Materiały źródłowe

• #1 Desmoid Tumors: A Comprehensive Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10427533/

  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. […] Patients with DT experience a high symptom burden: up to 63% of patients experience chronic pain, which leads to sleep disturbance (73% of cases), irritability (46% of cases), and anxiety/depression (15% of cases). Frequently mentioned symptoms are pain, limited function and mobility, fatigue, muscle weakness, and swelling around the tumor. Overall, quality of life in patients with DT is lower than in healthy controls. […] The substantial burden of illness of DT is related to difficulties in timely and accurate diagnosis, high symptom burden (pain and functional limitations), and decreased quality of life.

• #2

  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. […] Patients with DT experience a high symptom burden: up to 63% of patients experience chronic pain, which leads to sleep disturbance (73% of cases), irritability (46% of cases), and anxiety/depression (15% of cases). Frequently mentioned symptoms are pain, limited function and mobility, fatigue, muscle weakness, and swelling around the tumor. Overall, quality of life in patients with DT is lower than in healthy controls. […] The substantial burden of illness of DT is related to difficulties in timely and accurate diagnosis, high symptom burden (pain and functional limitations), and decreased quality of life.

• #3 Desmoid tumors: 8 things to know | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/desmoid-tumors–8-things-to-know.h00-159622590.html

  Desmoid tumors are slow-growing, locally invasive tumors that develop in the body’s connective tissues. […] Desmoid tumors can grow to impressive sizes that cause serious symptoms and make people miserable. […] Most desmoid tumors arise in the extremities, such as your arms and legs, but sometimes they develop in the abdomen, too. So, the symptoms depend on the tumors size and location. […] For tumors in the abdomen, symptoms can range from mild pain or a vague sense of discomfort or heaviness on one side to severe pain and a palpable lump. In extreme scenarios, patients may develop a bowel obstruction.

• #4 Desmoid tumors | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/desmoid-tumors?content_id=CON-20213624

  Desmoid tumor symptoms differ based on where the tumors occur. Desmoid tumors most often happen in the abdomen, arms and legs. But they can form anywhere in the body. […] In general, signs and symptoms include: A mass or area of swelling, Pain, Loss of function in the affected area, Cramping and nausea, when desmoid tumors occur in the abdomen. […] If your desmoid tumor causes no signs or symptoms, your doctor may recommend monitoring the tumor to see if it grows. You may undergo imaging tests every few months. Some tumors never grow and may never require treatment. Some tumors may shrink on their own without any treatment. […] If your desmoid tumor causes signs and symptoms, your doctor may recommend an operation to remove the entire tumor and a small margin of healthy tissue that surrounds it. But sometimes the tumor grows to involve nearby structures and can’t be completely removed. In these cases, surgeons may remove as much of the tumor as possible. […] Chemotherapy uses strong drugs to kill tumor cells. Your doctor may recommend chemotherapy if your desmoid tumor is growing quickly and surgery isn’t an option.

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

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

  Desmoid tumors often appear as infiltrative, usually well-differentiated, firm overgrowths of fibrous tissue, and they are locally aggressive. The synonym aggressive fibromatosis describes the marked cellularity and aggressive local behavior. This course and the tendency for recurrence make the treatment of these relatively rare fibrous tumors challenging. […] Although desmoid tumors can arise in any skeletal muscle, they most commonly develop in the anterior abdominal wall and shoulder girdle. […] A history of trauma (often surgical) to the site of the desmoid tumor is elicited in 1 in 4 cases. […] Primary surgery with negative surgical margins is the most successful primary treatment modality for desmoid tumors. Positive margins after surgery reflect a high risk for recurrence. […] Despite their benign histologic appearance and negligible metastatic potential, the tendency of desmoid tumors to cause local infiltration is significant in terms of (1) deformity, morbidity, and mortality resulting from pressure effects and (2) potential obstruction of vital structures and organs.

• #6 Desmoid Tumors: The Tumor That Isn’t a Cancer

  https://www.onclive.com/view/desmoid-tumors-the-tumor-that-isn-t-a-cancer

  Desmoid tumors are mesenchymal neoplasms that are locally invasive but do not metastasize. They are rarely life threatening, but the condition can be destructive and lead to severe pain and functional impairment. Desmoid tumors are typically diagnosed in young adults—the incidence is greatest in those aged 35 to 40 years. Women in this age group are particularly susceptible. […] […] The disease can be difficult to identify, and up to one-third of patients are misdiagnosed initially. Desmoid tumors can appear in any part of the body in different types of connective tissues, including muscle, fascia, and aponeurosis. Tumors appearing in the abdominal wall and extra-abdominal locations may present as painless mass. Larger lesions and those adjacent to neurovascular structures may be associated with pain and functional impairment. […]

• #7 Desmoid Tumor – NCI

  https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor

  Desmoid tumors grow from the connective tissue in your body. Desmoid tumors are locally aggressive tumors. While the cells of the desmoid tumor do not travel to other parts of the body like cancer can, they can invade nearby tissue and are often very painful. Desmoid tumors can grow slowly or very quickly. The quicker they grow the more serious they are. […] Some people with desmoid tumors have symptoms, while others do not. Symptoms can include pain, swelling in the area of the tumor, sleep loss, or difficulty moving. […] Desmoid tumors can be hard to predict. They can shrink and go away on their own, they can remain the same size, or they can grow quickly. […] Most desmoid tumors do not impact lifespan but they are very difficult to get rid of and can be painful to live with. Desmoid tumors growing in the abdomen can cause problems, such as blocking your intestines. It is important that your doctor monitor the growth of these tumors carefully.

• #8 Diagnosis – The Desmoid Tumor Research Foundation

  https://dtrf.org/about-desmoid-tumors/diagnosis/

  Symptoms can differ based on where the desmoid tumor is located in the body. Some patients will be able to actually feel a palpable lump or see swelling. By contrast, some patients may have no symptoms at all and no visible physical manifestation of the tumor. Below are some other common signs and symptoms associated with a desmoid tumor depending on location: […] Pain […] Swelling […] Visible deformities […] Cramping and nausea for abdominal tumors […] Impact on mobility […] Impact on bodily function such as urinary or bowel function, breathing, or swallowing […] Fatigue.

• #9 Desmoid tumors – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/desmoid-tumors/symptoms-causes/syc-20355083

  Desmoid tumor symptoms differ based on where the tumors occur. Desmoid tumors most often happen in the abdomen, arms and legs. But they can form anywhere in the body. […] In general, signs and symptoms include: A mass or area of swelling, Pain, Loss of function in the affected area, Cramping and nausea, when desmoid tumors occur in the abdomen. […] Make an appointment with your doctor if you have any persistent signs or symptoms that worry you. […] Some desmoid tumors are slow growing and don’t require immediate treatment. Others grow quickly and are treated with surgery, radiation therapy, chemotherapy or other drugs. […] But they can be very aggressive, acting more like cancers and growing into nearby structures and organs.

• #10 Desmoid Tumors: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/22075-desmoid-tumors

  Desmoid tumors dont always cause symptoms. When they do, symptoms may include: […] Pain, soreness or a tingling sensation from a tumor pressing on nearby nerves, organs or blood vessels. […] A lump or swollen area, usually in your arms, legs and belly. […] Trouble moving your arm or leg. […] Nausea and vomiting from a tumor in your belly. […] Desmoid tumors can come back after treatment. Not every lump or bump is a desmoid tumor, but you should talk to a healthcare provider if you have a new lump or bump thats larger than 2 inches, keeps getting bigger or hurts. […] Fast-growing desmoid tumors can be painful, make you feel nauseated or make it hard for you to move your arms or legs.

• #11 Desmoid Tumors | Huntsman Cancer Institute | University of Utah Health

  https://healthcare.utah.edu/huntsmancancerinstitute/sarcoma/desmoid-tumors

  Desmoid tumors can cause pain and can be hard to remove. […] Desmoid tumors can shrink and go away on their own, they can remain the same size, or they can grow quickly. […] These are common symptoms of desmoid tumors: Pain and swelling in the area of the tumor, Sleep loss, Difficulty moving arms, hands, or legs, Tingling feeling.

• #12 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 can grow quite large and lead to disability and severe (very bad) pain. In rare cases, they invade vital organs and lead to severe problems, including death. […] The most common sign of a desmoid tumor is a lump in the arms, legs, or abdomen (belly). Sometimes the lump is painful. There also can be swelling in the area that has the tumor. […] If the tumor is in the abdomen, you may have cramps or nausea (feeling like throwing up).

• #13 Desmoid Tumors: A Comprehensive Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10427533/

  Among all symptoms experienced by patients with DT, pain is the most debilitating and has a large impact on the patients QOL. In the survey conducted among the active members of the French patient advocacy group (SOS Desmode), pain was present in 63% of cases. […] The presence of pain was associated with lower performance status and functional impairment.

• #14

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

  Patients can experience compromised QOL due to diagnostic challenges and the high clinical burden of DT, including severe pain, impaired physical function and mobility, and high recurrence rates. This can limit their everyday activities and lead to deterioration in physical, social, and emotional functioning. […] Among all symptoms experienced by patients with DT, pain is the most debilitating and has a large impact on the patients QOL. […] The presence of pain was associated with lower performance status and functional impairment. The pain severity and associated burden of disease can lead to anxiety and depression in many patients.

• #14

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

  The morbidity and mortality associated with DT largely depend on the tumor location, as this determines the adjacent structures that the DT will infiltrate or compress. Depending on their location, DT tend to infiltrate adjacent organs, extend along fascial planes, compress blood vessels and nerves, erode bones, or obstruct organs such as the bowel. Muscle, nerve, and vessel involvement may cause debilitating symptoms, including pain, restricted mobility, or deformity. […] The clinical course of the disease varies, and data suggest that an initial tumor growth phase is followed by a long period of growth arrest and even regression. […] Overall, the clinical burden of patients with DT is considerable because of the pain and physical limitations resulting from the chronic infiltrative nature of this disease, in addition to the functional impacts resulting from surgical interventions.

• #15 About Desmoid Tumors | OGSIVEO® (nirogacestat) Patient Site Up to 1650 people2 to 3 timesBetween 20 & 44 years of ageUp to 77% of desmoid tumors may recur

  https://www.ogsiveo.com/about-desmoid-tumors/

  Desmoid tumors can grow aggressively and affect nearby tissues and organs. They can often return, even after surgery or other treatments. […] Desmoid tumor symptoms can vary based on where the tumor is located. For example: A tumor within the abdominal area may cause digestive system blockages, pain, malaise, abdominal swelling, weight loss, or muscle wasting. Tumors in the arms or legs may cause limited movement, deformity, pain, tiredness, weakness or muscle problems. […] It’s important to know if your tumor is progressing, or getting worse. Signs of progression may include: Changes in imaging tests. The tumor may appear bigger on a CT scan or MRI. New or changing symptoms. You may experience new symptoms, or your existing symptoms may worsen.

• #16 Desmoid Tumors: Symptoms, Causes, Treatment

  https://www.webmd.com/colorectal-cancer/desmoid-tumors-facts?page=4

  Signs of a desmoid tumor depend on where it is. If it’s close to the surface of your skin, you may have a painless or slightly painful lump. […] If it’s in your abdomen, it may be more aggressive. It can press against blood vessels and nerves and cause pain, a limp, or problems using your legs, feet, arms, or hands. It also can block your colon or grow into nearby tissues. When this happens, you may have severe pain, bleeding from your rectum, and other health problems.

• #17 9 Desmoid Tumor Symptoms To Watch For | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/desmoid-tumor-symptoms-to-watch-for

  In many cases, desmoid tumors form in tissues or organs within the abdomen. This can cause problems with the digestive system and may lead to significant weight loss. This may happen suddenly and over a short period of time. […] If the pain caused by a desmoid tumor becomes chronic (long-lasting), it can be hard to ignore. Dealing with constant pain may make it harder to get a good nights rest. Insomnia or trouble falling and staying asleep is common for people living with desmoid tumors. […] Desmoid tumors might form in the extremities these can be the arms, legs, or even the feet. Tumors in the extremities can damage the bones, muscles, or nerves in your limbs. This may make it harder to move your arms and legs properly and could even cause you to walk with a limp. […] If a desmoid tumor gets big enough, it can start to press on the nerves near the location of the tumor. Compression of the nerves can lead to a tingling sensation described as pins and needles, similar to when your foot falls asleep.

• #17 9 Desmoid Tumor Symptoms To Watch For | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/desmoid-tumor-symptoms-to-watch-for

  Desmoid tumors can cause significant pain. Unfortunately, getting relief can be difficult you may find that it doesnt go away with typical painkillers. If the pain becomes intense, it may negatively affect your daily life. Determining whether pain is caused by a desmoid tumor can be difficult, because the pain can occur in different parts of the body depending on the tumor’s location. […] Desmoid tumors may cause a part of your body to become swollen most likely near where the tumor is located. Depending on the tumors location, you may be able to see the swelling or even feel a lump under your skin. You may also notice the area becoming more swollen over time. […] Like many health conditions, desmoid tumors can cause you to feel fatigued (tired). You might also have a general feeling of being sick or unwell. This may be referred to as malaise and is especially linked to intra-abdominal tumors.

• #18 The Facts About Desmoid Tumors –

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

  Symptomatology related to desmoid tumors varies based on the location of tumor presentation. […] Compression may cause pain, cachexia, malaise, abdominal distention, or obstruction of the intestines or ureters. […] Large tumors may cause tissue stretching, blood vessel compression, and bowel or bladder displacement. […] Limited mobility, pain, muscle stiffness, or deformity can occur in the lower extremities. […] Dyspnea, dysphagia, pleural invasion, rib or spinal involvement, bone erosion, and pain can be symptoms associated with chest wall tumors. […] Pain, neurologic deficit, proximity to vital structures, including mortality risk from vascular or airway restriction can occur with head and neck tumors.

• #19 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. […] Despite their benign nature, they can be extremely disabling and sometimes life-threatening, causing severe pain and functional limitations. […] Patients with a desmoid tumour may be asymptomatic, or have severely limited function due to chronic pain, deformity, gastrointestinal symptoms or complications when located intra-abdominally, and psychological problems, contributing to a general decrease in the quality of life. […] These tumours are characterized by an extremely variable and unpredictable clinical course, so much so as to be hardly considered a single condition. […] Therefore, at least two different types of desmoid tumour can be described: those with indolent behaviour and an asymptomatic course, characterized by a natural tendency to regress spontaneously or remain stable, and those which are locally aggressive with extensive complications including the infiltration of neurovascular structures and impairment of vital organs, leading to life-threatening conditions. […] Progressive symptoms and/or enlargement of the tumour are the main indications for discontinuing surveillance in favour of active treatment. […] Quality of life and overall wellbeing of the patient should be the main goals during the entire decision-making process for treatment selection.

• #20 Cryotherapy in the Treatment of Extra-Abdominal Desmoid Tumors—A Review

  https://www.mdpi.com/1718-7729/32/3/137

  Desmoid tumors (DTs) are rare, connective tissue neoplasms which have no distant metastatic potential, but can be both locally aggressive and invasive. DTs pose an ongoing dilemma to healthcare providers in their management due to their variable behavior and their impact on both quality of life and activities of daily living for a proportion of patients. The variable behavior of DTs makes management complex; approximately 20–28% will regress spontaneously, 32–60% will remain stable, and 20–40% will progress. Treatment is generally initiated for both growth or progression and symptoms. Symptomatology remains one of the main indications for DT treatment with cryoablation. Early experiences suggest that the treatment can be effective in relieving pain and reducing tumor size. One of the first systematic reviews assessing the role of cryoablation in extra-abdominal desmoid management, with 146 patients across studies, showed that complete pain relief was reported in 40% to 66.7% of patients. Another systematic review involving 214 patients across nine studies showed that 37.5% to 96.9% of the patients reported having experienced partial or complete symptom relief following cryoablation. Improvement in both symptom intensity and effect on activities of daily living occurs gradually, typically over a period of approximately 6 months following the procedure. While pain remains the main symptom assessed in the literature, other symptoms reported to improve following ablative therapy include motor dysfunction, abdominal distension, and abdominal pressure. The recurrence of symptoms following treatment has been reported to occur in approximately 37.5% of patients, with a median time for symptom recurrence of around 10 months.

• #21 Desmoid Tumors: A Comprehensive Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10427533/

  The clinical course of the disease varies, and data suggest that an initial tumor growth phase is followed by a long period of growth arrest and even regression. According to estimates, approximately 10-28% of DT will resolve spontaneously without treatment (22% for extra-abdominal tumors to 28% for abdominal tumors). […] Overall, the clinical burden of patients with DT is considerable because of the pain and physical limitations resulting from the chronic infiltrative nature of this disease, in addition to the functional impacts resulting from surgical interventions. […] Patients can experience compromised QOL due to diagnostic challenges and the high clinical burden of DT, including severe pain, impaired physical function and mobility, and high recurrence rates. This can limit their everyday activities and lead to deterioration in physical, social, and emotional functioning.

• #22 The Facts About Desmoid Tumors –

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

  Desmoid tumors are locally aggressive, potentially morbid tumors of the soft tissues, with a tendency to infiltrate surrounding structures. […] Sometimes referred to as aggressive fibromatosis or desmoid fibromatosis, these mesenchymal tumors can be serious, debilitating and, in rare cases when vital organs are impacted, they can be life-threatening. […] 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. […] Desmoid tumor recurrence risk remains high even with clean margins: various independent studies report desmoid tumor recurrence rates after complete surgical resection of 15% to 30%. Some studies suggest that the majority of tumors recur within 5 years. […] Progression can be symptomatic and/or radiographic. Symptomatic progression may precede radiographic progression.

• #23 Early results in the novel use of contrast-enhanced susceptibility-weighted imaging in the assessment of response and progression in desmoid fibromatosis: A pilot study in a specialized cancer institution

  https://accscience.com/journal/TD/2/3/10.36922/td.1414

  Routine radiologic reporting (RRR) often considers progressive desmoid tumors to have a higher proportion of T2-hyperintense and T1-shortened-enhancing components, while responsive or mature collagenized tumors demonstrate a higher proportion of T2-hypointense-non-enhancing components. […] We observed two cases of progression according to the T2-WI-based Response Evaluation Criteria in Solid Tumors standard (RECIST). […] RRR assessed both cases as progression; […] Out of the eight patients classified as having stable disease by T2-WI-based-RECIST, four discrepant progressions were determined: three patients showed an increase greater than 25% of T2-WI-based-volume, and two patients showed an increase greater than 25% of CE-SWI-based-volume. […] Notably, expected first-order response/progression-related changes in 10th-percentile, 90th-percentile, mean, and skewness were present in 90% of cases.

• #24 Desmoid Tumor Life Expectancy

  https://www.healthline.com/health/cancer/desmoid-tumor-life-expectancy

  The primary factors that influence life expectancy for people with desmoid tumors include: Where the tumor is located. Intra-abdominal desmoid tumors are considered more serious and often lead to lower life expectancy. Desmoid tumors in the head and neck are also considered more serious and potentially life threatening. […] One 2012 study found that the 5-year survival rate of people with stage IV intra-abdominal desmoid tumors with any of the above factors was 53 percent. […] Life expectancy for people with this type of tumor is high. However, certain factors can influence each persons individual life expectancy. Tumor size, stage, location, and complications may change the outlook. The treatment you receive will also have an impact on your outlook. […] Desmoid tumors also often recur, or come back, after surgery, even if the surgeon removes the tumor entirely. The Desmoid Tumor Research Foundation suggests that 25 to 60 percent of people who have surgery to remove a desmoid tumor will see a recurrence at or near the original site. […] According to Orphanet, altogether, 70 percent of people with a desmoid tumor will see a recurrence.

• #25 The Facts About Desmoid Tumors –

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

  In fact, evidence of pain can be a prognostic indicator of progression and can be associated with worse outcomes. […] Symptoms impairing or threatening in function, tumor growth documented on imaging (e.g., MRI or CT) are indications for initiating treatment. […] Threatening to function or quality of life, life-threatening, and persistent tumor growth are also considerations for treatment initiation. […] Patients may experience severe pain, limited function and mobility, and compromised quality of life. […] As desmoid tumors locally invade the bodies of patients, the physical toll can feel almost impossible to escape, and is often magnified by psychological, emotional, social, and even professional burdens. […] In many patients, pain severity and burden of disease can lead to anxiety and depression.

• #26 Prospective Development of a Patient-Reported Outcomes Instrument for Desmoid Tumors or Aggressive Fibromatosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6980244/

  Desmoid tumors (or aggressive fibromatosis) are locally infiltrative connective-tissue tumors that can arise in any anatomic location; they can be asymptomatic, or they can result in pain, deformity, swelling, and loss of mobility and/or threaten visceral organs with bowel perforation, hydronephrosis, neurovascular damage, and other complications. […] Depending on its location, patients can present with pain, a loss of range of movement or immobility, bowel obstructions and/or perforations, hydronephrosis, and a host of other symptoms. […] A total of 33 unique symptoms were identified. Those most frequently mentioned included disfigurement/altered appearance, nerve pain, decreased range of motion, fatigue, and nausea. […] In addition to pain and functional impairments, health care workers should also address fatigue, insomnia, anxiety, fear, and body dysmorphia.

• #27 FAQs – The Desmoid Tumor Research Foundation

  https://dtrf.org/about-desmoid-tumors/faqs/

  Symptoms from desmoid tumors vary greatly depending on the tumor location but can include pain, swelling, visible deformities, impact on mobility, and pressure on nearby organs affecting bodily functions such as urinary or bowel function, breathing, or swallowing. By contrast, some patients may have no symptoms at all and no visible physical manifestation of the tumor. […] Although desmoid tumors do not metastasize, they can be locally aggressive and severely damage surrounding tissues and vital structures as they grow. They also have a high rate of regrowth or “recurrence” after surgery, and should be monitored long-term in most cases. […] While most desmoid tumors are not life-threatening, they can still be locally aggressive and can severely damage surrounding tissues and vital structures as they grow. This can cause pain and impair mobility and function. Proper monitoring of these tumors by a sarcoma specialist is important as symptoms and tumor growth rates can change over time. […] Desmoid tumors can become life-threatening when they compress or involve vital organs such as intestines, kidneys, lungs, blood vessels, nerves, etc., which is why it’s important to see a sarcoma expert experienced in desmoid tumors to better understand your particular situation.

• #28 Balancing Symptoms and Current Therapies in Desmoid Tumor Management

  https://www.targetedonc.com/view/balancing-symptoms-and-current-therapies-in-desmoid-tumor-management

  Nam Quoc Bui, MD: I think this is a reasonable approach for patients with asymptomatic or minimally symptomatic desmoids or something that, if it grows, it’s not going to cause immediate end organ dysfunction or complication. […] We also have intraabdominal and retroperitoneal [tumors]. Those tumors can get a bit tricky, because patients can get symptoms related to bowel obstructions or ureter obstructions. There’s lots of stuff in the abdomen that can get obstructed with these desmoid tumors. I have patients who have chronic small bowel obstruction from their desmoids, and they have to be on total parenteral nutrition. It’s very difficult to manage. Even though they’re not that big, they can be in bad locations. […] For patients who need active therapy for progressive, morbid, or symptomatic disease, if it’s intraabdominal or retroperitoneal, options include systemic therapy and surgery if resectable. Options for all other sites include…systemic therapy, ablation/embolizationdefinitive radiationthat has been used before in desmoid tumors, and we use it for select patients.

• #29 Orphanet: Desmoid tumor

  https://www.orpha.net/en/disease/detail/873

  A desmoid tumor (DT) is a benign, locally invasive soft tissue tumor associated with a high recurrence rate but with no metastatic potential. […] Depending on the location of the tumor, symptoms may include pain, fever, and functional impairment or loss of function of the organ involved. […] Local recurrence occurs in around 70 % of cases. Prognosis depends on the type of tumor. Life expectancy is normal for abdominal and extra-abdominal tumors. However, it is lower in cases of intra-abdominal DTs due to complications such as intestinal obstruction, hydronephrosis or sepsis.

• #30 Desmoid Tumor – Brigham and Women’s Hospital

  https://www.brighamandwomens.org/surgery/surgical-oncology/desmoid-tumor

  Desmoid tumors most commonly appear in young women during or after pregnancy. […] A sarcoma may appear as a painless lump under the skin. Sarcomas that begin in the abdomen may not cause signs or symptoms until they get very big. As the sarcoma grows bigger and presses on nearby organs, nerves, muscles, or blood vessels, signs and symptoms may include: Pain, Trouble breathing. […] Other conditions may cause the same signs and symptoms. Check with your doctor if you have any of these problems.

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

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

  Local desmoid tumor recurrence rates are reported to be as high as 70%. A positive surgical margin is a significant risk factor for recurrence. […] Intra-abdominal desmoid tumors may kill patients with familial adenomatous polyposis. […] Five-year survival rates of such patients with stage I, II, III, and IV intra-abdominal desmoid tumors were found to be 95%, 100%, 89%, and 76%, respectively. The 5-year survival rate of stage IV patients with severe pain/narcotic dependency, tumor size larger than 10 cm, and need for total parenteral nutrition was only 53%.

• #32 Life Expectancy With Desmoid Tumors | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/life-expectancy-with-desmoid-tumors

  The researchers also looked at other factors that affect life expectancy. Participants who had severe pain, a large tumor, and needed nutrition from an intravenous (IV) injection had a five-year survival rate of 53 percent. […] If left untreated, the median life expectancy with FAP is 42 years. This means that half of people with FAP live at least 42 years. If you’re living with FAP, it’s important to have regular checkups. Be sure to tell your doctor if you’re experiencing any desmoid tumor symptoms. Your doctor can run tests if they think you may have a desmoid tumor. […] Some desmoid tumors return after treatment or grow larger. If they return to the same spot they were in before, it’s known as a local recurrence. Your doctor may perform more imaging tests to check for any new tumor growth. […] If your desmoid tumor is progressing or has returned, your doctor may suggest trying another treatment option. Researchers continue looking for new ways to treat desmoid tumors.

• #33 Desmoid tumors: Treatment – UpToDate

  https://www.uptodate.com/contents/desmoid-tumors-treatment

  Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis, and fibrosarcoma grade I of the desmoid type) are locally aggressive tumors with no known potential for metastasis or dedifferentiation. However, tumor invasion into vital structures and/or organs can result in substantial morbidity and may be fatal, especially in the case of intra-abdominal desmoids, particularly those arising in patients with familial adenomatous polyposis (FAP). […] Desmoid tumors can follow an unpredictable clinical course, with some tumors undergoing regression in the absence of therapy. For many patients, especially those with asymptomatic or minimally symptomatic tumors, an initial strategy of active surveillance is the preferred approach. However, if a desmoid tumor progresses on serial imaging or is associated with significant symptoms that are difficult to manage, a multidisciplinary team should determine the best individualized approach. […] The development of symptoms in a previously asymptomatic or minimally symptomatic patient should prompt earlier evaluation with imaging and initiation of therapy.

• #34

  https://journals.lww.com/co-oncology/fulltext/2017/07000/adult_desmoid_tumors__biology,_management_and.8.aspx

  Desmoid tumors can lead to severe pain, functional impairment and, more rarely, a life-threatening condition. […] The course of desmoid tumor is unpredictable, as spontaneous regression, long-lasting stable disease and disease progression can occur, and reliable and validated predictive factors are lacking. […] The wait and see policy avoids inadequate treatment for desmoid tumor that could spontaneously regress and discourages treatment for stable and pauci-symptomatic desmoid tumor. […] The probability of spontaneous regression after pregnancy was approximately 10%, the documented progression after the wait and see policy was 60%, the failure of medical treatment occurred in 10%, and the risk of relapse after surgery was 13%. […] In patients with preexisting desmoid tumor, the occurrence of pregnancy was associated with a risk of relapse or progression of approximately 40%; this relapse/progression spontaneously regressed in approximately 10% of cases, and medical therapy was effective in approximately 90% of cases. […] Desmoid tumors are a major concern in FAP patients and cause significant morbidity and mortality, as most cases are intra-abdominal (in the small bowel mesentery) and can cause bowel obstruction or ulceration and ureter stenosis.

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