卵巢幼年型粒层细胞瘤8例临床病理分析

Ovarian juvenile granulosa cell tumor:a clinicopathological study of 8 cases

目的探讨卵巢幼年型粒层细胞瘤(juvenile granulosa cell tumor,JGCT)的临床病理特点、诊断及鉴别诊断。方法回顾本院诊治的8例JGCT的临床、病理特征及免疫表型特点,并进行随访获知其预后情况。结果8例JGCT患者发病年龄6～21岁,平均15.1岁。临床主要表现为腹部包块、腹水及女性假性性早熟。巨检表现为囊实性肿块。光镜下肿瘤细胞呈实性巢状,片状弥漫性排列,部分可排列成多个圆形或椭圆形大小不等的滤泡,少数可形成巨滤泡结构,有的滤泡腔内还可见均质红染物质。瘤细胞呈圆形、多边形,中等大小或较大,胞质丰富,空淡或微嗜酸性,核圆形,染色质均质状,无明显核沟,有一定异型性,可见核分裂象。免疫表型:瘤细胞均表达inhibin-α、CD99、vimentin,部分病例Melan-A、calretinin、S-100阳性,瘤细胞不表达CKpan、EMA、PLAP、Syn和CgA。结论JGCT非常少见,属于低度恶性肿瘤,预后较好。确诊依赖于临床特点、组织形态学及免疫组化标记。病理诊断时要与卵巢的成人型粒层细胞瘤、高钙血症型小细胞癌、类癌、无性细胞瘤等肿瘤相鉴别。

Purpose To investigate the clinicopathologic features,diagnosis and differential diagnosis of ovarian juvenile granulosa cell tumor（JGCT）.Methods The history records,pathologic features and immunophenotype of 8 cases of JGCT were retrospectively evaluated and their prognosis was achieved by follow-up.Results The age of patients ranged from 6～21 years old,with an average age of 15.1 years.The main clinical manifestations included an abdominal mass,ascites and isosexual pseudoprecocity.Cut surface of the tumor was typically solid with cysts formed.The histopathological changes displayed solid nests,diffuse sheet,multiple round or ovoid follicles in variable size.Macrofollicles could be seen in some cases.The follicular pattern consisted of small cystic cavities containing eosinophilic secretions.The tumor cells were round or polygonal,medium in size.The tumor cells had abundant pale or slightly eosinophilic cytoplasm,round nuclei with fine chromatin.Nuclear grooves were inconspicuous.Mitosis figures could be found.Immunohistochemical results showed that the tumor cells expressed inhibin-α,CD99,vimentin;while Melan-A,calretinin and S-100 were positive staining in part of the cases.CKpan,EMA,PLAP,Syn and CgA were negative in all the cases.Conclusions Ovarian juvenile granulosa cell tumor is a rather rare,low malignant tumor with good prognosis.Its diagnosis depends on the histologic and immunohistochemical findings and clinical features.Its differential diagnosis includes adult granulose cell tumor,hypercalcaemic type small cell carcinoma, carcinoid and dysgerminoma.