摘要
目的报道1例起源于臀部的骨骼外尤文肉瘤/原始神经外胚层瘤(ES/PNET)。结合文献资料,复习其临床、病理特征、免疫表型、细胞基因、鉴别诊断、治疗及预后等。方法采用经常规制片、HE染色,又辅以免疫组化染色。结果肿瘤由幼稚深染的小圆形细胞组成,免疫组化可有神经源性表达;病理诊断(左臀部)骨外ES/PNET。结论ES/PNET诊断标准除原始小细胞外,常向神经分化,其免疫表型表达CD99及两项以上的神经标志物,遗传学上有染色体t(11;22)(q24;q12)的易位。
Objective To report a case of extra - skeletal Ewing＇ s sarcoma/primitive neuroectodermal tumor （KS/ PNET） origined from hip and discuss their clinical manifestations, pathological features, immuno - phenotypes, genetics, differential diagnosis, treatments and prognosis with review of the literatur..Methods The case was conventional HE and adjuvant immunohistochemical staining. Results This was composed of small round cell.A neurogenic immunophenotype was identified. A diagnosis of primary ES/PNET of hip was confirmed. Conclusion Its microscopic diagnostic criteria should include the indentification of neural differentiation except for uniform small round cells. Positive expression for more than 2 neual endocrine markers and CD99 must be present . Cytogenetic finding is t （11 ;22） （q24; q12）.
出处
《黑龙江医学》
2009年第9期690-691,712,共3页
Heilongjiang Medical Journal
关键词
尤文肉瘤
神经外胚叶肿瘤
原始
外周
Ewing＇ s sarcoma
Primitive nerves
Neuroectodennal tumor
Pefipheal
