阴道尤文肉瘤/原始神经外胚层瘤临床病理观察

Ewing's sarcoma/primitive neuroectodermal tumor of vagina:a clinicopathological study

目的报道1例起源于阴道的骨骼外尤文肉瘤/原始神经外胚层瘤(ES/PNET),结合文献资料,复习其临床、病理、超微结构、免疫表型、细胞基因、鉴别诊断、治疗及预后等。方法该病例选自天津市病理会诊中心2001-06—2005-06间的1998例疑难病理标本,经常规制片,HE染色,又辅以免疫组化染色。结果阴道肿物呈带蒂息肉,由幼稚核深染小圆细胞组成,免疫组化呈神经及肌源性双向免疫表型,病理诊断阴道原发性ES/PNET。结论ES/PNET诊断标准除原始小细胞外常向神经分化,必须具备免疫组化CD99及神经内分泌标记2项以上阳性,细胞遗传学分析有染色体易位t(11;22)(q24;q12)。

Objective To report a case of extra-skeletal Ewing＇s sarcoma /primitive neuroeetodermal tumor （ES/PNET） origined from vagina and to discuss their clinical manifestations, pathological features, uhrastructures, immuno-phenotypes, genetics, differential diagnosis, treatments and prognosis with review of the literature. Methods In 1998 cases of difficult consultation titan Tianjin Pathological Consultation Center from June 2001 to June 2005, only 1 case was confirmed as extraskeletal ES/PNET of vagina by using conventional HE and adjuvant pediculate polypoid tumor, composed of small round cells with hyperchromatic nuclei. A neurogenic and myogenic diphasic immunophenotype was identified. A diagnosis of primary ES/PNET of vagina was confirmed. Conclusion This case belongs to the third report in China and the sixth in the English literature. Its microscopic diagnostic criteria include the identification of neural differentiation except for uniform small round cells. Positive expression for more than 2 neural endocrine markers and CD99 must be present. Cytogenetic finding is t （11;22）（q24;q12）.