鞍区恶性混合性生殖细胞肿瘤临床病理分析

Clinicopathological features of malignant mixed germ cell tumors in sellar region

目的探讨鞍区恶性混合性生殖细胞肿瘤临床病理特征、诊断及鉴别诊断,并分析其生物学行为。方法回顾性分析1例原发于鞍区及鞍上恶性混合性生殖细胞肿瘤临床病理学资料,并复习相关文献。结果患者女,12岁,因'多饮、多尿3月余'入院。血清学检查β-HCG升高。镜检:肿瘤由两种成分组成,绒毛膜癌与生殖细胞瘤混杂分布,瘤细胞异型性明显,核分裂象可见。免疫组化:瘤细胞PLAP弥漫(+),绒毛膜癌区β-HCG、HPL(+),生殖细胞瘤区CD117、D2-40(+),Ki-67约70%。术后症状明显改善,β-HCG降至正常,已行术后放疗,后期将继续跟进随访。结论鞍区恶性混合性生殖细胞肿瘤非常少见,组织学伴有绒毛膜癌成分的恶性混合性生殖细胞肿瘤更为罕见,送检标本应广泛取材,避免误诊、漏诊。

Objective To investigate the clinicopathological features,diagnosis and differential diagnosis of malignant mixed germ cell tumors in sellar region,and to analyze its biological behaviors.Methods Clinicopathological data of one case of malignant mixed germ cell tumor in sellar region was analyzed,and the related literatures were reviewed.Results A 12-year-old girl was admitted to the hospital because of polydipsia and polyuria for more than 3 months.The level ofβ-HCG was increased.The tumor consisted of two components:choriocarcinoma and germinoma;the neoplastic cells were markedly atypical,and mitotic figures were visible.Tumor cells were diffusely positive for PLAP.β-HCG and HPL were positive in choriocarcinoma,and CD117 and D2-40 were positive in germ cell tumor area.Postoperative symptoms were significantly improved,β-HCG was reduced to normal,postoperative radiotherapy was performed,and follow-up will be continued later.Conclusions Malignant mixed germ cell tumors in sellar region are very rare,and that histologically accompanied by choriocarcinoma is even rarer.The specimens must be widely sampled to avoid misdiagnosis.