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Deep fibromatosis, aggressive fibromatosis, and desmoid-type fibromatosis are all names given to desmoid tumor, a rare condition. Desmoid tumors are locally aggressive, noncancerous tumors that form in the soft connective tissue.
The many medical terms can be confusing. It’s true that desmoid tumor is a type of fibromatosis. However, key differences separate these tumors from other types of fibromatosis.
In this article, we’ll cover the different types of fibromatosis and how desmoid tumors are similar to and different from them.
Fibromatosis refers to an overgrowth of connective tissue. The overgrowth creates tumors, or fibromas.
These tumors are typically not cancerous. This means that they don’t metastasize (spread to other parts of the body). Fibromas are made from tough or fibrous tissue.
Doctors typically divide fibromatoses into two groups — superficial and deep.
“Superficial” means the growths form near the surface of the skin. They often affect the hands, feet, or other areas just under the skin. These tumors are usually small, grow slowly, and tend to be less aggressive than deep fibromatoses.
Examples of superficial fibromatoses include:
Deep fibromatoses develop deeper in the body and may affect muscles or organs.
Desmoid tumor is a type of deep fibromatosis that forms inside the body. These tumors can affect the muscles, organs, and bones. They can grow large and spread into nearby tissues.
In some cases, desmoid tumors can block nerves and blood vessels. They can interfere with organ function and cause pain and discomfort.
Doctors classify desmoid tumor as a type of deep fibromatosis. The term “desmoid” comes from the Greek word “desmos,” which means “tendonlike.” This is because desmoid tumors look like tendons that connect muscles to bone.
Researchers aren’t quite sure what causes desmoid tumor. They believe that some people develop mutations (variations) in their DNA over time.
This is true for people with mutations in the catenin beta 1 (CTNNB1) gene. This gene gives instructions for making a protein called beta-catenin. Cells use beta-catenin to grow and divide.
Other people have a genetic condition known as familial adenomatous polyposis (FAP). People with FAP have changes in the adenomatous polyposis coli (APC) gene.
Some factors may increase your risk of developing a desmoid tumor. Examples of desmoid tumor risk factors include surgery, injury, and pregnancy.
These factors all cause trauma that triggers the body’s wound-healing process. People with abnormal beta-catenin proteins can’t shut this process off. Eventually, scar tissue builds up and forms desmoid tumors.
Unlike superficial fibromatoses, desmoid tumors tend to form deep within the body’s tissues. They include:
All fibromatoses develop in fibrous connective tissue. Research shows that both superficial fibromatoses and desmoid tumors have changes in growth signaling.
These abnormal growths also look similar under a microscope. However, there are some key differences between the two.
Desmoid tumors can involve mutations in the CTNNB1 and APC genes. People with superficial fibromatoses usually don’t have these changes. But researchers have found high beta-catenin levels in cells from some superficial fibromatoses.
This protein may be the reason superficial fibromatoses form. More research is needed to find out its exact role.
Studies show that superficial fibromatoses are also linked to genetics. Dupuytren’s contracture can be passed down through family members, but it can also develop randomly.
Knuckle pads are also linked to genetic syndromes that affect the skin. Unlike desmoid tumors, no specific genes play a role in these superficial fibromatoses.
Superficial fibromatoses usually stay where they grow. They rarely spread into other nearby tissues. If your doctor removes one, it likely won’t grow back.
On the other hand, desmoid tumors can be more aggressive. They can also grow very large.
Some tumors grow into surrounding tissues and keep growing. Surgery for desmoid tumor usually isn’t recommended because these tumors often come back.
Instead of surgery, doctors typically use other approaches to shrink desmoid tumors. Treatment options can include:
Many of these treatments also help manage malignant (cancerous) tumors. It’s important to note that desmoid tumors and fibromatoses don’t spread like cancer.
A person diagnosed with desmoid tumor may not need treatment. Instead, doctors may recommend active surveillance, or periodic appointments to check for tumor growth.
Treatment for superficial fibromatosis depends on the type and location of your growth. For instance, if you have a dermatofibroma, you may see a dermatologist (skin expert) and an oncologist (cancer and tumor specialist).
People with Dupuytren’s contracture may see an orthopedic surgeon if they need surgery. They may also see a radiation oncologist if radiation therapy is needed to shrink tumors.
Because desmoid tumor is rare, many doctors likely haven’t treated people with this condition. This makes it hard to get an accurate diagnosis. Many people living with desmoid tumor are misdiagnosed at some point in their journey.
The Desmoid Tumor Research Foundation recommends seeing a sarcoma specialist to ensure you get the best care possible. A sarcoma specialist is an oncologist who treats soft tissue tumors such as sarcoma (a type of cancer) or desmoid tumor.
The sarcoma specialist may be part of a larger desmoid tumor care team to manage your overall health.
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