Desmoid type fibromatosis of the distal pancreas: A case report

Abstract

A 23-year-old Korean female presented epigastric pain of two-months' duration. She had a laparoscopic ovarian cyst excision 8 months previously. Clinical examination was normal. An abdominal computed tomogram (CT) demonstrated a 10-cm solid mass in the distal pancreas, with signs of splenic artery and vein occlusion, gastric and transverse colon invasion. Operative findings showed a mass involving distal pancreas, invasive to the posterior wall of the antrum of the stomach and transverse colon and 4th portion of the duodenum without lymph node involvement. The surgery consisted of a distal pancreatectomy, splenectomy and combined partial resection of the stomach, transverse colon and 4th portion of the duodenum. The immunohistochemistry and histopathological features were consistent with a confirmed diagnosis of intra-abdominal desmoid type fibromatosis (DTF). The prognosis of pancreatic DTF is not known and she showed no recurrence or distant metastasis during a 3 year follow-up. Herein we report a rare case with an isolated, sporadic, and non-trauma-related DTF, located at the pancreatic body and tail.

A desmoid-type fibromatosis in the retroperitoneum of the gastro-pancreatic region that was resected with a distal pancreatomy: a case report

“…A DTF is a distinct rare entity that accounts for 0.03% of all tumors and 3% of all soft tissue tumors, with an incidence of 2-4 cases per million per year in the general population [8] . The intra-abdominal type is around 8% of all DTFs [9] .…”

“…Most DTFs arise sporadically; however, between 5% and 15% are associated with a familial adenomatous polyposis [5] . A history of trauma to the site of the tumor, often surgical in nature, is noted in approximately 25% of the cases [8 , 11] .…”

“…On CT, a DTF usually appears as a well-circumscribed solid mass with homogeneous density. Contrast-enhanced CT demonstrates typical isoattenuation to hyperattenuation to muscle, commonly demonstrating mild to moderate enhancement [8] . The soft tissue component often appears similar to a solid tumor, such as a gastrointestinal stromal tumor, lymphoma, or soft tissue sarcoma [13] .…”