外科ジャーナル

A Rare and Interesting Case of Mediastinal Fibromatosis

Abstract

Taneja V, Tatavarthy S and Gupta A

Mediastinal fibromatosis is a rare entity affecting individuals of all age groups, and is extremely rare in children. Fibromatosis can occur anywhere in the body; most commonly in the superficial soft tissues, extremities, or abdomen. Bilateral pleural and mediastinal fibromatosis extending to neck along the tissue planes has not previously been reported in the literature. Most fibromatoses are benign but 10-20% can undergo malignant transformation. These tumours are initially asymptomatic, but can present with compressive symptoms as they grow in size. We present a rare case of fibromatosis of neck and mediastinum which developed in a 4 year old boy who had undergone cardiac surgery 2 years previously. Diagnostic dilemma and challenges in surgical removal are outlined.

This report documents an extremely rare case of anterior mediastinal fibromatosis, desmoid type that occurred immediately after cardiac surgery in a young child.