Idiopathic desmoid-type fibromatosis of the pancreatic head: case report and literature review

Abstract

Desmoid-type fibromatosis (DTF) is an uncommon nonmetastatic fibrous neoplasm. Sporadic intraperitoneal DTF is rarely described in current literature. We herein report a case of DTF of unknown cause involving the pancreatic head. A 41-year-old man presented with recurrent epigastric pain and weight loss. An abdominal computed tomography scan showed a well-delineated solid cystic mass inside the pancreatic head. Pylorus-preserving pancreaticoduodenectomy was performed due to the patient’s debilitating symptoms and suspected malignancy. The pathological examination revealed massive fibroblastic proliferation arising from the musculoaponeurotic tissues, consistent with a diagnosis of DTF. Immunohistochemical phenotyping determined positive immunoreactivity to vimentin and β-catenin, but negative immunoreactivity to smooth muscle actin, CD117, CD34, or S-100, confirming the diagnosis of DTF. No local recurrence or distant metastasis was found during a 24-month follow-up. Radical resection is recommended as first-line treatment for pancreatic DTF. Long-term follow-up studies are required to establish the prognosis of pancreatic DTF.

Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1—Benign Tumors:From the Radiologic Pathology Archives

“…Only 10 cases of desmoid tumors of the pancreas have been described in the English literature over the past 3 decades (14,15). The tumors have no known metastatic potential but may invade adjacent structures (16).…”

“…Surgery is the mainstay of treatment of desmoid tumors, with clear margins constituting a cure. However, there is a high likelihood of recurrence, ranging from 19% to 77%, with a higher rate in patients with familial adenomatous polyposis or Gardner syndrome (15).…”

A Sporadic Desmoid Tumor: an Exceptional Pancreatic Cystic-Solid Mass

“…Pancreatic DTs are rare, with only 10 cases having been reported in the last 3 decades in the English medical literature [4]. Almost all the cases occurred in middle-aged patients [5].…”

“…The first case of a pancreatic DT, reported in 1956 by Wilson, resembled a pseudocyst. Only 10 similar cases have been described in the English medical literature thus far [4].…”

An Intra-abdominal Solid-cystic Desmoid That Emerged after Distal Gastrectomy

“…The differential diagnosis of desmoid tumor includes neuroendocrine tumor, lymphoma, retractile sclerosing mesenteritis, gastrointestinal stromal tumor, and mesenteric metastases (11). Since desmoids scarcely cause necrosis (12) and bleeding (13), cystic formation of desmoids has rarely been reported; indeed, only 13 cases have been published in the English literature, as shown in Table (origin: 12 pancreas and 1 mesenterium) (14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26). Due to its rarity, preoperative diagnoses of solid-cystic desmoids are very difficult, and these lesions are often misdiagnosed as malignant tumors, such as a pancreatic cancer (14-16), a neuroendocrine tumor with cystic changes 17, a cystic neoplasm of the pancreas (18)(19)(20)(21)(22), and an inflamed teratoma (23).…”