性母细胞瘤3例临床病理分析

Gonadoblastoma: a clinicopathological study of 3 cases

目的探讨性母细胞瘤的临床病理特征、肿瘤的性质和生物学行为。方法采用光镜、免疫组化对3例性母细胞瘤进行观察,并复习相关文献。结果3例患者分别表现为停经及不规则阴道出血、原发性闭经和盆腔包块。组织学上主要由两种细胞组成,一种为类似于无性细胞瘤及精原细胞瘤的生殖细胞,另一种为与未成熟的睾丸支持细胞或颗粒细胞相似的性索间质细胞。肿瘤细胞呈巢状分布,分为冠状结构及微滤泡结构。3例均表现为无性细胞过度生长形成无性细胞瘤伴钙化。1例检测到Y染色体。结论性母细胞瘤是一种罕见的混合性生殖-性索间质肿瘤,具有独特的病理形态特征。它属于原位生殖细胞肿瘤,具有恶性转化的潜能。

Objective To investigate the clinicopathological features, nature and biological behaviour of gonadoblastoma （GB）. Methods Three cases of GB were investigated by light microscopy and the related literature was reviewed. Results Secondary cessation of menstruation or abnormal vaginal bleeding, primary amenorrhea and abdominal tumor were the most common clinical presentations. Histologically, GB was a tumor composed of two main cell types： germ cells that were similar to those present in dysgerminoma or seminoma and sex cord derivatives resembling immature Sertoli or granulose cells. The tumor an-anged in collections of cellular nests of two different forms, the microfollicular and coronary patterns. Gonadoblastoma was overgrown by dysgerminoma in 3 cases. Calcification was seen in 3 cases. Y chromosome was detected in 1 case. Conclusions GB is a rare mixed germ cell-sex cord-stromal tumor with distinct pathological features. It is a true neoplasm in situ that has the potential of malignant transformation.