What is Aggressive Fibromatosis?
Also known as a desmoid tumor, aggressive fibromatosis is a benign but locally aggressive soft tissue tumor arising from the musculoaponeurotic structures.
How common is Aggressive Fibromatosis (Desmoid Tumors)?
The annual incidence is estimated to be 0.2-0.4 per 100,000 with two incidental peaks; 1) between ages 5 and 15; and 2) around age 40.
Our Patient’s Story
Our patient was 9 months old when she presented with a large non-tender mass of her right mandible.
On MRI, there was a large mass originating from her right mandble (red arrow) causing tracheal deviation (yellow arrow). Given how rapidly the mass was growing and compromising her airway, she underwent a parital mandibulectomy to achieve clear margins and reconstruction with rib and split calvarial grafts.
Postoperatively, she went on to heal well without recurrence.
Given how desmoid tumors are locally aggressive and tend to have a high rate of recurrence, it is very important to seek the expertise of a specialist who can obtain adequate margins to reduce recurrence and can perform proper reconstruction to reduce morbidity. Dr. Kelley has over 13 years of experience in Austin, Texas treating pediatric craniofacial tumors. Visit https://www.craniofacialteamtexas.com/craniofacial-conditions-we-treat/craniofacial-tumors/ to learn more!