role in activation of these mutations and signaling pathways leading to desmoids. […] Associated conditions include Turcot syndrome, Gardner syndrome, Familial adenomatous polyposis and estrogen therapy.

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

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

  Independent predictors of increased desmoid risk in one study were said to be (1) germline mutation distal to codon 1399, (2) any family history of gastrointestinal disease, and (3) a strong family history of desmoid tumors. […] Most sporadic desmoid tumors are linked with -catenin gene mutations. […] The relationship between certain extracolonic manifestations and sites of the APC mutation suggests specific roles of the APC protein in different tissues. […] FAP results from a germline mutation in the APC gene. Desmoid tumors are associated with a biallelic APC mutation in the affected tissue. […] This process is an example of the Knudsen „two hit” hypothesis, in which a tumor suppressor gene, such as APC, must be biallelically mutated in order for a specific type of tumor to occur.

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

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

  Desmoid tumors tend to infiltrate adjacent muscle bundles, frequently entrapping them and causing their degeneration. […] The myofibroblast is the cell considered to be responsible for the development of desmoid tumors. […] Desmoid tumors occur at a rate of 10-15% in patients with FAP, an autosomal inherited disease caused by germline mutations in the APC gene. […] Desmoid tumors show biallelic APC mutation, with one change usually occurring distal to the second beta-catenin binding/degradation repeat of the gene (3′ to codon 1399). […] The relationship between extracolonic manifestations and the site of the APC mutation suggests a specific role of the APC protein in different tissues. […] In desmoid tumors, one of the two mutations usually occurs distal to the second beta-catenin binding/degradation repeat of the gene (3′ to codon 1399).

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

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

  In genetically normal individuals, with normal germline genes, this necessitates a rare combination of events, such that at least 2 somatic mutations must occur in both alleles of a single tumor suppressor gene, in this case the APC gene. […] In FAP syndrome patients, one APC germline gene is already mutated in every cell in the body (barring a rare reverse somatic mutation in some cells), and, therefore, only one new somatic mutation is required in the opposite APC gene for the tumor to develop. […] CTNNB1 mutations are common in sporadic desmoid tumors. […] CTNNB1 mutations and APC mutations are mutually exclusive in this entity; accordingly, the presence of a somatic CTNNB1 mutation facilitates exclusion of a syndromic disorder.

• #12 Desmoid Tumors: A Clear Perspective or a Persisting Enigma? A Case Report and Review of Literature | Mishra | World Journal of Oncology

  https://www.wjon.org/index.php/wjon/article/view/961/718

  In genetically normal individuals, with normal germline genes, this necessitates a rare combination of events, such that at least two somatic mutations must occur in both alleles of a single tumor suppressor gene, in this case the APC gene. […] In FAP syndrome patients, one APC germline gene is already mutated in every cell in the body (barring a rare reverse somatic mutation in some cells), and, therefore, only one new somatic mutation is required in the opposite APC gene for the tumor to develop. […] The aforementioned features are in contrast to those in a fibrosarcoma, which has greater mitotic activity, an increased nuclear-to-cytoplasm ratio, greater vascularity, less collagen production, and a paucity of immune cells.

• #12 Desmoid Tumors: A Clear Perspective or a Persisting Enigma? A Case Report and Review of Literature | Mishra | World Journal of Oncology

  https://www.wjon.org/index.php/wjon/article/view/961/718

  The myofibroblast is the cell considered to be responsible for the development of desmoid tumors. […] Desmoid tumors occur at a rate of 10-15% in patients with FAP, an autosomal inherited disease caused by germline mutations in the APC gene. Sporadic forms have no hereditary background. […] Desmoid tumors show biallelic APC mutation, with one change usually occurring distal to the second beta-catenin binding/degradation repeat of the gene (3′ to codon 1399). […] The relationship between extracolonic manifestations and the site of the APC mutation suggests a specific role of the APC protein in different tissues. […] FAP results from a germline mutation in the APC gene. Desmoid tumors are associated with a biallelic APC mutation in the affected tissue. This usually results from a spontaneous somatic mutation in the unaffected APC gene of a single cell in a patient with the FAP syndrome.

• #13 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 overexpression of Notch pathway components in DT provides a rationale for development of pharmacologic agents targeting Notch. […] Results from preclinical and early-phase clinical trials suggest that inhibition of -secretase, which prevents the release of the NICD and its translocation to the nucleus, may be a promising therapeutic target for the treatment of DT.

• #14 Whole-Genome and Transcriptome Sequencing Identified NOTCH2 and HES1 as Potential Markers of Response to Imatinib in Desmoid Tumor (Aggressive Fibromatosis): A Phase II Trial Study

  https://www.e-crt.org/journal/view.php?number=3354

  Desmoid tumor, also known as aggressive fibromatosis, is well-characterized by abnormal Wnt/-catenin signaling. […] However, the molecular mechanism of why imatinib works remains unclear. Here, we describe potential roles of NOTCH2 and HES1 in clinical response to imatinib at genome and transcriptome levels. […] Our gene-wise recurrence model using 1,009 pan-cancer whole-genome data indicated that NOTCH2 regulatory mutations are associated with the response of desmoid tumors to imatinib. […] We discovered that HES1, a well-known downstream target of Notch signaling pathway, is directly associated with imatinib sensitivity. […] Our analyses suggest HES1 overexpression, potentially regulated by NOTCH2, can serve as a predictor of the clinical response to imatinib in desmoid tumor patients.

• #15 Desmoid Tumours — DermNet

  https://dermnetnz.org/topics/desmoid-tumours

  Desmoid tumours are locally infiltrative and destructive, with no risk of metastasis. […] Pathogenesis is multifactorial, although genetics is thought to play a central role, including mutations in the Wnt/APC/beta-catenin pathway. […] Broadly, desmoid tumours can be classified into two categories based on their genetics: Sporadic associated with mutations in the CTNNB1 gene which codes for beta-catenin; Associated with APC mutations tumours occurring as part of syndromic conditions such as in patients with a family history of desmoid tumours, known FAP, or Gardner syndrome. […] Other proposed risk factors for desmoid tumour development include trauma, recent surgery, and high oestrogen states. […] Intra-abdominal tumours carry the highest risk of morbidity, and patients with syndromic conditions such as familial adenomatous polyposis (FAP) are more likely to have more invasive, symptomatic tumours.

• #16 Appendicular desmoid tumour, an uncommon cause for abdominal pain | Gynecological Surgery | Full Text

  https://gynecolsurg.springeropen.com/articles/10.1007/s10397-009-0467-5

  Desmoid tumours are rare non-encapsulated, locally invasive soft tissue tumours of fibrous origin that lack the ability to metastasise but are very notorious for recurrence; they are also referred to as fibromatoses. […] The risk factor most strongly associated with desmoid tumours is familial adenomatous polyposis (FAP). […] Well-recognised risk factors are trauma, female sex, oestrogens, pregnancy, trisomy 20 and 8 and the position of the APC germline mutation. […] Estrogens have been implicated in the pathogenesis of sporadic desmoid tumours because females have a higher incidence, particularly those of childbearing age. […] The growth of tumours in this group has been noted to be significantly faster than in males or in post- or pre-menopausal women, and one of these studies directly correlated the rate of growth of tumours to the level of endogenous estrogens. […] The optimal treatment has not yet been established and, in many cases, a multidisciplinary approach including surgery, chemotherapy and radiation therapy has been employed. […] Given the unpredictable behaviour of desmoid tumours, it is wise to establish a follow-up plan.

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

  https://radiopaedia.org/articles/desmoid-tumour?lang=us

  Desmoid tumors are benign, non-inflammatory fibroblastic tumors with a tendency for local invasion and recurrence post resection. […] Their exact etiology remains uncertain, although they are frequently associated with previous trauma or surgical incision. On the molecular level, desmoids are characterized by mutations in the -catenin gene, CTNNB1, or the adenomatous polyposis coli (APC) gene.

• #18 The Facts About Desmoid Tumors –

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

  The pathogenesis of desmoid tumors is thought to be linked to dysregulated wound healing after trauma, such as childbirth, injury, or invasive surgery. […] Growth factors released during wound healing that promote beta-catenin activation may lead to recurrence of desmoid tumors.

• #19

  https://link.springer.com/article/10.1007/s10238-022-00849-6

  The pathogenesis of DTs is not entirely clear: aside genetic predisposition, development of desmoids is most commonly associated with pregnancy, hormonal exposure and physical factors such as trauma including previous motor vehicle accidents and/or surgery. […] While it is difficult to prove the relationship between trauma and development of this disease, the incidence of trauma preceding the development of DT is greater than that reported for other soft tissue tumors. […] It has been proposed that this association may in fact be the result of aberrant proliferation after a traumatic insult, with an underlying over-activation of the molecular signaling pathway for normal wound healing. […] A possible derivation of DT was also described from multipotent mesenchymal stromal cells (MSCs) acquiring mutations in the APC gene with increase of -catenin levels during a defective wound healing; the presence of MSCs has been shown within human DT, which could explain the correlation between surgery and DT localization. […] Considering that sporadic DTs with the S45F mutation have a greater tendency for local recurrence, with increasing implementation of watchful-waiting for DT management, it will be important to determine whether mutation type predicts outcome for these patients.

• #20 Postoperative recurrence of desmoid tumors: clinical and pathological perspectives | World Journal of Surgical Oncology | Full Text

  https://wjso.biomedcentral.com/articles/10.1186/s12957-015-0450-8

  The clinical features and the pathological changes of desmoid tumors were studied to point out the key factors affecting the recurrence. […] The invasion of major vessels and nerves and quality of surgical margins are the key factors for the high postoperative recurrence rate. […] Noticeably, in order to increase the quality of the resection margins and decrease the recurrence rate, it is of importance to study the invasion mechanism of desmoid tumors into the surrounding structures. […] The invasion of the major vessels and the quality of the surgical margins were statistically significant. […] The quality of surgical margins and the invasion of major vessels and nerves are the two independent risk factors causing the recurrence based on our statistical analysis of the clinical documents. […] Our data showed that either the positive surgical margin or the tumors invading major vessels and nerves were able to cause the recurrence.

• #21 Nirogacestat’s Mechanism of Action to Treat Desmoid Tumors | CheckRare

  https://checkrare.com/nirogacestats-mechanism-of-action-to-treat-desmoid-tumors/

  Desmoid tumors are benign tumors that can grow into surrounding healthy tissues, including joints, muscle, and viscera. […] As Smith explains in this video, nirogacestat is an oral, selective, small molecule, gamma-secretase inhibitor. Gamma secretase cleaves multiple transmembrane protein complexes, including Notch, which may contribute to desmoid tumor growth. The drug is currently under investigation in a Phase 3 clinical trial involving adults and a Phase 2 clinical trial involving children with Desmoid tumors.

• #22 FDA Approves Nirogacestat, the First-Ever Targeted Therapy for Desmoid Tumors | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/news/nirogacestat-new-desmoid-tumor-treatment-improves-outcomes-people-sarcoma

  Update: On November 27, 2023, the U.S. Food and Drug Administration approved nirogacestat (Ogsiveo) for adults with desmoid tumors that cannot be treated with surgery alone. The approval was based on research led by investigators at Memorial Sloan Kettering Cancer Center. Nirogacestat is the first targeted therapy ever to be approved for desmoid tumors. […] Nirogacestat, which blocks a protein called Notch, is a new type of targeted drug called a gamma-secretase inhibitor. The paper reported that 41% of patients tumors significantly shrank after they took nirogacestat. […] Dr. Gounder has led a number of clinical trials studying drugs that target the defective proteins that cause these tumors to grow. […] Another side effect of nirogacestat was that in many female patients it caused temporary ovarian dysfunction, which was reversible in most patients. Because of this, the trial also included follow-up research to study reproductive function. This tumor affects many people who may want to have children, so this is an important part of finding better treatments, Dr. Gounder adds.

• #23 Nirogacestat Shrinks Desmoid Tumors – NCI

  https://www.cancer.gov/news-events/cancer-currents-blog/2023/nirogacestat-shrinks-desmoid-tumors

  Nirogacestat blocks the activity of an enzyme called gamma secretase, which is involved in driving desmoid tumor growth. […] Nirogacestat works by blocking the activity of an enzyme called gamma secretase, which helps activate a signaling protein called Notch. Researchers have hypothesized that desmoid tumors produce high amounts of Notch protein, which is thought to drive their growth. […] Nirogacestat was initially developed to treat Alzheimers disease, but the drug proved to be ineffective against that disease. However, in clinical trials, nirogacestat showed signs of activity in people with desmoid tumors. […] The success of that study led SpringWorks Therapeutics to launch a phase 3 clinical trial of nirogacestat for adults with desmoid tumors.

• #24 Desmoid Tumors: An In-Depth Review

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

  Desmoid tumors are characterized by specific genetic alterations, most notably mutations in the Adenomatous Polyposis Coli (APC) gene or CTNNB1 (-catenin) gene, which are involved in the Wnt signaling pathway. […] These genetic mutations lead to the stabilization of -catenin, resulting in the aberrant activation of transcription factors that drive tumorigenesis. […] In sporadic desmoid tumors, -catenin mutations are more commonly observed, indicating a different molecular pathogenesis. […] Hormonal factors also play a role in the development and progression of desmoid tumors. […] There is evidence suggesting that estrogen and progesterone may influence tumor growth, as desmoid tumors often present or grow during pregnancy or with the use of oral contraceptives. […] However, the exact mechanisms by which hormones impact desmoid tumors remain an area of ongoing research. […] Recent advances have introduced targeted therapies that specifically address the molecular pathways involved in desmoid tumor pathogenesis. […] Drugs such as sorafenib, a tyrosine kinase inhibitor, and other agents targeting the Wnt/-catenin pathway are being investigated in clinical trials.

• #25 Role of the Interventional Radiologist in the Treatment of Desmoid Tumors

  https://www.mdpi.com/2075-1729/13/3/645

  Desmoid tumors, also called aggressive fibromatosis, are rare, locally invasive tumors with no potential for metastasis. However, they are locally aggressive, demonstrating proliferation of myofibroblasts, and can occur anywhere in the body. […] The sporadic form of desmoid tumor presents with mutations in the CTNNB1 gene, part of the Wnt signaling pathway. […] Unlike sporadic desmoid tumors, familial adenomatous polyposis associated desmoid tumors result from mutations in both the wild-type CTNNB1 and adenomatous polyposis coli (APC) genes. […] Classically, desmoid tumors were managed aggressively with medical, surgical, and radiation therapy; however, it can be highly morbid and has a 30–50% recurrence rate even when negative margins are achieved. […] A number of different systemic therapy classes and specific agents, such as hormonal agents (tamoxifen, toremifiene, aromatase inhibitors), anti-inflammatory drugs (sulindac, celecoxib), cytotoxic agents (doxorubicin, vinorelbine, vinblastine, methotrexate), and molecular targeted therapies (sorafenib, pazopanib, imatinib, nirogascetat) have been utilized.

• #26 Role of the Interventional Radiologist in the Treatment of Desmoid Tumors

  https://www.mdpi.com/2075-1729/13/3/645

  Image guided, percutaneous chemical and thermal ablation techniques have been used to successfully treat desmoid tumors through minimally invasive means. […] Percutaneous ablation has been used to treat intra-abdominal, abdominal wall, and extra-abdominal desmoid tumors. […] The first reported use of image guided, percutaneous cryoablation in the literature was by Kujak et al., who performed ablation on five patients with extra abdominal desmoid tumors demonstrating local control in four out of five (80%) patients with small and moderately sized tumors. […] To date, the evaluation of nearly all minimally invasive based interventions for desmoid tumors have included small, single institution cohort studies.

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