Desmoid tumors are rare, noncancerous tumors that develop in the body’s soft tissues. The head and neck are two possible, though less common, locations where desmoid tumors can occur.

These tumors do not metastasize — meaning spread — to other parts of the body. However, they can cause pain and other complications. Treatment requires care from experienced health care providers. Here are seven important things to know about desmoid tumors in the neck and head.

1. Neck and Head Desmoid Tumors Are Rare

Desmoid tumors are rare. In the U.S., only about 900 to 1,500 people are diagnosed with a desmoid tumors every year. Because they are so rare, it’s hard to know exactly how many cases affect the head and neck.

Anywhere from 31 percent to 72 percent of desmoid tumors develop outside of the abdomen or abdominal wall. These are known as extra-abdominal tumors. Extra-abdominal tumors can also appear in the hands, arms, or legs.

Desmoid tumors in the head and neck are estimated to make up 10 percent of extra-abdominal cases, though other studies suggest they account for 12 percent to 15 percent. Even on the higher end of the estimated ranges, this means only about 162 people in the U.S. are diagnosed with head and neck desmoid tumors each year.

Head and neck desmoid tumors are more frequent in children than adults. They make up 22 percent to 35 percent of all extra-abdominal desmoid tumor cases in children.

2. Neck Desmoid Tumors Are More Likely To Cause Pain

Desmoid tumors don’t always cause symptoms. Possible symptoms include:

• A lump
• Swelling
• Pain
• Loss of function

One study found zthat desmoid tumors in the neck and shoulder areas are more likely to cause pain than desmoid tumors in other locations.

3. Getting Diagnosed With a Desmoid Tumor Can Be Difficult

Desmoid tumors can look similar to other diseases, such as lipomas and sarcomas, which also occur in soft tissue and cause swelling or lumps. Incorrect diagnoses are common with rare diseases like desmoid tumors. Studies show that up to 30 percent to 40 percent of desmoid tumors are diagnosed incorrectly.

The diagnosis process usually starts with a physical exam. Then, you may have either a magnetic resonance imaging (MRI) scan or a computed tomography (CT) scan. MRI and CT scans can help identify features of desmoid tumors, which is helpful for diagnosis.

The only way to know for sure if you have a desmoid tumor is through a biopsy. In a biopsy, a tissue sample is removed using a needle or surgery. This biopsy is then evaluated by a doctor trained to examine tissue samples. Your biopsy may also be checked for genetic mutations (also called variants) linked to desmoid tumors.

Because desmoid tumors are frequently misdiagnosed, it’s a good idea to get a second opinion from a provider with expertise in desmoid tumors. A correct diagnosis is crucial for receiving the right treatment.

4. You May Not Need Treatment for a Neck or Head Desmoid Tumor

The progression of desmoid tumors can vary. In some cases, tumors may grow, shrink, disappear, or stay the same. Doctors can’t always predict how a tumor will progress.

In 20 percent to 30 percent of cases, desmoid tumors will shrink or disappear on their own (known as spontaneous regression). This is more common with desmoid tumors in the abdominal wall, although spontaneous regression happens in other locations as well. To avoid overtreatment or unnecessary complications, doctors now often recommend active surveillance (watchful waiting) instead of immediate treatment.

With active surveillance, your doctor will monitor the desmoid tumor and your symptoms over a period of time.

Because head and neck desmoid tumors are located in critical areas, you’re more likely to start active therapy sooner than with tumors in other parts of the body.

5. Medical Therapies Are the First Recommended Option

If your head or neck tumor is growing or causing more symptoms, medical therapies are usually the first-line option. Medical therapies include targeted therapies and chemotherapy to slow tumor growth.

In special cases, if medical therapies are not effective or well tolerated, or if you’re older, radiation therapy can serve as the first treatment option. Radiation uses X-rays to kill tumor cells and can sometimes be combined with surgery to prevent tumors from returning.

Surgery is also possible for head and neck desmoid tumors. However, people who are treated with surgery are at greater risk of their tumors coming back than people who don’t have surgery. Additionally, head and neck desmoid tumors are difficult to remove and may result in functional disability and changes to appearance.

Another treatment option is cryoablation, which uses extreme cold to suppress tumor growth and kill abnormal cells. Treatments like cryoablation have been shown to shrink the tumor and reduce pain, and they may be better tolerated than other treatments.

However, keep in mind that head and neck desmoid tumors are also very close to the brain and other important organs. For this reason, and due to limited testing, cryoablation and similar treatments for head and neck desmoid tumors are usually reserved for cases where other treatments have not worked.

6. There Are Special Considerations for Children With Head and Neck Desmoid Tumors

Head and neck desmoid tumors are more common in children. Head and neck desmoid tumors also seem to be more aggressive in children and recur (come back) around 20 percent to 30 percent of the time.

In children, head and neck desmoid tumors can interfere with normal development. They can grow quickly and impact vital structures. While surgery can be an option, it comes with a high risk of recurrence. Long-term follow-up care is recommended. Radiation therapy should be used with caution and only with older children, as it can impact development.

7. It’s Important To Talk to Your Doctors

It’s important to get care from health care providers who specialize in treating desmoid tumors. These providers are sometimes called sarcoma specialists. Sarcoma specialists are oncologists who treat sarcomas and soft tissue tumors like desmoid tumors. Talk to your care team about your treatment options. Be sure to ask what to expect during treatment and discuss the potential outcomes.

Find Your Team Today

MyDesmoidTumorTeam is the social network for people with desmoid tumors and their loved ones. On MyDesmoidTumorTeam, members come together to ask questions, give advice, and share their stories with others who understand life with desmoid tumors.

Do you have questions about your head and neck desmoid tumor? What treatment plan are you following? Share your experience in the comments below, or start a conversation by posting on your Activities page.