Primary pancreatic paraganglioma: a case report and literature review

Abstract

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.

Fig. 1

On contrast-enhanced CT, the tumor demonstrated marked enhancement in the arterial phase

Fig. 2

Pathology slides of the resected specimen with different stains. a Hematoxylin and eosin staining demonstrating the classic Zellballen pattern of paraganglioma (HE, ×400). b The chief cells showed positive staining to NSE (IHC, ×400). c The chief cells showed positive staining to Syn (IHC, ×400). d The sustentacular cells showed positive staining to S-100 (IHC, ×400)