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.

Surgical management of a hepatic hydatid cyst fistulized into the duodenum: A case report

“…Few cases in the literature reported the use of upper gastrointestinal endoscopy to diagnose cysto-duodenal fistula [17] , [18] .…”

Desmoid-type fibromatosis of the splenic hilum: A rare tumor at an unusual location

“…Fibromatosis can be categorized into superficial and DTF (also known as deep fibromatosis, aggressive fibromatosis, or desmoid tumor). [ 4 ] DTF is a rare fibroblastic neoplasm with an annual incidence of 3–4 cases/million. [ 2 3 5 ] It accounts for approximately 3% of soft-tissue tumors.…”

“…Depending on the localization, it can be categorized into extra-abdominal (50%–60%), abdominal wall (25%), and intra-abdominal fibromatosis (12%–15%). [ 4 ] Reported cases of DTF are usually into the abdominal cavity, arising from the mesentery or pancreatic tissue. To our knowledge, this is the second case at the splenic hilum and first described in a patient with oncological history.…”

“…Conventionally, complete surgical resection has been the aim in the treatment of DTF and it remains the ideal treatment when R0 resection is possible. [ 4 ] However, wide resections may lead to large tissue defects and functional impotence or even short-bowel syndrome. In that sense, many nonsurgical treatments have been tried to minimize surgical consequences, but the results are still not conclusive.…”

“…[ 1 2 ] Sporadic DTF has a young female predominance, whereas PAF-associated DTF has no gender predilection. [ 2 3 4 ]…”