Desmoid tumor overview

Desmoid tumor Microchapters
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Case #1

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2]

Overview

Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors. Desmoid tumors are tumors that arise from cells called fibroblasts. Fibroblasts are found throughout our body and their main function is to provide structural support and protection to the vital organs such as lung, liver, blood vessels, heart, kidneys, skin, intestines etc. and they also play a critical role in wound healing. When fibroblast cells undergo mutations they can become cancerous and become desmoid tumors (also known as "aggressive fibromatosis"). Desmoid tumors can arise in virtually any part of the body. These tumors often occur in women in their 30's, but can occur in anyone at any age. Desmoid tumors can be slow growing or extremely aggressive. These are slow-growing musculoaponeurotic tumors without any metastatic potential. However, aggressive fibromatosis is locally aggressive. When they are aggressive they can cause life threatening problems or even death when they compress vital organs such as intestines, kidney, lungs, blood vessels, nerves etc. Most cases are sporadic, but some are associated with familial adenomatous polyposis (FAP). Approximately 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors.