Objective: To explore the clinicopathologic features and differential diagnosis of extra-adrenal paraganglioma. Method:The clinical and histopathologic features of 15 cases of extra-adrenal paraganglioma confirmed by our department were analyzed and the related literatures was reviewed. Results: Among these 15 patients,5 were male and 10 were female aged from 16 to 74 years.The tumors were located in retroperitoneal,vertebral,neck, bladder,scrotum and vagina, respectively. Tumor were composed of oval or polygonal main cell and sustentacular cells which surrounded the main cell. The cells arranged in Zellballen or cellular. All cases were benign. Immunohistochemical investigations revealed main cells were positive for neuron specific endolase, chromogranin A, synaptophysin, but negative for cytokeratin,epithelial membrane antigen. Sustentacular cells were positive for S-100 protein. Conclusion: Extra-adrenal paraganglioma can arise in any location in adult tissues. Awareness of the existence of extra-adrenal paraganglioma in unusual locations will reduce errors in diagnosis.
 TISCHLER A S,PACAK K,EISENHOFER G.The adrenal medulla and extra-adrenal paraganglia:then and now[J].Endocr Pathol,2014,25(1):49-58.
 CORSSMIT E P,ROMIJN J A.Clinical management of paragangliomas[J].Eur J Endocrinol,2014,171(6):231-243.
 LAM A K.Update on adrenal tumours in 2017 World Health Organization (WHO) of endocrine tumours[J].Endocr Pathol,2017,28(3):213-227.
 MENG L,WANG J,FANG S H.Primary pancreatic paraganglioma:a report of two cases and literature review[J].World J Gastroenterol,2015,21(3):1036-1039.
 BURA R,MANCA A,AMBU R,et al.Gastric paraganglioma:case report and review of the literature[J].G Chir,2017,38(2):84-89.
 LEVY M T,BRAUN J T,PENNANT M,et al.Primary paraganglioma of the parathyroid:a case report and clinicopathologic review[J].Head Neck Pathol,2010,4(1):37-43.
 HU K,PERSKY M S.Treatment of head and neck paragangliomas[J].Cancer Control,2016,23(3):228-241.
 GUNAWARDANE P T K,GROSSMAN A.Phaeochromocytoma and paraganglioma[J].Adv Exp Med Biol,2017,956:239-259.
 WASSERMAN P G,SAVARGAONKAR P.Paragangliomas:classification,pathology,and differential diagnosis[J].Otolaryngol Clin North Am,2001,34(5):845-862.
 SO J S,EPSTEIN J I.GATA3 expression in paragangliomas:a pitfall potentially leading to misdiagnosis of urothelial carcinoma[J].Mod Pathol,2013,26(10):1365-1370.
 TURCHINI J,CHEUNG V K Y,TISCHLER A S,et al.Pathology and genetics of phaeochromocytoma and paraganglioma[J].Histopathology,2018,72(1):97-105.
 ZI J,MA C,XU C,et al.A pediatric malignant paraganglioma and brief review of the literature[J].Hell J Nucl Med,2016,19(3):281-284.
 ROMAN-GONZALEZ A,JIMENEZ C.Malignant pheochromocytoma-paraganglioma:pathogenesis,TNM staging,and current clinical trials[J].Curr Opin Endocrinol Diabetes Obes,2017,24(3):174-183.
 PIERRE C,AGOPIANTZ M,BRUNAUD L,et al.COPPS,a composite score integrating pathological features,PS100and SDHB losses,predicts the risk of metastasis and progression-free survival in pheochromocytomas/paragangliomas[J].Virchows Arch,2019,474(6):721-734.