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Primary pancreatic paraganglioma: a case report and literature review
Shengrong Lin1, Long Peng1, Song Huang2
1Department of General Surgery, The First Affiliated Hospital of Nanchang University, No.17 Yongwai Zhengjie, Nanchang, 330006, Jiangxi, China.
Backgroud:
Primary pancreatic paraganglioma is an extremely rare extra-adrenal paraganglioma.
Case Presentation:
We report a case of primary pancreatic paraganglioma undergoing middle segment pancreatectomy in a 42-year-old woman. Histological examination showed that the tumor was composed of well-defined nests of cuboidal cells separated by vascular fibrous septa, forming the classic Zellballen pattern. The chief cells showed positive staining to neuron-specific enolase, chromogranin A, synaptophysin, and the chief cells were surrounded by S-100 protein-positive sustentacular cells. The patient has remained tumor free for 12 months after surgery. A brief discussion about the histopathological features, clinical behavior, and treatment of primary pancreatic paraganglioma, and review of the relevant literature is presented.
Conclusions:
Primary pancreatic paraganglioma is a rare clinical entity, its diagnosis mainly depends on histopathological and immunohistochemical examinations. Complete surgical resection is the first choice of treatment and close postoperative follow-up is necessnary.
