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 healthcare providers.

Here are seven important things to know about desmoid tumors in the neck and head.

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Desmoid tumors are rare. In the U.S., only about 1,000 people are diagnosed with 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. Because these tumors are so rare and estimates vary between studies, it is hard to know exactly how many people in the U.S. are diagnosed with them 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.

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

• A lump
• Swelling
• Pain
• Loss of function

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

Desmoid tumors can look like other conditions, 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 can help with 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.

The tissue is then evaluated by a doctor trained to examine biopsy samples. Your biopsy may also be tested for genetic mutations (variants) linked to desmoid tumors.

Because desmoid tumors are frequently misdiagnosed, you may want to get a second opinion from a provider with expertise in desmoid tumors. A correct diagnosis is crucial to getting the right treatment.

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 shrink or disappear on their own (spontaneous regression). This is more common with desmoid tumors in the abdominal wall, although spontaneous regression happens in other areas too.

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 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.

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 aren’t 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 coming back.

Surgery is also possible for head and neck desmoid tumors, though it’s not usually a first approach. This is because desmoid tumors often come back after they’ve been removed. Head and neck desmoid tumors are also difficult to remove and may result in functional disability and changes to appearance.

Another treatment option is cryoablation, which uses extreme cold to shrink the tumor and reduce pain.

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.

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 affect 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 affect development.

Seek care from healthcare 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.