肾上腺外副神经节瘤47例临床病理分析

Extra⁃adrenal paraganglioma:a clinicopathologic analysis of 47 cases

目的探讨肾上腺外副神经节瘤临床病理特点、生物学行为。方法对安徽省立医院2013年1月至2018年12月47例肾上腺外副神经节瘤病例的临床表现、组织学特点及免疫组织化学染色结果进行分析。结果47例肾上腺外副神经节瘤,其中男性23例,女性24例,年龄范围为31~75岁,平均年龄51.0岁。24例位于腹膜后,20例位于头颈部,3例位于膀胱。1例位于腹膜后者伴有肝转移,其余46例术中未发现远处转移。肿瘤实质由主细胞和支持细胞组成。免疫组织化学染色显示主细胞表达嗜铬素A(CgA)、突触素(Syn)、神经细胞黏附分子56(CD56)、不表达细胞角蛋白(CK),支持细胞表达酸性钙结合蛋白(S⁃100)。与其余46例肾上腺外副神经节瘤相比,发生肝转移者无特殊病理形态学特征。结论肾上腺外副神经节瘤组织学形态与生物学行为不一。所有肿瘤均有转移潜能,需要长期随访。

Objective To investigate the clinicopathologic features and biological behaviors of extra⁃adrenal paraganglioma.Meth⁃ods Analysis was made of the clinical manifestations,histological characteristics and immunohistochemical results in 47 cases of extra⁃adrenal paraganglioma hospitalized in Anhui Provincial Hospital from January 2013 to December 2018.Results There were 23 male and 24 female patients aged 31 to 75 years(mean 51.0 years)in 47 cases.Twenty⁃four cases occurred in the retroperitone⁃um,20 cases in the head⁃and⁃neck region and 3 cases in the bladder.One paraganglioma case located in the retroperitoneum had liver metastasis,and the remaining 46 cases were not found with distant metastasis.The tumor was composed of chief cells and sus⁃tentacular cells.Immunohistochemical staining revealed chief cells intensely positive for neuroendocrine markers such as chromo⁃granin A(CgA),synaptophysin(Syn),neural cell adhesion molecule(CD56),but negative for cytokeratin(CK).Sustentacular cells were positive for S⁃100 protein.Compared with the remaining 46 cases of extra⁃adrenal paraganglioma,the patient with liver metastases showed no special pathological features.Conclusions The histological morphology and biological behavior of the adre⁃nal paraganglioma are different.All tumors have the potential of metastasis and require long⁃term follow⁃up.