Materiały źródłowe

• #1 Desmoid Tumor – StatPearls – NCBI Bookshelf

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

  Desmoid tumors are mesenchymal neoplasms that are considered locally invasive but nonmetastasizing. […] The management of desmoid tumors poses a significant challenge due to the lack of a standard treatment approach. […] Although watchful observation is recommended for asymptomatic desmoid tumors (20% can regress spontaneously), surgical resection has fallen out of favor due to high rates of recurrence. […] Collaborate with all interprofessional team members, including specialists such as surgical oncologists, orthopedic oncologists, sarcoma pathologists, radiologists, and pharmacists, to provide efficient, comprehensive, and coordinated care to patients diagnosed with desmoid tumors. […] There is no standard approach to managing desmoid tumor. Most experts recommend watchful observation in asymptomatic patients.

• #2 Desmoid Tumors: Symptoms, Causes & Treatment

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

  Desmoid tumors are rare benign (noncancerous) tumors that develop in connective tissue. […] Healthcare providers can treat desmoid tumors, but they often come back (recur). […] Healthcare providers often treat desmoid tumors the same way they treat cancerous tumors. Specific treatment depends on several factors, like tumor size, location and the kind of symptoms it causes. Surgery is the most common treatment. Other treatments include: […] Treatment side effects vary depending on the treatment type. In general, desmoid tumor treatment side effects may include fatigue from chemotherapy or surgery, or infections that develop after surgery. Your healthcare provider will discuss all treatment side effects, so you know what to expect. […] 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. […] Fortunately, healthcare providers have treatments that can ease symptoms and sometimes eliminate the tumor.

• #3 Childhood Desmoid Tumors | Dana-Farber Cancer Institute

  https://www.dana-farber.org/cancer-care/types/childhood-desmoid-tumors

  Desmoid tumors develop in the fibrous tissue that forms tendons and ligaments, most often in the arms, legs, and abdomen. […] At Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, our doctors have deep expertise in diagnosing, treating, and caring for children with desmoid tumors. When you come to us for care, your child will be in the hands of renowned specialists in our Bone and Soft Tissue Tumors Program. […] Since Desmoid tumors and fibromatosis do not spread, surgery is often the only treatment required. Because these tumors often return, additional operations may be needed. […] We will continue to care for your child after treatment through our comprehensive pediatric cancer survivorship programs. We provide everything from ongoing medical monitoring and care to psychosocial and nutritional support to ensure your child receives the best possible care.

• #4 Desmoid tumors: Treatment – UpToDate

  https://www.uptodate.com/contents/desmoid-tumors-systemic-therapy

  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). […] 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.

• #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. […] 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. There is a high unmet need for treatments that specifically target DT and improve quality of life.

• #6

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

  Patients with DT experience symptoms of pain and impaired mobility that can negatively impact their daily activities and overall quality of life. […] Guidelines for DT recommend options such as active surveillance, locoregional treatments, and systemic treatments. […] Unmet needs in DT include early and accurate diagnosis and approved treatments indicated for patients with DT.

• #7 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. […] The NCCN Guidelines and DTWG Guideline recommend engaging a multidisciplinary care team with experience in desmoid tumors. […] Guidelines recommend consulting a desmoid tumor expert. […] The NCCN Guidelines and Desmoid Tumor Working Group (DTWG) Guideline recommend an initial evaluation and management by a multidisciplinary team with expertise and experience in desmoid tumors. […] Consulting with desmoid tumor experts can help support more accurate patient assessment, the prompt initiation of treatment when appropriate, and individualized management of symptoms.

• #8 Desmoid tumors – Care at Mayo Clinic – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/desmoid-tumors/care-at-mayo-clinic/mac-20355086

  Mayo Clinic doctors are trusted and respected for their expertise in diagnosing and treating rare conditions, including desmoid tumors. Specialists draw on their experience caring for people with desmoid tumors to provide you with a personalized treatment plan. […] At Mayo Clinic, you’ll receive coordinated care from a team of doctors and health care professionals. Doctors involved in your care may include experts in pathology, radiology, oncology, radiation oncology and surgery. Other professionals are included as needed. […] Mayo Clinic doctors will work with you to review all of your treatment options and choose the treatment that best suits your needs and goals. A full range of treatment options is available to people with desmoid tumors, including surgery, radiation therapy, chemotherapy and other medications. […] These endorsements reinforce our commitment to providing expert care, tailored to the needs of each patient with desmoid tumors. […] Mayo Clinic is consistently recognized by external organizations for providing outstanding care.

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

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

  Desmoid tumors may also regress after exposure to oral contraceptives. […] Careful monitoring and the involvement of an experienced surgical oncologist are important to the successful outcome for patients with desmoid tumors. […] The day of surgery, you will be cared for in the operating room by surgeons, anesthesiologists and nurses who specialize in surgery for patients with desmoid tumors. After surgery, you will recover in the post-surgical care unit where you will receive comprehensive care by an experienced surgical and nursing staff. […] The Center for Sarcoma and Bone Oncology at Dana-Farber Brigham Cancer Center provides advanced and innovative multidisciplinary care for patients with sarcoma, including desmoid tumors. Our treatment team includes surgical oncologists, medical oncologists, radiologists, plastic surgeons, nutritionists, pathologists and anesthesiologists.

• #10 Desmoid Tumor – StatPearls – NCBI Bookshelf

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

  Desmoid tumors are mesenchymal neoplasms that are considered locally invasive but nonmetastasizing. […] The management of desmoid tumors poses a significant challenge due to the lack of a standard treatment approach. […] Although watchful observation is recommended for asymptomatic desmoid tumors (20% can regress spontaneously), surgical resection has fallen out of favor due to high rates of recurrence. […] Collaborate with all interprofessional team members, including specialists such as surgical oncologists, orthopedic oncologists, sarcoma pathologists, radiologists, and pharmacists, to provide efficient, comprehensive, and coordinated care to patients diagnosed with desmoid tumors. […] There is no standard approach to managing desmoid tumor. Most experts recommend watchful observation in asymptomatic patients.

• #11 Desmoid Tumors Treatment | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/childhood-cancer/solid-tumors/desmoid-tumors.html

  Desmoid tumors care at St. Jude provides the highest quality of care for patients with desmoid tumors: […] St. Jude offers a dedicated team of specialists to meet the needs of children with desmoid tumors, including: Surgeons, doctors, and nurses who treat this cancer […] The expert skills and experience of St. Jude specialty surgeons can help improve patients chances for best outcomes. […] The nurse-to-patient ratio at St. Jude is about 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit.

• #12 Desmoid Tumor – StatPearls – NCBI Bookshelf

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

  Desmoid tumors are mesenchymal neoplasms that are considered locally invasive but nonmetastasizing. […] The management of desmoid tumors poses a significant challenge due to the lack of a standard treatment approach. […] Although watchful observation is recommended for asymptomatic desmoid tumors (20% can regress spontaneously), surgical resection has fallen out of favor due to high rates of recurrence. […] Collaborate with all interprofessional team members, including specialists such as surgical oncologists, orthopedic oncologists, sarcoma pathologists, radiologists, and pharmacists, to provide efficient, comprehensive, and coordinated care to patients diagnosed with desmoid tumors. […] There is no standard approach to managing desmoid tumor. Most experts recommend watchful observation in asymptomatic patients.

• #13 Current Management Strategies for Desmoid Tumors Are Marked by Less Surgery, More Systemic Options

  https://www.onclive.com/view/current-management-strategies-for-desmoid-tumors-are-marked-by-less-surgery-more-systemic-options

  Despite their rarity, the management of desmoid tumors has been an area of significant clinical change as the field has shifted away from aggressive surgical interventions to active surveillance and systemic therapies that are less morbid for patients. […] We want to turn this into a disease that is amenable with systemic therapies rather than surgery. […] The experts highlighted key challenges that remain in the management of desmoid tumors, emerging systemic options that are positioned to transform the paradigm further away from surgical interventions, and the importance of getting second opinions in this space. […] The first inclination most people have when they are told they have a desmoid tumor is to cut it out. The problem is that doing surgery on desmoid tumors can trigger recurrences in a fair number of patients, as many as up to 50% of tumors can recur after surgery.

• #14 Desmoid tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/desmoid-tumors/diagnosis-treatment/drc-20446388

  Our caring team of Mayo Clinic experts can help you with your desmoid tumors-related health concerns […] Treatments for desmoid tumors include: […] Monitoring the growth of the tumor. If your desmoid tumor causes no signs or symptoms, your doctor may recommend monitoring the tumor to see if it grows. […] Surgery. 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. […] Radiation therapy. Radiation therapy uses high-powered beams, such as X-rays and protons, to kill tumor cells. […] Chemotherapy and other medications. 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. […] Learn enough about desmoid tumors to make decisions about your care. […] Don’t hesitate to ask other questions. […] What else should I know about you that will help me make the right recommendations about your care?

• #15 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

  Dr. Aimee Crago specializes in caring for people with desmoid tumors. […] Desmoid tumors should be treated by doctors with special training in caring for people with soft tissue sarcoma. […] Treatments for desmoid tumors can vary widely. They include active monitoring, chemotherapy, targeted therapies, cryoablation, surgery, or research studies, also known as clinical trials. […] At MSK, we have a team of experts in treating desmoid tumors. […] Our doctors base their treatment recommendations on the kind of tumor you have. Treatment depends on the desmoid tumors size and location. […] Surgery to remove the desmoid tumor has been the most common treatment in the past. But we now have a number of other treatment options for people with desmoid tumors. […] Active surveillance, also known as watchful waiting. This can be a good option for some people, but not everyone. Your care team closely monitors the tumor with regular scans. […] MSK strongly recommends people use this nomogram with a doctor very experienced in managing desmoid tumors.

• #16 Desmoid tumors: Treatment – UpToDate

  https://www.uptodate.com/contents/desmoid-tumors-systemic-therapy

  Active surveillance typically involves imaging studies at least every three to six months. We typically will obtain our first imaging at three months after diagnosis to determine the growth rate. […] Support for observation as a treatment strategy is supported in several studies. As an example, in a phase III trial comparing the use of sorafenib with placebo in patients with desmoid fibromatosis, 20 percent of patients in the placebo arm had a partial response.

• #17 Desmoid tumors: Treatment – UpToDate

  https://www.uptodate.com/contents/desmoid-tumors-systemic-therapy

  Active surveillance typically involves imaging studies at least every three to six months. We typically will obtain our first imaging at three months after diagnosis to determine the growth rate. […] Support for observation as a treatment strategy is supported in several studies. As an example, in a phase III trial comparing the use of sorafenib with placebo in patients with desmoid fibromatosis, 20 percent of patients in the placebo arm had a partial response.

• #18 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 develop anywhere in the body where there is soft connective tissue. […] Desmoid tumors do not spread to other parts of the body (metastasize). Although they are not malignant, they can cause serious health problems as they grow. They can affect and damage surrounding organs, blood vessels, and nerves. […] 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. […] Regularly monitoring your symptoms and reporting them to your healthcare providers may help them create a more personalized treatment plan.

• #19 Approaches to Patient Management of Desmoid Tumors –

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

  NCCN Guidelines recommend that patients having tumors that are progressing, symptomatic, or impairing or threatening in function be offered therapy with the decision based on the location of the tumor and the potential morbidity of the therapeutic option. […] 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 tumors that are symptomatic, or impairing or threatening in function, patients should be offered therapy with the decision based on the location of the tumor and potential morbidity of the therapeutic option. […] Surgery is not considered a first-line treatment option for desmoid tumors, except in certain situations if agreed upon by a multidisciplinary tumor board.

• #20 Approaches to Patient Management of Desmoid Tumors –

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

  Due to the infiltrative nature of desmoid tumors, clear margins can be difficult to achieve and may require extensive resection that can lead to additional morbidity. […] Surgery can cause additional morbidity for patients with desmoid tumors. […] More aggressive local treatment may be associated with poorer long-term functional outcomes. […] According to the NCCN Guidelines, radiation therapy, cryoablation, and drug-eluting bead chemoembolization are sometimes used to help treat desmoid tumors. […] For patients with progressive and/or symptomatic desmoid tumors who are not candidates for systemic therapy, or whose disease is refractory to systemic therapy, consideration of cryoablation and radiation therapy is appropriate.

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

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

  Desmoid tumors may also regress after exposure to oral contraceptives. […] Careful monitoring and the involvement of an experienced surgical oncologist are important to the successful outcome for patients with desmoid tumors. […] The day of surgery, you will be cared for in the operating room by surgeons, anesthesiologists and nurses who specialize in surgery for patients with desmoid tumors. After surgery, you will recover in the post-surgical care unit where you will receive comprehensive care by an experienced surgical and nursing staff. […] The Center for Sarcoma and Bone Oncology at Dana-Farber Brigham Cancer Center provides advanced and innovative multidisciplinary care for patients with sarcoma, including desmoid tumors. Our treatment team includes surgical oncologists, medical oncologists, radiologists, plastic surgeons, nutritionists, pathologists and anesthesiologists.

• #22 What To Expect After Desmoid Tumor Surgery: Recovery Time and More | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/what-to-expect-after-desmoid-tumor-surgery-recovery-time-and-more

  Desmoid tumors are known for being aggressive and often growing back after surgery, making them hard to treat. […] If your doctor has recommended that you get desmoid tumor surgery, its important that you understand why and know what to expect after the procedure. […] Surgery still may be considered for some cases, such as if desmoid tumors cause significant symptoms or are located in areas where they pose a risk. […] The recovery process after desmoid tumor surgery can vary. […] Pain is common after surgery, and youll receive medication to manage discomfort. […] It’s important to take proper care of your surgical site. This helps prevent infection and promotes healing. […] Regular follow-up appointments will be scheduled to monitor the healing process and address any concerns.

• #23 What To Expect After Desmoid Tumor Surgery: Recovery Time and More | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/what-to-expect-after-desmoid-tumor-surgery-recovery-time-and-more

  Depending on the surgerys impact, physical therapy may be recommended to help rebuild your strength and mobility. […] Even after a successful surgery, follow-up care is critical for managing desmoid tumors. […] You’ll need regular imaging tests such as MRI or CT scans to check for signs of new tumor growth. […] If a tumor returns, your treatment plan may involve nonsurgical options such as medication, radiation therapy, or other localized treatments. […] You might need to make lifestyle changes to support your overall health and well-being. […] Chronic pain or discomfort can be an ongoing issue for some people after desmoid tumor surgery. […] Ongoing emotional and psychological support can help you manage anxiety and maintain a positive outlook. […] It’s important to educate yourself about the nature of desmoid tumors and any signs that they might return. […] Although surgery is no longer the first-line treatment for desmoid tumors, it remains an important option in certain cases.

• #24 Desmoid tumors | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/desmoid-tumors

  Desmoid tumors are rare tumors in the United States and usually affect adults. Also known as aggressive fibromatosis, desmoid tumors are considered benign (not cancerous) soft-tissue tumors. […] Desmoid tumors can be difficult to remove because they intertwine with the tissues around them. It is not always possible for surgeons to locate the boundaries of the tumor when they are removing it. This makes the rate of recurrence (return of the tumor in the same spot) of a desmoid tumor as high as 70 percent. […] Most children with desmoid tumors can be treated with limb-sparing (also known as limb-salvage) surgery. […] After surgery for a desmoid tumor, your child will remain in the hospital for one or two days. Your child may require pain medication until the surgical site heals, and ongoing physical therapy. […] Desmoid tumors have a high rate of recurrence even after being successfully treated so it is important for your child to see a physician regularly.

• #25 Desmoid Tumor – StatPearls – NCBI Bookshelf

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

  Pharmacists and nurses play a critical role in managing the toxicities of the various systemic treatments. […] Desmoid tumors are usually detected as a solitary mass in a patient presenting to the emergency room with symptoms of pain or in the office of a primary care physician. […] The genetics team needs to be involved in the care of young patients.

• #26

  https://journals.lww.com/nursing/fulltext/2018/06000/caring_for_a_patient_with_a_desmoid_tumor.9.aspx

  Caring for a patient with a desmoid tumor […] GENERAL PURPOSE: To provide information about desmoid tumors. LEARNING OBJECTIVES/OUTCOMES: After completing this continuing-education activity, you should be able to: 1. Describe the characteristics of desmoid tumors. 2. Identify available treatment strategies for patients with a desmoid tumor. 3. List the nursing considerations for a patient with a desmoid tumor. […] What should be encouraged postoperatively to prevent pneumonia? early ambulation […] For patients receiving chemotherapy, the recommended diet usually is high in calories and protein. […] A common manifestation in patients receiving chemotherapy for desmoid tumors is fatigue.

• #27 FDA OKs Nirogacestat for Adults Patients With Desmoid Tumors

  https://www.oncnursingnews.com/view/fda-oks-nirogacestat-for-adults-patients-with-desmoid-tumors

  The toxicity of nirogacestat was examined in 69 patients who were enrolled in DeFi. […] Twenty percent of patients experienced serious adverse effects (AEs). […] Dose reductions and interruptions were needed in 41% and 51% of patients, respectively. […] Moreover, 20% of patients permanently discontinued nirogacestat due to AEs.

• #28

  https://journals.lww.com/nursing/fulltext/2018/06000/caring_for_a_patient_with_a_desmoid_tumor.9.aspx

  Caring for a patient with a desmoid tumor […] GENERAL PURPOSE: To provide information about desmoid tumors. LEARNING OBJECTIVES/OUTCOMES: After completing this continuing-education activity, you should be able to: 1. Describe the characteristics of desmoid tumors. 2. Identify available treatment strategies for patients with a desmoid tumor. 3. List the nursing considerations for a patient with a desmoid tumor. […] What should be encouraged postoperatively to prevent pneumonia? early ambulation […] For patients receiving chemotherapy, the recommended diet usually is high in calories and protein. […] A common manifestation in patients receiving chemotherapy for desmoid tumors is fatigue.

• #29 FDA OKs Nirogacestat for Adults Patients With Desmoid Tumors

  https://www.oncnursingnews.com/view/fda-oks-nirogacestat-for-adults-patients-with-desmoid-tumors

  The toxicity of nirogacestat was examined in 69 patients who were enrolled in DeFi. […] Twenty percent of patients experienced serious adverse effects (AEs). […] Dose reductions and interruptions were needed in 41% and 51% of patients, respectively. […] Moreover, 20% of patients permanently discontinued nirogacestat due to AEs.

• #30 Case Number: 202112-144619 | Department of Financial Services

  https://www.dfs.ny.gov/public-appeals/case-number-202112-144619

  Sorafenib has undergone Phase I and II testing in children showing that it is safe and tolerable at BSA (body surface area)-based doing with a similar side effect profile as adults. […] Based on this data, sorafenib (Nexavar) would likely be more beneficial for this patient than intravenous (IV) systemic chemotherapy due to equal efficacy with improved quality of life and decreased potential risks and side effects. […] Desmoid fibromatosis tumors can be aggressive and occur in both children and adults. They are usually localized tumors arising from mesenchymal tissues. […] There is scientific controlled trial data and results in adults to support its use and efficacy as well as multiple case reports in pediatrics also supporting its efficacy and safety.

• #31 Nirogacestat Shrinks Desmoid Tumors – NCI

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

  People treated with nirogacestat also reported reduced pain, improved physical functioning, and improved health-related quality of life. […] Side effects were mild and included nausea and fatigue. […] For some whose tumors are stable and not causing pain or other problems, observation may be warranted, he said. […] And other potential treatment options for desmoid tumors that are already in clinical trials may be on the horizon.

• #32 Case Number: 202112-144619 | Department of Financial Services

  https://www.dfs.ny.gov/public-appeals/case-number-202112-144619

  There is no standard of care for systemic treatment of desmoid fibromatosis in children but options include systemic intravenous chemotherapy (methotrexate and vinblastine) and oral tyrosine kinase therapy (sorafenib). […] While there is no established standard of care for systemic therapy for desmoid fibromatosis, there are several effective approaches, including systemic intravenous chemotherapy (methotrexate and vinblastine) and the oral multi-targeted tyrosine kinase inhibitor, sorafenib. […] The benefit of sorafenib over intravenous chemotherapy is that it is taken orally and had a tolerable side effect profile, while IV chemotherapy requires surgical placement of a mediport for administration, frequent office visits for treatments, and harbors an increased risk for infection due to myelosuppression as well as other toxicities including hepatotoxicity, mucositis, and neuropathy.

• #33 Desmoid Tumor Treatment – San Diego – Scripps Health

  https://www.scripps.org/services/cancer-care/desmoid-tumor-treatment

  Your cancer care team includes professionals from every area of oncology, including physicians, surgeons, radiation oncologists and nurses who specialize in cancer care. […] Oncology nurses and nurse navigators with extensive clinical expertise in cancer care to help guide you and your caregivers to make informed decisions and ensure your optimal care. […] Palliative care to provide an extra layer of supportive care to manage pain and relieve symptoms, offer emotional and spiritual support, and improve your quality of life. […] Scripps Cancer Center offers a variety of patient support services to ensure your physical, psychological and emotional well-being as well as resources for dealing with the logistical and financial aspects of cancer care.

• #34 What To Expect After Desmoid Tumor Surgery: Recovery Time and More | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/what-to-expect-after-desmoid-tumor-surgery-recovery-time-and-more

  Depending on the surgerys impact, physical therapy may be recommended to help rebuild your strength and mobility. […] Even after a successful surgery, follow-up care is critical for managing desmoid tumors. […] You’ll need regular imaging tests such as MRI or CT scans to check for signs of new tumor growth. […] If a tumor returns, your treatment plan may involve nonsurgical options such as medication, radiation therapy, or other localized treatments. […] You might need to make lifestyle changes to support your overall health and well-being. […] Chronic pain or discomfort can be an ongoing issue for some people after desmoid tumor surgery. […] Ongoing emotional and psychological support can help you manage anxiety and maintain a positive outlook. […] It’s important to educate yourself about the nature of desmoid tumors and any signs that they might return. […] Although surgery is no longer the first-line treatment for desmoid tumors, it remains an important option in certain cases.

• #35 Empowering Desmoid Tumor Patients: Education and Resources

  https://www.ajmc.com/view/empowering-desmoid-tumor-patients-education-and-resources

  Gain insights into valuable educational resources for patients coping with desmoid tumors, such as global consensus papers and advocacy group websites, and the significance of seeking multiple expert opinions for informed decision-making. […] Gounder underscores the importance of patient advocacy groups such as the Desmoid Tumor Research Foundation (DTRF) and consensus guidelines for reliable, evidence-based information. […] Ratan reinforces the credibility of the Global Consensus Paper and DTRF resources, emphasizing their efforts to limit bias. […] Both physicians stress the value of seeking second opinions for informed decision-making, even among experts. […] Haumschild concludes the discussion by acknowledging the significance of multidisciplinary care, ongoing education, and seeking opinions at centers of excellence. […] The physicians emphasize the dynamic nature of desmoid tumor management, with evolving treatments and the need for holistic care. […] They encourage patients to use trusted information sources and collaborate with health care providers for personalized treatment plans.

• #36 Desmoid tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/desmoid-tumors/diagnosis-treatment/drc-20446388

  Our caring team of Mayo Clinic experts can help you with your desmoid tumors-related health concerns […] Treatments for desmoid tumors include: […] Monitoring the growth of the tumor. If your desmoid tumor causes no signs or symptoms, your doctor may recommend monitoring the tumor to see if it grows. […] Surgery. 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. […] Radiation therapy. Radiation therapy uses high-powered beams, such as X-rays and protons, to kill tumor cells. […] Chemotherapy and other medications. 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. […] Learn enough about desmoid tumors to make decisions about your care. […] Don’t hesitate to ask other questions. […] What else should I know about you that will help me make the right recommendations about your care?

• #37 Desmoid tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/desmoid-tumors/diagnosis-treatment/drc-20446388

  Our caring team of Mayo Clinic experts can help you with your desmoid tumors-related health concerns […] Treatments for desmoid tumors include: […] Monitoring the growth of the tumor. If your desmoid tumor causes no signs or symptoms, your doctor may recommend monitoring the tumor to see if it grows. […] Surgery. 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. […] Radiation therapy. Radiation therapy uses high-powered beams, such as X-rays and protons, to kill tumor cells. […] Chemotherapy and other medications. 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. […] Learn enough about desmoid tumors to make decisions about your care. […] Don’t hesitate to ask other questions. […] What else should I know about you that will help me make the right recommendations about your care?

• #38 What To Expect After Desmoid Tumor Surgery: Recovery Time and More | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/what-to-expect-after-desmoid-tumor-surgery-recovery-time-and-more

  Depending on the surgerys impact, physical therapy may be recommended to help rebuild your strength and mobility. […] Even after a successful surgery, follow-up care is critical for managing desmoid tumors. […] You’ll need regular imaging tests such as MRI or CT scans to check for signs of new tumor growth. […] If a tumor returns, your treatment plan may involve nonsurgical options such as medication, radiation therapy, or other localized treatments. […] You might need to make lifestyle changes to support your overall health and well-being. […] Chronic pain or discomfort can be an ongoing issue for some people after desmoid tumor surgery. […] Ongoing emotional and psychological support can help you manage anxiety and maintain a positive outlook. […] It’s important to educate yourself about the nature of desmoid tumors and any signs that they might return. […] Although surgery is no longer the first-line treatment for desmoid tumors, it remains an important option in certain cases.

• #39 What To Expect After Desmoid Tumor Surgery: Recovery Time and More | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/what-to-expect-after-desmoid-tumor-surgery-recovery-time-and-more

  Depending on the surgerys impact, physical therapy may be recommended to help rebuild your strength and mobility. […] Even after a successful surgery, follow-up care is critical for managing desmoid tumors. […] You’ll need regular imaging tests such as MRI or CT scans to check for signs of new tumor growth. […] If a tumor returns, your treatment plan may involve nonsurgical options such as medication, radiation therapy, or other localized treatments. […] You might need to make lifestyle changes to support your overall health and well-being. […] Chronic pain or discomfort can be an ongoing issue for some people after desmoid tumor surgery. […] Ongoing emotional and psychological support can help you manage anxiety and maintain a positive outlook. […] It’s important to educate yourself about the nature of desmoid tumors and any signs that they might return. […] Although surgery is no longer the first-line treatment for desmoid tumors, it remains an important option in certain cases.

• #40 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

  Management of recurrence is challenging. […] If all other modalities fail, radical surgery can be considered as a salvage option. […] 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.

• #41 What To Expect After Desmoid Tumor Surgery: Recovery Time and More | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/what-to-expect-after-desmoid-tumor-surgery-recovery-time-and-more

  Depending on the surgerys impact, physical therapy may be recommended to help rebuild your strength and mobility. […] Even after a successful surgery, follow-up care is critical for managing desmoid tumors. […] You’ll need regular imaging tests such as MRI or CT scans to check for signs of new tumor growth. […] If a tumor returns, your treatment plan may involve nonsurgical options such as medication, radiation therapy, or other localized treatments. […] You might need to make lifestyle changes to support your overall health and well-being. […] Chronic pain or discomfort can be an ongoing issue for some people after desmoid tumor surgery. […] Ongoing emotional and psychological support can help you manage anxiety and maintain a positive outlook. […] It’s important to educate yourself about the nature of desmoid tumors and any signs that they might return. […] Although surgery is no longer the first-line treatment for desmoid tumors, it remains an important option in certain cases.

• #42 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

  Management of recurrence is challenging. […] If all other modalities fail, radical surgery can be considered as a salvage option. […] 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.

• #43 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

  Management of recurrence is challenging. […] If all other modalities fail, radical surgery can be considered as a salvage option. […] 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.

• #44 What To Expect After Desmoid Tumor Surgery: Recovery Time and More | MyDesmoidTumorTeam

  https://www.mydesmoidtumorteam.com/resources/what-to-expect-after-desmoid-tumor-surgery-recovery-time-and-more

  Depending on the surgerys impact, physical therapy may be recommended to help rebuild your strength and mobility. […] Even after a successful surgery, follow-up care is critical for managing desmoid tumors. […] You’ll need regular imaging tests such as MRI or CT scans to check for signs of new tumor growth. […] If a tumor returns, your treatment plan may involve nonsurgical options such as medication, radiation therapy, or other localized treatments. […] You might need to make lifestyle changes to support your overall health and well-being. […] Chronic pain or discomfort can be an ongoing issue for some people after desmoid tumor surgery. […] Ongoing emotional and psychological support can help you manage anxiety and maintain a positive outlook. […] It’s important to educate yourself about the nature of desmoid tumors and any signs that they might return. […] Although surgery is no longer the first-line treatment for desmoid tumors, it remains an important option in certain cases.

• #45 Desmoid Tumor Treatment – San Diego – Scripps Health

  https://www.scripps.org/services/cancer-care/desmoid-tumor-treatment

  Your cancer care team includes professionals from every area of oncology, including physicians, surgeons, radiation oncologists and nurses who specialize in cancer care. […] Oncology nurses and nurse navigators with extensive clinical expertise in cancer care to help guide you and your caregivers to make informed decisions and ensure your optimal care. […] Palliative care to provide an extra layer of supportive care to manage pain and relieve symptoms, offer emotional and spiritual support, and improve your quality of life. […] Scripps Cancer Center offers a variety of patient support services to ensure your physical, psychological and emotional well-being as well as resources for dealing with the logistical and financial aspects of cancer care.

• #46 Desmoid Tumours — DermNet

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

  Desmoid tumours can be more complex in pregnancy a combination of hormonal signalling, trauma, and mechanical constraints may worsen symptoms during this time. […] Pain management is crucial due to the potential for long-lasting morbidity. […] In asymptomatic patients, the treatment of desmoid tumours relies on active surveillance with multi-disciplinary team (MDT) input, ideally in an experienced centre given the rarity of the condition. […] In patients with active symptoms (ie, volumetric progression and compression symptoms), treatment options are primarily surgical with some scope for radiation therapy and systemic therapy. […] Pregnant patients need to be managed in experienced centres with joint care. […] Treatment focuses on preventing symptomatic disease rather than total cure; in 70% of patients, the tumour recurs following definitive management, such as surgery or radiotherapy.

• #47 Desmoid Tumours — DermNet

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

  Desmoid tumours can be more complex in pregnancy a combination of hormonal signalling, trauma, and mechanical constraints may worsen symptoms during this time. […] Pain management is crucial due to the potential for long-lasting morbidity. […] In asymptomatic patients, the treatment of desmoid tumours relies on active surveillance with multi-disciplinary team (MDT) input, ideally in an experienced centre given the rarity of the condition. […] In patients with active symptoms (ie, volumetric progression and compression symptoms), treatment options are primarily surgical with some scope for radiation therapy and systemic therapy. […] Pregnant patients need to be managed in experienced centres with joint care. […] Treatment focuses on preventing symptomatic disease rather than total cure; in 70% of patients, the tumour recurs following definitive management, such as surgery or radiotherapy.

• #48 Desmoid Tumours — DermNet

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

  Desmoid tumours can be more complex in pregnancy a combination of hormonal signalling, trauma, and mechanical constraints may worsen symptoms during this time. […] Pain management is crucial due to the potential for long-lasting morbidity. […] In asymptomatic patients, the treatment of desmoid tumours relies on active surveillance with multi-disciplinary team (MDT) input, ideally in an experienced centre given the rarity of the condition. […] In patients with active symptoms (ie, volumetric progression and compression symptoms), treatment options are primarily surgical with some scope for radiation therapy and systemic therapy. […] Pregnant patients need to be managed in experienced centres with joint care. […] Treatment focuses on preventing symptomatic disease rather than total cure; in 70% of patients, the tumour recurs following definitive management, such as surgery or radiotherapy.

• #49 Desmoid Tumours — DermNet

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

  Desmoid tumours can be more complex in pregnancy a combination of hormonal signalling, trauma, and mechanical constraints may worsen symptoms during this time. […] Pain management is crucial due to the potential for long-lasting morbidity. […] In asymptomatic patients, the treatment of desmoid tumours relies on active surveillance with multi-disciplinary team (MDT) input, ideally in an experienced centre given the rarity of the condition. […] In patients with active symptoms (ie, volumetric progression and compression symptoms), treatment options are primarily surgical with some scope for radiation therapy and systemic therapy. […] Pregnant patients need to be managed in experienced centres with joint care. […] Treatment focuses on preventing symptomatic disease rather than total cure; in 70% of patients, the tumour recurs following definitive management, such as surgery or radiotherapy.

• #50 Care of patient with desmoid tumour. – Document – Gale OneFile: Health and Medicine

  https://go.gale.com/ps/i.do?id=GALE%7CA691481885&sid=googleScholar&v=2.1&it=r&linkaccess=abs&issn=22307354&p=HRCA&sw=w

  Desmoid tumours are uncommon mesenchymal neoplasms with a fibrotic band-like consistency. They are also called aggressive fibromatosis tumours which are locally aggressive with no potential for distant metastases. The important causes of morbidity and mortality are local recurrence and adjacent organ involvement. […] Treatment options include observation, surgical resection, radiotherapy, conventional chemotherapy, hormonal agents and newer molecular-targeted agents. A multidisciplinary approach tailored to the individual patient is usually needed, depending on the location, local effects and the clinical course of the disease. […] This type of tumour is aggressive locally and can invade other surrounding tissues and adjacent organs although it generally cannot metastasise or seldom spread to distant parts of the body. Desmoid tumour is also referred to as 'aggressive fibromatosis’ and the clinical course of desmoid tumour is unpredictable and some spontaneous regression has been observed. […] The following are the common risk factors: Family history of FAP, Gardner syndrome, repeated irritation to the body part which includes surgical trauma, trauma, women with high oestrogen levels, trauma such as laparotomy, abdominal trauma, oral contraceptive use, pregnancy.

• #51 Desmoid Tumor | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/desmoid-tumor

  Patients with Desmoid tumors and fibromatosis are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through the Bone and Soft Tissue Tumors Program. […] Treatment depends on the extent of the problem and your child’s overall health and medical history. […] Surgical removal of your child’s tumor by a surgeon is the primary treatment; and because Desmoid tumor rarely metastasizes, surgery alone is often the only treatment. However, Desmoid tumors often return, so sometimes more than one surgery is needed. […] Anti-inflammatory medication: Given to help manage pain and swelling. In some cases these medications will cause the tumor to slowly shrink. […] Radiation therapy: Using high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors; radiation is used alone or in conjunction with surgery.

• #52 Desmoid Tumor | Conditions | UCSF Benioff Children’s Hospitals

  https://www.ucsfbenioffchildrens.org/conditions/desmoid-tumor

  Desmoid tumors vary greatly depending on size and location of the tumor, the extent to which it has invaded surrounding tissues and your child’s age. […] Treatment options include surgery, radiation, chemotherapy and hormone therapy. […] Surgery to remove the tumor is the primary treatment for a Desmoid tumor. Because these tumors rarely spread or metastasize, surgery alone often is the only necessary treatment. However, Desmoid tumors have a high recurrence rate, meaning they return after treatment, if they aren’t completely removed. Frequently, more than one surgery is needed. […] Anti-inflammatory medication may be given to your child to help manage pain and swelling. […] We encourage you to discuss any questions or concerns you may have with your child’s provider.

• #53 Desmoid tumors | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/desmoid-tumors

  Desmoid tumors are rare tumors in the United States and usually affect adults. Also known as aggressive fibromatosis, desmoid tumors are considered benign (not cancerous) soft-tissue tumors. […] Desmoid tumors can be difficult to remove because they intertwine with the tissues around them. It is not always possible for surgeons to locate the boundaries of the tumor when they are removing it. This makes the rate of recurrence (return of the tumor in the same spot) of a desmoid tumor as high as 70 percent. […] Most children with desmoid tumors can be treated with limb-sparing (also known as limb-salvage) surgery. […] After surgery for a desmoid tumor, your child will remain in the hospital for one or two days. Your child may require pain medication until the surgical site heals, and ongoing physical therapy. […] Desmoid tumors have a high rate of recurrence even after being successfully treated so it is important for your child to see a physician regularly.

• #54

  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. There is a high unmet need for treatments that specifically target DT and improve quality of life.

• #55

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

  Patients with DT experience symptoms of pain and impaired mobility that can negatively impact their daily activities and overall quality of life. […] Guidelines for DT recommend options such as active surveillance, locoregional treatments, and systemic treatments. […] Unmet needs in DT include early and accurate diagnosis and approved treatments indicated for patients with DT.

• #56 Nirogacestat Shrinks Desmoid Tumors – NCI

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

  People treated with nirogacestat also reported reduced pain, improved physical functioning, and improved health-related quality of life. […] Side effects were mild and included nausea and fatigue. […] For some whose tumors are stable and not causing pain or other problems, observation may be warranted, he said. […] And other potential treatment options for desmoid tumors that are already in clinical trials may be on the horizon.

• #57 Multidisciplinary Care for Desmoid Tumors

  https://www.ajmc.com/view/multidisciplinary-care-for-desmoid-tumors

  Experts discuss the role of multidisciplinary teams, gene expression patterns, rare disease drug designation, and education gaps in managing desmoid tumors, emphasizing the need for comprehensive care and precision medicine. […] Van Amerongen underscores the significance of a multidisciplinary team in evaluating and treating desmoid tumors, highlighting the evolving treatment options and the importance of case sharing. […] The discussion converges on the pivotal role of multidisciplinary care in optimizing patient outcomes. […] The segment emphasizes the importance of comprehensive, patient-centric approaches in the management of desmoid tumors.

• #58 Current Management Strategies for Desmoid Tumors Are Marked by Less Surgery, More Systemic Options

  https://www.onclive.com/view/current-management-strategies-for-desmoid-tumors-are-marked-by-less-surgery-more-systemic-options

  Despite their rarity, the management of desmoid tumors has been an area of significant clinical change as the field has shifted away from aggressive surgical interventions to active surveillance and systemic therapies that are less morbid for patients. […] We want to turn this into a disease that is amenable with systemic therapies rather than surgery. […] The experts highlighted key challenges that remain in the management of desmoid tumors, emerging systemic options that are positioned to transform the paradigm further away from surgical interventions, and the importance of getting second opinions in this space. […] The first inclination most people have when they are told they have a desmoid tumor is to cut it out. The problem is that doing surgery on desmoid tumors can trigger recurrences in a fair number of patients, as many as up to 50% of tumors can recur after surgery.

• #59 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. […] This is the first-ever FDA approval of a treatment for people with desmoid tumors. […] This disease currently has no standard treatment options. […] There is no standard of care for this disease. Surgery and chemotherapy are commonly used, but they typically fail to keep the disease at bay for long. […] In the past, there was very little for patients with desmoid tumors, said Marlene Portnoy, co-founder of the Desmoid Tumor Research Foundation. […] Over the past decade, however, some progress has been made in developing a targeted therapy for desmoid tumors. […] Nirogacestat works by blocking the activity of an enzyme called gamma secretase, which helps activate a signaling protein called Notch.

• #60 Empowering Desmoid Tumor Patients: Education and Resources

  https://www.ajmc.com/view/empowering-desmoid-tumor-patients-education-and-resources

  Gain insights into valuable educational resources for patients coping with desmoid tumors, such as global consensus papers and advocacy group websites, and the significance of seeking multiple expert opinions for informed decision-making. […] Gounder underscores the importance of patient advocacy groups such as the Desmoid Tumor Research Foundation (DTRF) and consensus guidelines for reliable, evidence-based information. […] Ratan reinforces the credibility of the Global Consensus Paper and DTRF resources, emphasizing their efforts to limit bias. […] Both physicians stress the value of seeking second opinions for informed decision-making, even among experts. […] Haumschild concludes the discussion by acknowledging the significance of multidisciplinary care, ongoing education, and seeking opinions at centers of excellence. […] The physicians emphasize the dynamic nature of desmoid tumor management, with evolving treatments and the need for holistic care. […] They encourage patients to use trusted information sources and collaborate with health care providers for personalized treatment plans.

• #61 Current Management Strategies for Desmoid Tumors Are Marked by Less Surgery, More Systemic Options

  https://www.onclive.com/view/current-management-strategies-for-desmoid-tumors-are-marked-by-less-surgery-more-systemic-options

  Historically, surgery plus or minus radiation therapy was the best treatment modality for patients with desmoid tumors. Now, surgery plays an important but less broad role in the management of desmoid tumors. […] The take-home message for medical oncologists is to slow down, talk to someone who has experience with desmoid tumors, and then have a very informed discussion with the patient. […] In general, the types of desmoid tumors that are the hardest to deal with are those that occur in conjunction with a disease called familial adenomatous polyposis. […] Our hope is that newer drugs will prove to be just as effective in that group of patients, but that is an area where we still have big challenges to help our patients live better lives and control the disease. […] We have a lot more tools for desmoid tumors than we used to. Some of these new systemic therapies have demonstrated efficacy or are currently in clinical trials, which will give us new tools.

• #62 Current Management Strategies for Desmoid Tumors Are Marked by Less Surgery, More Systemic Options

  https://www.onclive.com/view/current-management-strategies-for-desmoid-tumors-are-marked-by-less-surgery-more-systemic-options

  Historically, surgery plus or minus radiation therapy was the best treatment modality for patients with desmoid tumors. Now, surgery plays an important but less broad role in the management of desmoid tumors. […] The take-home message for medical oncologists is to slow down, talk to someone who has experience with desmoid tumors, and then have a very informed discussion with the patient. […] In general, the types of desmoid tumors that are the hardest to deal with are those that occur in conjunction with a disease called familial adenomatous polyposis. […] Our hope is that newer drugs will prove to be just as effective in that group of patients, but that is an area where we still have big challenges to help our patients live better lives and control the disease. […] We have a lot more tools for desmoid tumors than we used to. Some of these new systemic therapies have demonstrated efficacy or are currently in clinical trials, which will give us new tools.

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