An unusual finding in a desmoid-type fibromatosis of the pancreas: a case report and review of the literature

Summary

We describe the case of a 15-year-old boy, a Chinese national, who was diagnosed as having a pancreatic desmoid-type fibromatosis. He underwent a radical resection of the pancreatic mass, partial pancreatectomy, splenectomy, segmental resection of transverse colon and distal jejunum, and subsequently a one-stage jejunojejunostomy and colonic anastomosis. Postoperatively, he was diagnosed as having a desmoid-type fibromatosis of the pancreas by pathological and immunohistochemical studies. Pathological and immunohistochemical testing are essential for confirming diagnosis where demonstration of beta-catenin nuclear staining is probably the single most important characteristic. Other markers, such as vimentin, are usually positive, while S100 protein is usually negative. Our case however, confirms that there are rare cases that can be S100 positive. This is the first diagnosed case of pancreatic desmoid-type fibromatosis with S100 protein positivity.