What is a desmoid tumor?

(DEZ-moyd TOO-mer) A type of soft tissue tumor that forms in fibrous (connective) tissue, usually in the arms, legs, or abdomen. It may also occur in the head and neck. Desmoid tumors are usually benign (not cancer).

What causes desmoid Tumours?

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.

What are the symptoms of a desmoid tumor?

Is a desmoid tumor life threatening?

Most desmoid tumors do not impact lifespan but they are very difficult to get rid of and can be painful to live with. Desmoid tumors growing in the abdomen can cause problems, such as blocking your intestines. It is important that your doctor monitor the growth of these tumors carefully.

Do desmoid tumors need to be removed?

If your desmoid tumor causes signs and symptoms, your doctor may recommend an operation to remove the entire tumor and a small margin of healthy tissue that surrounds it. But sometimes the tumor grows to involve nearby structures and can't be completely removed.

Desmoid Tumor Overview

What is the death rate of desmoid tumors?

Because desmoid tumors are so rare, it is hard to determine accurate survival rates, but 1 report found that more than 98% of patients were alive 5 years after their diagnosis.

What type of doctor treats desmoid tumor?

Surgical oncologists and orthopedic oncologists are doctors who specialize in treating desmoid tumors and other sarcomas using surgery.

What is the other name for desmoid tumor?

Desmoid tumors begin in cells called fibroblasts, which are the most common type of cells in connective tissue. Fibroblasts also play an important role in wound healing. Desmoid tumors are sometimes referred to as aggressive fibromatosis, desmoid fibromatosis, or deep fibromatosis. They can occur in adults or children.

What is the new treatment for desmoid tumors?

High-frequency ultrasound. This emerging, noninvasive therapy uses focused ultrasound beams to target and destroy a desmoid tumor without harming nearby tissue.

How big can desmoid tumors get?

The median size of the desmoid tumor on cross-sectional imaging was 7.5 cm (range, 2.0–34.0 cm).

Can a desmoid tumor rupture?

We present a patient with a perforated intraabdominal desmoid tumor with hemoperitoneum and peritonitis mimicking acute appendicitis. To the best of our knowledge, this is the first such case reported in the literature.

How many people in the US have desmoid tumors?

Frequency. Desmoid tumors are rare, affecting an estimated 1 to 2 per 500,000 people worldwide. In the United States, 900 to 1,500 new cases are diagnosed per year.

What is the best hospital to treat desmoid tumors?

Mayo Clinic doctors are trusted and respected for their expertise in diagnosing and treating rare conditions, including desmoid tumors. Specialists draw on their experience caring for people with desmoid tumors to provide you with a personalized treatment plan.

Are desmoid tumors hereditary?

Hereditary desmoid disease usually presents as an extraintestinal manifestation of familial adenomatous polyposis (FAP; 175100), also known as Gardner syndrome, which is an autosomal dominant disorder caused by germline mutation in the APC gene.

Can desmoid tumors metastasize?

Desmoid tumor is called aggressive fibromatosis as it has similarities with a malignant (cancerous) tumor called fibrosarcoma. However, it is considered benign because it does not metastasize (spread) to other parts of the body.

Can desmoid tumors metastasis?

Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis, and also formerly termed fibrosarcoma grade I of the desmoid type) are locally aggressive tumors with no known potential for metastasis or dedifferentiation.

Can you see a desmoid tumor with ultrasound?

On ultrasound, desmoid tumors often have a variable appearance. They may appear as round, well-circumscribed, heterogenous masses, though they also commonly appear as irregular, spiculated, hypoechoic masses with color Doppler flow mimicking malignancy [1,5].

Can you tell if a mass is cancerous from an ultrasound?

Ultrasound images are not as detailed as those from CT or MRI scans. Ultrasound cannot tell whether a tumor is cancer. Its use is also limited in some parts of the body because the sound waves can't go through air (such as in the lungs) or through bone.

How long do you stay in hospital after tumor removal?

Everyone takes a different amount of time to recover. You might stay in hospital for around 3 to 10 days after surgery. How long you stay in hospital depends on your operation and how long you take to recover. As soon as it is safe, you will be allowed to go home where you continue to recover.

How many people in the US have desmoid tumors?

Frequency. Desmoid tumors are rare, affecting an estimated 1 to 2 per 500,000 people worldwide. In the United States, 900 to 1,500 new cases are diagnosed per year.

How long can you live with giant cell tumor?

The data on long-term prognosis of metastatic GCT (mGCT) are mainly limited to small retrospective studies. The largest of them, recently published by Yang et al., found a 94.4% 5-year survival rate based on data from 42 patients (21).

How big can desmoid tumors get?

The median size of the desmoid tumor on cross-sectional imaging was 7.5 cm (range, 2.0–34.0 cm).

What is the recurrence rate of desmoid tumors?

Although desmoid tumors have few mitotic figures, their typical malignant features, such as a lack of distant metastatic potential, locally aggressive growth, and invasion of the surrounding tissues, make the full resection very hard. Besides, their recurrence rate was estimated ranging from 19% to 77% [1].