Definition

Hereditary desmoid disease (HDD) is a condition that causes people to develop a benign (noncancerous) growth known as a desmoid tumor. Desmoid tumors may also be called fibromatosis.

Description

In HDD, multiple family members from several generations develop desmoid tumors. These tumors are very rare. They account for fewer than 0.1% of all tumors diagnosed. The term "desmoid" comes from the Greek word for "band." That describes these tumors well, as they have a tendon- or ligament-like appearance. They usually occur in the abdomen, but they may also develop in the neck, chest, arms, and legs.

Desmoid tumors may appear due to mutations, or changes, in a gene called adenomatous polyposis coli (APC). Most...