肾上腺外副神经节瘤临床病理分析

Extra-adrenal paraganglioma:a clinicopathologic study of 21 cases and literature review

目的探讨肾上腺外副神经节瘤临床病理学特征、分类、良恶性组织学判断标准及鉴别诊断。方法对21例肾上腺外(副交感神经和交感神经)副神经节瘤进行光镜及免疫组化观察,同时跟踪随访并复习相关文献。结果肾上腺外副神经节瘤21例,男性10例、女性11例,年龄9～81岁,平均年龄46.5岁。肿瘤位于腹膜后10例,头颈部7例,纵隔、膀胱、精索及肾脏各1例。有功能者3例,无功能者18例。组织学上肿瘤主要由主细胞和支持细胞两种细胞组成,主细胞排列成巢状、束状、腺泡状或实体样结构,周围被支持细胞部分或完全包绕。1例诊断为恶性,瘤细胞异型性显著,核分裂象多见,并见灶性或融合性坏死和血管侵犯,伴局部淋巴结转移,余20例诊断为良性。免疫组化肿瘤细胞表达NSE、CgA、Syn、NF,而CK、EMA和SMA阴性;支持细胞S-100蛋白阳性。1例恶性副神经节瘤仅NSE阳性,CgA弱阳性。随访18例,其中1例1年后因多脏器转移而死亡,另有1例组织学诊断为良性者3年后肿物复发并侵犯邻近器官。结论肾上腺外副神经节瘤组织形态学改变与其生物学行为不一,临床上应长期随访。同时应与形态相似的肿瘤鉴别。

Purpose To study the clinicopathologic feature,classification,benign or malignant histological features and differential diagnosis of extra-adrenal paraganglioma.Methods We performed a retrospective analysis of 21 cases of extra-adrenal （sympathetic and parasympathetic） paraganglioma.Histological,immunohistochemical findings,and follow-up data were reported with review of the relative literatures.Results There were 10 male and 11 female patients aged from 9 to 81 years （mean 46.5 years）.Ten tumors occurred in the retroperitoneum,seven in the neck,and one case in the mediastinum,urinary bladder,spermatic cord and kidney,respectively.There were 3 cases of functional and 18 cases of non-functional paragangliomas.Histologically,the tumor was composed of chief cells and sustentacular cells.The chief cells were arranged in nests,alveolar-like and stereo-like structures.They were surrounded by sustentacular cells partly or completely.There was one malignant case,in which tumor cells had significant cell atypia and nuclear mitosis,and focal or diffuse necrosis,regional lymph node metastasis and blood vessel involvement were also observed.Immunohistochemical investigations revealed chief cells were intensely positive for neuroendocrine markers such as NSE,CgA,Syn and NF,but negative for CK,EMA and SMA.Sustentacular cells were positive for S-100 protein.The malignant cells were only positive for NSE and weakly positive for CgA.Eighteen cases were followed up.One patient developed to multi-organ metastasis and died.One patient was diagnosed as benign extra-adrenal paraganglioma that recurred and involved adjacent organs after 3 years.Conclusions It is generally considered that malignant potential,local invasiveness and distant metastasis are not correlated with the histological appearances of the extra-adrenal paraganglioma.So,further follow-up will be needed.It is necessary to emphasize the differential diagnoses of extra-adrenal paraganglioma from other pleiomorphic tumors.