Coexistence of duodenum derived aggressive fibromatosis and paraduodenal hydatid cyst: A case report and review of literature

Abstract

Intra-abdominal aggressive fibromatosis is a locally aggressive tumor mostly originating from the mesentery or retroperitoneal space, infiltrating adjacent tissues, and very rarely metastasizing to distant organs. There are only two case reports in the English language literature where intra-abdominal aggressive fibromatosis originated from the intestinal wall. In this study, we aimed to report a case of aggressive fibromatosis originating from the muscularis propria layer of the duodenum and invading pancreas. Another interesting aspect of this case is that a primary paraduodenal hydatid cyst was incidentally detected in the surgical specimen. A 46-year-old female patient presented to our clinic with postprandial nausea and vomiting. A contrast-enhanced abdominal computerized tomography revealed a mass lesion with a size of 100 mm × 80 mm which originated from the distal pancreas and compressed the gastric pilor externally. Upon exploration the distal part of duodenum, proximal jejunum, and pancreatic mass were noted to form a conglomerated structure. Therefore, the fourth part of the duodenum, a 25 cm part of the proximal jejunum, distal pancreas, and the spleen were excised en-bloc. The pathology report of the specimen indicated fibromatosis with a diameter of 55 mm that originated from the muscularis propria of the duodenum and extended into the pancreatic parenchyma. There was also an incidentally detected 10 mm paraduodenal hydatid cyst. No tumor recurrence was detected at a follow-up period of 24 mo. In conclusion, the most ideal treatment of desmoid-type fibromatosis is surgical resection of the mass lesion with clean surgical borders. Although rare, this tumor may originate from the intestinal wall. Histopathological verification is of great significance for a proper diagnosis.

Keywords: Duodenal wall; Hydatid cyst; Aggressive fibromatosis; Intra-abdominal fibromatosis; Desmoid tumor; Desmoid-type fibromatosis

Core tip: Fibromatosis can be categorized into two broad categories depending on their localization: superficial and deep (aggressive fibromatosis or desmoid tumor). Desmoid-type fibromatoses can be categorized into three groups depending on their localization, namely extra-abdominal, abdominal wall, and intra-abdominal fibromatosis. Intra-abdominal desmoid-type fibromatosis may develop from the small intestinal mesentery, omentum, retroperitoneum, pelvis, and very rarely, the intestinal wall (such as our case). We aimed to report a case of aggressive fibromatosis originating from the muscularis propria layer of the duodenum and the invading pancreas. Another interesting aspect of this case is that a primary paraduodenal hydatid cyst was incidentally detected.