Surgical Extirpation of a Huge Desmoid Fibromatosis of the Right Buttock: Case Report of a Successful International Collaboration

Abstract

The rate of an accurate diagnosis for pediatric cancer is said to be low in developing countries; furthermore, due to inconvenient medical access and economic reasons, there are restrictions on the selection of treatments. Desmoid fibromatosis (DF) is a locally progressive, non-metastasizing fibroblast soft tissue tumor classified as intermediate type. We made a pathological diagnosis gross total tumor resection by the international collaboration with Myanmar, Cambodia, and Japan. The case was a 5-year-old Burmese boy. A recurrent growing tumor was found in his right buttock, which caused gait disturbance and aesthetic problems. In order to remove this giant DF, the oncologist consulted our department. Because DF had localized outside the pelvic ring, it could be resected with the embolization of the branch of the internal iliac artery. In operation, the sciatic nerve was preserved and a gross total resection was performed with R1 surgery. The patient is alive without any functional disorders and recurrences and is maintaining a good QOL. This is the first report on treating DF in Myanmar by an international collaboration team.