Here are some quick answers to common questions about desmoid tumors. Remember to always consult with your healthcare provider if you have questions.
A tumor is an abnormal mass of tissue. It forms when cells grow and divide more than they should or do not die when they are supposed to.1
Desmoid tumors may develop in the soft tissues that connect, support, and surround other body parts (connective tissue). Connective tissue is found all throughout the body. That’s why desmoid tumors can grow anywhere in the body.2 Desmoid tumors are also sometimes called desmoid fibromatosis.2-6
Because desmoid tumors are so rare, a desmoid tumor diagnosis can raise more questions for patients than answers. To learn more about desmoid tumors, click here.
If your care team thinks you may have a desmoid tumor, they should consider imaging and a biopsy.4,7,8 These tests will help them determine if you have a desmoid tumor and what your course of treatment might look like. Don’t be afraid to ask your doctor about these tests if they have not yet been recommended.
NCCN Guidelines for Patients® provides a roadmap to help patients understand their diagnosis and treatment options. According to the NCCN Guidelines for Patients, it is important that a biopsy be performed. Imaging of the primary site with a CT or MRI will be done as needed.13 Otherwise, a wrong diagnosis can lead to delayed or incorrect treatment, or unnecessary surgery. Your doctor may choose to perform additional tests as well. If you have questions about getting diagnosed, be sure to ask your care team.
It is recommended that you consult with a multidisciplinary care team.4,8 It may include a medical oncologist and sarcoma specialist, surgeon, radiologist, pathologist and nurses.13 Your primary care physician, mental health professionals, and other healthcare professionals may be involved in your continuing care.14
Desmoid tumors are rare—so most healthcare providers may not have experience with diagnosing or treating this type of tumor. You may benefit from consulting experts at a sarcoma center. If there is not a sarcoma center close to you, it may be possible for your local doctors to work with desmoid tumor experts to create a treatment plan for you.13
Find a sarcoma center*
SpringWorks Therapeutics is providing this link to help patients find a sarcoma specialist by region, but SpringWorks had no role in developing this list and inclusion on this list does not represent an endorsement or a recommendation from SpringWorks for any center or physician.
Together with the medical oncologist, other healthcare providers may be part of your multidisciplinary care team. Depending on your needs, each of these providers can offer their expertise to assist in your ongoing care.
Nurses – Nurses may conduct physical exams, provide chemotherapy or other medications, identify needs, coordinate care with other members of the team, and educate people living with desmoid tumors and families about the disease.14
Advanced Practice Providers – Nurse practitioners (NPs) and physician assistants (PAs) may meet with you as part of the care team. They collaborate with the different team members, including your lead medical oncologist.13
Mental Health Professionals – Some people may also find it helpful to have a Mental Health Professional (psychiatrist, psychologist, social worker, or licensed counselor) to speak with. Counseling can help you understand your feelings and reactions. And if you’re feeling worried or down, a safe place to talk is always a good thing.15,16
Pain specialist – A pain specialist is a health professional who helps to manage pain.17 Pain is one of the most common and debilitating symptoms reported by people with desmoid tumors.18 This expert may recommend medicines to help block or relieve pain. They may also recommend complementary treatments (physical therapy, acupuncture, mindfulness techniques).17
Physical Therapist – A physical therapist is a health professional who can help you regain or improve impaired physical functioning. A physical therapist uses different methods to help strengthen muscles, relieve pain, and improve overall movement.19
Genetic Counselor – Genetic counselors are health professionals who can help you understand hereditary syndromes such as FAP, as well as what this might mean for you and your family. Genetic information may be an important part of your desmoid tumor treatment planning.20
Based on medical studies, 60% to 98% of desmoid tumors show beta-catenin staining under a microscope,12 so it can be an important clue to diagnosis.
Beta catenin is a protein found in cells throughout the body. One of the roles of beta catenin is to help regulate “cell signaling.”3 Cell signaling can instruct cells in the body when to grow and divide.21 Abnormal cell signaling may lead to tumor development and growth.21 Medical experts have found that beta catenin often accumulates excessively in desmoid tumor cells, causing tumors to overgrow.3
Lipomas are the most common type of soft tissue tumor.22 They occur far more frequently than desmoid tumors.
- 50% of soft-tissue tumors are lipomas23
- 3% of soft-tissue tumors are desmoid tumors6
The rarity of desmoid tumors compared to lipomas may be one reason that desmoid tumors could be misdiagnosed as lipomas.24 For instance, among a sample of people whose desmoid tumors were misdiagnosed, 4 out of 10 tumors were mistaken for lipomas.25,†
From market research conducted by SpringWorks Therapeutics.25
Desmoid tumors and their “tendril-like” growths may be difficult to see fully using imaging tests like MRI, CT scans, or ultrasound.26 This may be another reason that a desmoid tumor could be misdiagnosed as a lipoma or another tumor type.
It is not clear why some people develop desmoid tumors. However, there are some things that may make certain people more likely to get them than others.
- Women are approximately 2 to 3 times as likely as men to be diagnosed with a desmoid tumor27-29
- Recent pregnancy, injury, or surgery may increase risk as well30,31
- Patients with familial adenomatous polyposis (FAP) have an approximately 850-fold higher risk of developing desmoid tumors than the general population32
If you are diagnosed with a desmoid tumor, you may want to ask your doctor directly about whether it is cancer. They will be able to advise you on the exact status of your tumor and what your next steps might look like.
To learn more about desmoid tumors, click here.
Though they do not metastasize (spread) to other parts of the body, desmoid tumors can grow, and sometimes very quickly.6 Their “tendril-like” growths can attach to and wrap around surrounding tissues and structures.24 If you are diagnosed with a desmoid tumor, your care team may focus on active surveillance to see if your tumor is growing before starting treatment.4
You may have heard desmoid tumors described as locally aggressive or invasive. This means that their “tendril-like” growths can attach to and wrap around surrounding tissues and structures.26 As these tumors grow, they can put pressure on blood vessels and nerves, or organs, like your intestines and lungs.6,33
While some people may not feel anything, the impact of desmoid tumors and their “tendril-like” growths wrapping around surrounding structures may cause pain (often neuropathic), changes to body shape and physical function, limited range of motion, sleep changes, shortness of breath, and fatigue.6 While these are some of the physical effects you may experience when living with a desmoid tumor, remember that these effects can be different for each person.2,34
In rare instances, desmoid tumors can be life-threatening depending on where they are located, how they grow, and your overall health.2,3,5,24 However, building a care team with the right specialists can help you get the best care for your specific needs. That’s why it’s so important to talk to your doctor about the location of your tumor and symptoms you may be experiencing.
Having FAP increases a person’s risk for developing desmoid tumors. Most often, desmoid tumors in people with FAP occur in abdominal locations.13 Doctors who diagnose desmoid tumors will want to know about any family history of FAP. A genetic test for FAP may be recommended for people who have a desmoid tumor.13
Sometimes, it’s recommended that people with FAP have a colectomy (surgery to remove the colon) as a way to prevent colon cancer. For some patients, this procedure can increase risk for desmoid tumors developing in the future.8
For more information about FAP or if you have additional questions, please ask your doctor.
Currently, there are no FDA-approved treatment options indicated for desmoid tumors.35 Once a desmoid tumor has been diagnosed, there are several management options for you and your healthcare provider to consider depending on the size, location, aggressiveness, and discomfort of the tumor.12 Healthcare providers who specialize in desmoid tumors may be based at a sarcoma center. If there is not a sarcoma center close to you, it may be possible for your local doctors to work with desmoid tumor experts to create a treatment plan for you.13
Results and side effects can vary from person to person. It may be helpful to talk about what to expect with your care team. Together, you and your care team can come up with strategies to help handle side effects if you experience them. Current approaches to care include:
Desmoid tumor experts no longer recommend surgery as the first option to treat desmoid tumors in most locations.4,8 When making a decision about surgery, a team of experts should agree on whether surgery is appropriate. They will assess the tumor size and location, the person’s overall health, and how symptoms may be affecting daily life.13
During surgery, the surgeon tries to remove the entire desmoid tumor and some surrounding healthy tissue—called a “clear margin” where no tumor cells are visible.6
Unfortunately, even with a clear margin, sometimes desmoid tumors still come back (recurrence).38-41Also, completely removing a desmoid tumor and getting a clear margin may be difficult. That’s because desmoid tumors have far-reaching “tendril-like” growths that radiate outward.37,42 If any part of the desmoid tumor is left after surgery (called a “positive margin”), there is a high risk of recurrence.40,44
Desmoid tumors can come back after surgery 24%-77% of the time.29,43 The risk may be higher for people who are diagnosed at a younger age. Other factors for a higher risk of recurrence include: tumor location (intra-abdominal, chest wall or limbs), a larger tumor size38, and having a recurrence after a previous surgery.44
The infiltrative nature of desmoid tumors may make them challenging to remove surgically. Their “tendril-like” growths can extend into layers of tissue beyond the tumor itself.24 In contrast, lipomas are generally well defined within a thin capsule of tissue.48
After surgery lipomas generally do not come back (recur).22 However, desmoid tumors can come back after surgery—24% to 77% of the time.29,43 The risk may be higher for people who are diagnosed at a younger age. Other factors for a higher risk of recurrence include: tumor location (intra-abdominal, chest wall, or limbs), a larger tumor size38, and having a recurrence after a previous surgery.44
Under a microscope, the cells of a lipoma look very different from the cells of a desmoid tumor.12,22 A biopsy of the tumor is the only way to see these differences before surgery. Ask your doctor whether a biopsy is appropriate for you.
Right now, there is no FDA-approved medication for desmoid tumors.35 However, there are treatments your care team may prescribe to help shrink and/or stabilize the tumor and manage symptoms.
The recent clinical guidelines recommend the following treatment options:4,8
- Tyrosine kinase inhibitors (TKIs, may include sorafenib, pazopanib, and imatinib)
- Chemotherapy (options may include methotrexate and vinblastine/vinorelbine, doxorubicin-based regimens)
- Nonsteroidal anti-inflammatory drugs (NSAIDs, for pain)
Antihormonal therapies (tamoxifen or toremifene, with or without NSAIDs) do not have guideline recommendations.4,8
Remember, results and side effects can vary from person to person. You may want to talk about what to expect with your care team. Together, you can come up with strategies to help handle side effects if you experience them.
The following websites are good places to check:§
Advocacy Groups and Additional Resources
You may find it helpful to reach out to the organizations listed below for information, support, or to connect with others who are living with desmoid tumors.
SpringWorks Therapeutics is providing these links to help patients find more information about desmoid tumors, but inclusion on this list does not represent an endorsement or a recommendation from SpringWorks for any group or organization.
- NIH. National Cancer Institute. NCI dictionary of cancer terms: tumor. Accessed February 12, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/tumor
- NIH. National Cancer Institute Center for Cancer Research. Desmoid tumor. Accessed February 12, 2023. https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor
- Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;29(4):268-274.
- Gronchi A, et al. Desmoid Tumor Working Group. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020;127:96-107.
- Joglekar SB, Rose PS, Sim F, Okuno S, Petersen I. Current perspectives on desmoid tumors: the Mayo Clinic approach. Cancers (Basel). 2011;3(3):3143-3155.
- Constantinidou A, Scurr M, Judson I, Litchman C. Clinical presentation of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012:chap 2. Accessed February 12, 2023. https://www.researchgate.net/publication/226455135
- Cancer.Net. Desmoid tumor: diagnosis. Accessed February 12, 2023. https://www.cancer.net/cancer-types/desmoidtumor/diagnosis
- Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.2.2023. © National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed August 8, 2023. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use, or application, and disclaims any responsibility for their application or use in any way.
- Cancer.Net. Magnetic resonance imaging (MRI). Accessed February 12, 2023. https://www.cancer.net/navigating-cancercare/diagnosing-cancer/tests-and-procedures/magnetic-resonance-imaging-mri
- Cancer.Net. Biopsy. Accessed February 12, 2023. https://www.cancer.net/navigating-cancer-care/diagnosing-cancer/tests-and-procedures/biopsy
- Carlson JW, Fletcher CDM. Immunohistochemistry for β-catenin in the differential diagnosis of spindle cell lesions: analysis of a series and review of the literature. Histopathology. 2007;51(4):509-514.
- Gronchi A, et al. Desmoid Tumor Working Group. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients [supplementary appendix]. Eur J Cancer. 2020;127:96-107.
- Referenced with permission from the NCCN Guidelines for Patients® for Soft Tissue Sarcoma 2023. © National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed July 24, 2023. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use, or application, and disclaims any responsibility for their application or use in any way.
- Cancer.Net. The oncology team. Accessed February 12, 2023. https://www.cancer.net/navigating-cancer-care/cancerbasics/cancer-care-team/oncology-team
- Cancer.Net. Counseling. Accessed February 12, 2023. https://www.cancer.net/coping-with-cancer/finding-social-support-and-information/counseling.
- Granek L, Nakash O, Ariad S, et al. Strategies and barriers in addressing mental health and suicidality in patients with cancer. Oncol Nurs Forum. 2019;46(5):561-571.
- Cancer.Net. Treating pain with medication. Accessed February 12, 2023. https://www.cancer.net/coping-with-cancer/physical-emotional-and-social-effects-cancer/managing-physical-side-effects/pain/treating-pain-with-medication.
- Husson O, Younger E, Dunlop A, et al. Desmoid fibromatosis through the patients’ eyes: time to change the focus and organisation of care? Support Care Cancer. 2019;27(3):965-980.
- NIH. National Cancer Institute. NCI dictionary of cancer terms: physical therapist. Accessed July 24, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/physical-therapist
- NIH. National Cancer Institute. NCI dictionary of cancer terms: genetic counselor. Accessed July 24, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/genetic-counselor
- NCI (National Cancer Institute). Definition of cell signaling. Accessed February 12, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/cell-signaling
- Kolb L, Yarrarapu SNS, Ameer MA, et al. Lipoma. [Updated 2022 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507906/
- Li M, Zhang L, Xu XJ, Shi Z, Zhao XM. CT and MRI features of tumors and tumor-like lesions in the abdominal wall. Quant Imaging Med Surg. 2019;9(11):1820-1839.
- Kasper B, Baumgarten C, Garcia J, et al. Desmoid Working Group. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol. 2017;28(10):2399-2408.
- Data on file. SpringWorks Therapeutics.
- Rosa F, Marinetti C, Piscopo F, et al. Multimodality imaging features of desmoid tumor: a head-to-toe spectrum. Insights into Imaging. 2020;11(1):1-3.
- Penel N, Coindre JM, Bonvalot S, et al. Management of desmoid tumours: a nationwide survey of labelled reference centre networks in France. Eur J Cancer. 2016;58:90-96.
- van Broekhoven DLM, Grünhagen DJ, den Bakker MA, van Dalen T, Verhoef C. Time trends in the incidence and treatment of extra-abdominal and abdominal aggressive fibromatosis: a population-based study. Ann Surg Oncol. 2015;22(9):2817-2823.
- Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92(6):947-964.
- Fiore M, Coppola S, Cannell AJ, et al. Desmoid-type fibromatosis and pregnancy: a multi-institutional analysis of recurrence and obstetric risk. Ann Surg. 2014;259(5):973-978.
- Lopez R, Kemalyan N, Moseley HS, Dennis D, Vetto RM. Problems in diagnosis and management of desmoid tumors. Am J Surg. 1990;159(5):450-453.
- Gurbuz AK, Giardiello FM, Petersen GM, et al. Desmoid tumours in familial adenomatous polyposis. Gut. 1994;35(3):377-381.
- Shinagare AB, Ramaiya NH, Jagannathan JP, et al. A to Z of desmoid tumors. AJR Am J Roentgenol. 2011;197(6):W1008-W1014.
- Gounder MM, Maddux L, Paty J, Atkinson TM. Prospective development of a patient-reported outcomes instrument for desmoid tumors or aggressive fibromatosis. Cancer. 2020;126(3):531-539.
- Riedel RF. Agulnik M. Evolving strategies for management of desmoid tumor. Cancer. 2022;128(16):3027-3040.
- Cancer.Net. Desmoid tumor: types of treatment. Accessed February 12, 2023. https://www.cancer.net/cancertypes/desmoidtumor/types-treatment.
- Kasper B, Baumgarten C, Bonvalot S, et al; Desmoid Working Group. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients’ and professionals’ expertise—a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative. Eur J Cancer. 2015;51(2):127-136.
- Crago AM, Denton B, Salas S, et al. A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg. 2013;258(2):347-353.
- Peng PD, Hyder O, Mavros MN, et al. Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients. Ann Surg Oncol. 2012;19(13):4036-4042.
- Janssen ML, van Broekhoven DL, Cates JM, et al. Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis. Br J Surg. 2017;104(4):347-357.
- Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3rd. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer. 2000;88(7):1517-1523.
- Ballo MT, Zagars GK, Pollack A, Pisters PWT, Pollock RA. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17(1):158-167.
- Easter DW, Halasz NA. Recent trends in the management of desmoid tumors. Summary of 19 cases and review of the literature. Ann Surg. 1989;210(6)765-769.
- Tsagozis P, Stevenson JD, Grimer R, Carter S. Outcome of surgery for primary and recurrent desmoid-type fibromatosis. A retrospective case series of 174 patients. Ann Med Surg (Lond). 2017;17:14-19.
- Fernandez M, Bell T, Tumminello B, Khan S, Zhou S, Oton A. Disease and economic burden of surgery in desmoid tumors: a review. Expert Rev Pharmacoecon Outcomes Res. 2023;23(6):607-618. doi: 10.1080/14737167.2023.2203915
- NCI (National Cancer Institute). Dictionary of cancer terms: cryoablation. Accessed February 12, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/cryoablation
- Cancer.Net. About clinical trials. Accessed February 12, 2023. https://www.cancer.net/research-and-advocacy/clinical-trials/aboutclinical-trials
- Charifa A, Azmat CE, Badri T. Lipoma Pathology. [Updated 2021 Dec 12]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK482343/