腹膜后副神经节瘤6例临床病理分析

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。方法分析观察6例腹膜后副神经节瘤的临床症状、病理形态、免疫组化、治疗及预后,并进行文献复习。结果本组男性4例,女性2例,年龄20～65岁,平均38岁。以间歇性腹痛、腰痛及阵发性高血压为主要临床表现,有功能者2例。肿瘤体积均较大,镜下肿瘤主要由主细胞和支持细胞组成。免疫组化瘤细胞CgA和Syn弥漫强(+),支持细胞S-100(+)。4例随访12～14个月,均未见肿瘤复发及转移。结论腹膜后副神经节瘤临床经过隐匿,确诊主要靠组织学检查和免疫组化结果,治疗以手术彻底切除为主。

Retroperitoneal paraganglioma: a clinicopathological analysis of six cases

Objective To study the clinical manifestations,pathological features and diagnosis of retroperitoneal paragangliomas.Methods Clinical manifestations,pathological morphology and immunohistochemical staining were analyzed in 6 case of retroperitoneal paragangliomas,with review of related literatures.Results There were 4 male and 2 female patients aged from 20 to 65 years(mean 38 years).Intermittent abdominal pain and lumbago,episodic high blood pressure were the main clinical presentations.2 of them were functional paragangliomas.All the tumors presented with a bulk mass.Histologically,the tumor was composed of chief cells and sustentacular cells.Immunohistochemical investigations revealed that chief cells were intensely positive for Syn and CgA.Sustentacular cells were positive for S-100 protein.Four cases were followed up,and there was no evidence of recurrence and metastasis.Conclusions Most of retroperitoneal paraganglioms are asympomatic and their finding is incidental.The diagnosis is mainly based on the pathological features and immunohistochemical patterns.Complete resection is the standard of treatment for paraganglioma.