Desmoid type Fibromatosis

Origin and Causes

Desmoid type fibromatosis is a benign but locally aggressive tumor. This means that the tumor does not have the ability to metastasize. About 10% of desmoid tumors are associated with a genetic predisposition syndrome called familial adenomatous polyposis, which can be detected through a colonoscopy. However, in the vast majority of patients with desmoid tumors, we do not find any risk factors.

 

Symptoms and Signs

Desmoid tumors can occur in any part of the body, primarily the abdominal wall, abdominal cavity, and extremities. They often present as a progressive mass that can be painful. They can sometimes cause limitations in movement of affected limbs. In the case of abdominal cavity desmoid tumors, an obstruction or intestinal bleeding can occur.

 

Diagnosis

When a desmoid tumor is suspected, the initial evaluation includes either a CT scan or MRI. The diagnosis is made by a biopsy of the suspected area.

 

Treatment

The evolution of desmoid tumors is unpredictable. Without treatment, about 20-30% of desmoid tumors can spontaneously regress, and another 30% may remain stable. Due to the morbidity and high risk of tumor recurrence after surgery, a majority of desmoid tumors are approached with an initial period of surveillance. Treatments are reserved only for patients with symptoms or progression in size of desmoid tumors. Depending on the location of the tumor, surgery or medical treatment may be considered. Current medical treatments include methotrexate-vinblastine, doxorubicin, liposomal doxorubicin, imatinib, sorafenib, and pazopanib. Radiation therapy may also be considered. Clinical trials may also be explored.

 

Follow-up

Following diagnosis, clinical imaging follow-up will be performed.