As researchers learn more about desmoid tumor, treatment guidelines continue to change. The 2023 updated global evidence-based consensus guideline helps doctors make treatment decisions for desmoid tumor. The approach considers factors such as tumor location, symptoms, and how the tumor behaves.

This article reviews updates from this global consensus paper for managing desmoid tumor. We’ll discuss the general treatment path, the role of surgery, and treatment advances.

How Do Doctors Choose a Desmoid Tumor Treatment Plan?

The Desmoid Tumor Working Group (DTWG) is a group of doctors, researchers, and people living with desmoid tumor. They developed treatment guidelines based on published research. The group first published these recommendations in 2020 and updated them in 2023. That version was published in 2024.

The First Step Usually Involves Active Surveillance

For decades, surgery was the main treatment for desmoid tumor. However, studies showed that tumors often come back after surgery and that surgery may not improve symptoms over time. Now, doctors often recommend watching and waiting instead of having surgery right away.

Doctors first look at whether a tumor is life-threatening or affects a person’s quality of life or daily function. If it does not, the preferred approach is active surveillance. This means your doctor monitors your tumor for signs of growth but doesn’t start treatment.

Active surveillance usually lasts one to two years. If the tumor stays the same size or shrinks, your doctor will continue to monitor it. However, if the tumor starts to grow, they may recommend treatment. Desmoid tumors that cause serious problems, such as bleeding or organ issues, may need a different treatment approach.

Desmoid Tumor Treatment Is Based on Location

Desmoid tumors can form in many parts of the body. The location usually guides the type of treatment your doctor recommends.

Abdominal Wall Tumors

If you have a desmoid tumor in the abdominal wall that needs treatment, your doctor may consider surgery, ablation, or medical therapy. Surgery may be appropriate in this area because it usually carries lower risk than in other areas.

Radiation (also called radiotherapy), which uses high-energy beams to shrink tumors, may be a secondary treatment option.

Intra-Abdominal Tumors

An intra-abdominal desmoid tumor grows inside the abdomen. According to the consensus guidelines, first-line treatment may include medication or, in rare cases, surgery.

For people with a genetic syndrome that raises the risk of desmoid tumor, surgery is typically used only to treat complications. The guidelines recommend that treatment decisions be made by a multidisciplinary team and take into account the risks of surgery.

Extra-Abdominal Tumors

Extra-abdominal desmoid tumors grow in areas such as:

• Extremities (arms and legs)
• Chest wall and chest cavity, which contain the heart and lungs
• Head and neck
• Girdles, such as the shoulder or hip area

Medication and ablation are often the first treatment options for extra-abdominal tumors, except for tumors in the head and neck. For those, medication is usually the first treatment.

If the tumor keeps growing during drug treatment, your doctor may try a different medication. If that doesn’t stop progression, they may consider:

• Surgery (if it’s unlikely to cause more damage)
• Radiation therapy
• Ablation (for head and neck tumors)
• Isolated limb perfusion, which delivers chemotherapy directly to the affected leg or arm

How Has Surgery Changed in Desmoid Tumor Treatment?

Surgery used to be the main treatment, but years of research show this approach may not be as effective as once thought. Desmoid tumors can grow back after surgery, sometimes more aggressively. There’s also a chance a tumor may shrink on its own without treatment. Today, surgery is usually avoided unless it’s considered low risk or needed to avoid complications.

To help prevent complications, surgery may sometimes be used for desmoid tumors growing in the mesentery — the tissue that connects and supports organs in the abdomen. Tumors there can cause bleeding and bowel blockages.

Doctors may also recommend surgery if a tumor keeps growing during active surveillance or despite drug treatment. Surgery is most often used for superficial tumors, especially those in the abdominal wall.

Cryoablation Is an Effective Treatment for Desmoid Tumors

Cryoablation uses extremely cold gas to destroy tumor tissue. It’s commonly used to treat sarcomas (soft tissue cancers). The DTWG noted in its recent guidelines that this approach is a promising treatment for desmoid tumor.

Research shows cryoablation works best for small- to medium-sized extra-abdominal tumors. It may shrink the tumors, reduce pain, and improve function. Cryoablation may not be suitable for large tumors near vital organs and nerves. Your doctor may suggest this treatment if other medications haven’t worked.

How Have Medications for Desmoid Tumor Changed Over Time?

Medications are systemic therapies that treat desmoid tumor throughout the body. Several medications are used, but just one — nirogacestat (Ogsiveo) — is approved by the U.S. Food and Drug Administration (FDA) for desmoid tumor. The DTWG also recommends certain treatments based on research. Chemotherapy drugs, tyrosine kinase inhibitors (TKIs), and nirogacestat show the most promise.

Health Experts Recommend Some Traditional Treatments More Than Others

In the past, treatment often included chemotherapy drugs used for cancers. Doctors may also prescribe tyrosine kinase inhibitors, which are targeted therapies that block specific proteins in tumor cells. Examples of TKIs include sorafenib and pazopanib.

The DTWG notes that studies show TKIs help control desmoid tumor growth. Many people respond well to sorafenib and pazopanib. Imatinib is another option, but it may be less effective.

Chemotherapy is also used, usually at low doses. Common options include:

• Methotrexate
• Pegylated liposomal doxorubicin
• Vinblastine
• Vinorelbine

Low-dose chemotherapy has shown promising results. From 35 percent to 40 percent of people respond to treatment, and 50 percent to 70 percent may have tumor shrinkage over time. If the desmoid tumor grows back, chemotherapy may be used again.

Nirogacestat Is FDA-Approved for Desmoid Tumor

In 2023, the FDA approved nirogacestat for desmoid tumor. In clinical trials, researchers found that nirogacestat slowed tumor growth more than a placebo (inactive treatment).

Nirogacestat may become a first-line treatment in the future. However, there isn’t enough evidence yet for medical experts to agree on the best order for using these medications. Doctors choose treatment based on the tumor and a person’s overall health. As more research becomes available, these recommendations may continue to change.

Talk With Your Doctor

Treatment recommendations for desmoid tumor have changed in recent years. If you want to learn more about your options, talk with your doctor. They can explain the latest advances and whether any changes to your treatment plan may be right for you.

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