Sporadic, nontrauma-related, desmoid tumor of the pancreas: a rare disease-case report and literature review

散发性、非外伤相关性胰腺纤维样瘤:一例罕见病例报告及文献综述

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Abstract

Desmoid tumors (DTs) are neoplasms of fibroblastic origin characterized by lack of a capsule. They are nonmetastatic and locally aggressive. Intraabdominal DTs are often observed in familial adenomatous polyposis and Gardner syndrome or subsequent to localized traumatic injury. Sporadic forms are defined as nontrauma- or nongenetic-related DTs. Isolated, sporadic pancreatic DTs have been considered anecdotal, with only 9 cases described in the literature. We report the case of a 68-year-old man with a case of sporadic cystic DT localized to the pancreatic tail. The tumor was discovered incidentally during computerized tomography performed for an unrelated condition. The patient was asymptomatic; however, biopsy was performed on the clinical suspicion of cystic cancer of the pancreas. Pathology analysis showed fibroblastic proliferation, and the diagnosis of DT was confirmed by immunohistochemical staining for beta-catenin. The patient underwent resection with no further treatment and remain disease-free 60 months after surgery.

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