Lin Shengrong, Peng Long, Huang Song, Li Yong, Xiao Weidong
Department of General Surgery, The First Affiliated Hospital of Nanchang University, No.17 Yongwai Zhengjie, Nanchang, 330006, Jiangxi, China.
Department of Pathology, The First Affiliated Hospital of Nanchang University, No.17 Yongwai Zhengjie, Nanchang, 330006, Jiangxi, China.
World J Surg Oncol. 2016 Jan 22;14(1):19. doi: 10.1186/s12957-016-0771-2.
Primary pancreatic paraganglioma is an extremely rare extra-adrenal paraganglioma.
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.
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.
原发性胰腺副神经节瘤是一种极其罕见的肾上腺外副神经节瘤。
我们报告一例42岁女性原发性胰腺副神经节瘤行胰中段切除术的病例。组织学检查显示，肿瘤由界限清楚的立方形细胞巢组成，被血管纤维间隔分隔，形成典型的Zellballen模式。主细胞对神经元特异性烯醇化酶、嗜铬粒蛋白A、突触素呈阳性染色，主细胞被S-100蛋白阳性的支持细胞包绕。患者术后12个月无肿瘤复发。本文对原发性胰腺副神经节瘤的组织病理学特征、临床行为及治疗进行了简要讨论，并复习了相关文献。
原发性胰腺副神经节瘤是一种罕见的临床实体，其诊断主要依靠组织病理学和免疫组化检查。完整的手术切除是首选治疗方法，术后密切随访是必要的。
