Desmoid Tumors

Overview

What are desmoid tumors?

Desmoid tumors are fibrous growths that begin in your connective tissue. They commonly occur in the arms, legs or abdomen but they can develop anywhere in your body. Desmoid tumors may also be called deep fibromatosis, aggressive fibromatosis or desmoid fibromatosis.

What is the difference between desmoid tumors and soft tissue sarcoma?

Desmoid tumors are related to a type of cancer called soft tissue sarcoma. Soft tissue sarcomas can metastasize (spread to other parts of the body), while desmoid tumors do not spread. However, desmoid tumors can be aggressive, and may grow into neighboring structures. For this reason, cancer doctors usually care for people with desmoid tumors.

Who do desmoid tumors affect?

Desmoid tumors most commonly occur in adults aged 20 to 40. They can occur in people of all ages, but they are much rarer in children and adults over the age of 40. Desmoid tumors are more common in females compared to males, and they often occur shortly after childbirth.

How common are desmoid tumors?

Desmoid tumors affect approximately 2 to 4 million people every year. They make up about 0.03% of all tumors.

How do desmoid tumors affect my body?

While most desmoid tumors don’t impact your overall lifespan, they can still be painful and difficult to treat. Abdominal desmoid tumors may interfere with organ function and cause internal bleeding. In very rare instances, desmoid tumors can be life-limiting.

Desmoid tumors are also unpredictable. While some grow rapidly, others may shrink and go away on their own.

Symptoms and Causes

What are desmoid tumor symptoms?

The most common desmoid tumor symptom is a lump or area of swelling. This can occur anywhere in your body, but it happens most often in your arms, legs and abdomen. Other warning signs include:

  • Pain.
  • Nausea and vomiting, especially if you have an abdominal desmoid tumor.
  • Loss of function in the affected area.

What causes desmoid tumors?

A desmoid tumor forms when a connective tissue cell develops changes in its DNA. But experts aren’t sure what causes this process to begin with. Most desmoid tumors are associated with a mutation of the CTNNB1 gene, and many of these appear during or after pregnancy. Some desmoid tumors are linked to previous injuries. In rare instances, desmoid tumors are associated with familial adenomatous polyposis (FAP), a genetic condition that’s linked to colon cancer. Approximately 10% of people with FAP develop a desmoid tumor at some point in their lives.

Diagnosis and Tests

How are desmoid tumors diagnosed?

Your healthcare provider will perform a physical examination and ask you about your symptoms. If they suspect that you have a desmoid tumor, they may recommend imaging tests — such as a CT scan or MRI — to determine how much the mass has grown. They may also perform a biopsy. During this procedure, a tissue sample is removed and sent to a pathology lab for further analysis.

Management and Treatment

How are desmoid tumors treated?

Specific treatment depends on several factors, including the size and location of the tumor and your personal preferences. Because desmoid tumors can potentially grow into surrounding structures, much like cancerous growths, they’re often treated in a similar fashion. Common desmoid tumor treatments include:

  • Surveillance of the tumor. If your desmoid tumor isn’t causing symptoms, your healthcare provider may simply monitor the growth. Some desmoid tumors never grow and may not require treatment at all.
  • Surgery. Sometimes it’s possible to remove the entire desmoid tumor. This is recommended when the tumor causes troublesome symptoms, such as pain or loss of function. In other instances, only part of the tumor may be removed, especially if it has already invaded nearby structures.
  • Cryoablation. This procedure uses extreme cold to kill targeted tissue. It’s commonly used for certain types of cancer — including cancers of the lung, liver and prostate — but it’s also effective when treating desmoid tumors.
  • Chemotherapy. Oral or intravenous chemotherapy drugs may be used to shrink or eliminate desmoid tumors.
  • Radiation therapy. This treatment uses high-powered X-ray beams to kill problematic cells. Radiation therapy may be used as a standalone treatment or in combination with surgery.
  • Targeted drug therapy. If other treatments are unsuccessful, targeted drug therapy may be recommended. This approach uses medications to identify and fight certain types of cells.

Are there complications regarding desmoid tumor treatment?

Like any procedure, desmoid tumor treatments carry the risk of complications. Side effects may include (but are not limited to):

  • Loss of appetite.
  • Pain.
  • Fertility issues.
  • Anemia.
  • Infection.
  • Fatigue.

How long does it take to recover from desmoid tumor treatment?

Recovery times can vary drastically depending on the treatment you receive. People who undergo surgery usually require two to three weeks of healing. If you receive chemotherapy or radiation, treatment could take several months, and it may be several more months before you feel like yourself again.

Prevention

Can I prevent desmoid tumors?

Because desmoid tumors are usually associated with genetic conditions, there isn’t anything you can do to prevent them.

Outlook / Prognosis

What can I expect if I have a desmoid tumor?

If you’ve been diagnosed with a desmoid tumor, your healthcare provider will discuss your treatment options with you. If your tumor isn’t causing problems, your provider may monitor your condition and only recommend treatment if the tumor grows. If your tumor is causing painful symptoms, then surgery, chemotherapy, radiation therapy, cryoablation or targeted drug therapy will likely be recommended.

People who’ve been treated for desmoid tumors in the past will likely need to see their healthcare providers for routine follow-ups. During these visits, your provider will look for signs of recurrence and recommend treatment if necessary.

Can desmoid tumors be fatal?

Desmoid tumors are considered benign (noncancerous) because they don’t spread to other areas of your body. However, sometimes desmoid tumors grow aggressively, affecting nearby structures. In very rare instances, they can be fatal. Desmoid tumors of the head, neck and abdomen are most likely to cause life-threatening problems.

What is the desmoid tumor life expectancy?

According to the American Society of Clinical Oncology (ASCO), more than 98% of people with desmoid tumors are still alive five years after their diagnosis. Survival rates depend on several factors, including the size and location of the tumor and how well your body responds to treatment. To learn more about survival rates, talk to your healthcare provider.

Living With

When should I see my healthcare provider?

Any time you notice a new lump or bump on your body, you should schedule a visit with your healthcare provider. It’s also important to understand that desmoid tumors can grow back. So, if you’ve been treated for one in the past, be sure to call your doctor any time you notice new symptoms.

What questions should I ask my healthcare provider?

If you’ve been diagnosed with a desmoid tumor, here are some questions to consider asking your healthcare provider:

  • Exactly where is my tumor located?
  • Is my tumor affecting any of my organs or body functions?
  • Can we monitor the tumor? If so, for how long?
  • What are my treatment options?
  • How long will my treatment last?
  • What can I expect in terms of recovery?

A note from Cleveland Clinic

Desmoid tumors are noncancerous and do not spread. However, in many cases, they can still cause significant pain and other serious health problems. If you develop pain, nausea, loss of function or other symptoms, call your healthcare provider right away. They can determine a diagnosis and recommend an appropriate treatment plan.

Last reviewed by a Cleveland Clinic medical professional on 11/16/2021.

References

  • Cancer.Net. Desmoid Tumor: Statistics. (https://www.cancer.net/cancer-types/desmoid-tumor/statistics) Accessed 10/26/21.
  • National Cancer Institute. Desmoid Tumor. (https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor) Accessed 10/26/21.
  • National Organization for Rare Disorders. Desmoid Tumor. (https://rarediseases.org/rare-diseases/desmoid-tumor/) Accessed 10/26/21.
  • Redifer Tremblay K, Lea WB, Neilson JC, King DM, Tutton SM. Percutaneous cryoablation for the treatment of extra-abdominal desmoid tumors. (https://pubmed.ncbi.nlm.nih.gov/31236956/) J Surg Oncol. 2019 Sep;120(3):366-375. doi: 10.1002/jso.25597. Epub 2019 Jun 24. PMID: 31236956; PMCID: PMC6790665. Accessed 10/26/21.
  • U.S. National Library of Medicine. Desmoid tumor. (https://medlineplus.gov/genetics/condition/desmoid-tumor/) Accessed 10/26/21.
  • Wang Z, Wu J, Tian X, Hao C. Targeted therapy of desmoid-type fibromatosis: mechanism, current situation, and future prospects. (https://pubmed.ncbi.nlm.nih.gov/30798508/) Front Med. 2019 Aug;13(4):427-437. doi: 10.1007/s11684-018-0672-6. Epub 2019 Feb 22. PMID: 30798508. Accessed 10/26/21.

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