32例腹膜后副神经节瘤的临床病理观察

Clinical and pathologic features of retroperitoneal paraganglioma: an observation of 32 cases

目的探讨腹膜后副神经节瘤的临床表现、病理特征及鉴别诊断。方法分析32例腹膜后副神经节瘤患者的临床特征、组织形态、免疫表型特点、治疗及预后,并复习相关文献。结果大部分患者以腰背酸痛为主要症状就诊,常伴有血压升高。副神经节瘤切面血管丰富,镜下肿瘤细胞胞浆丰富,部分嗜碱性,免疫组化CgA、Syn、S-100、Ki-67(2%～30%)阳性表达,Melan-A、CK均不表达。治疗以手术切除为主。获随访的13例患者均未见肿瘤复发转移。结论腹膜后副神经节瘤的定性诊断主要依靠形态学观察,免疫组化可以辅助诊断和鉴别。腹膜后副神经节瘤是潜在的恶性肿瘤,术后应长期随访。

Objective To investigate the clinical manifestations,pathologic features,differentiation and diagnosis of retroperitoneal paraganglioma. Methods The clinical manifestations,histopathologic morphology,immunohistologic features,treatment and prognosis were analyzed in 32 cases of retroperitoneal paraganglioma,and the literatures were reviewed. Results Low back pain was the main symptoms of retroperitoneal paraganglioma,and patients often accompanied by increased blood pressure. Blood vessels were rich in the neoplasm of retroperitoneal paraganglioma,and pathologic diagnosis showed that the cells of tumors were rich in cytoplasm,and some were basophilic. Immunohistochemical study showed that CgA,Syn,S-100,Ki-67（ 2%-30%） were positively expressed,while Melan-A and CK were negative. Surgical resection was the main treatment of this disease. Thirteen cases were followed up,and no recurrence and metastasis was found. Conclusion The diagnosis of retroperitoneal paraganglioma should be confirmed by morphologic features and immunophenotypes. Patients should be followed-up,and it has a potential of maligancy.