骨与软组织原始神经外胚层瘤的病理诊断与临床治疗

Primitive neuroectodermal tumor of bone and soft tissue

目的探讨原始神经外胚层瘤（ＰＮＥＴ）的病理学特点及其临床意义。方法报告本科１９８９年４月～１９９６年８月收治５例ＰＮＥＴ患者的经验。肿瘤发生部位为髂骨２例、腋窝软组织１例、股骨髁１例、颅骨１例。Ｘ线片显示骨组织呈溶骨性破坏，无骨膜反应及钙化征象。５例患者病理标本除常规石蜡切片和ＨＥ染色，均作了ＡＢＣ法免疫组化，其中２例还作了透射电镜观察。以光镜下具有Ｈｏｍｅｒ－Ｗｒｉｇｈｔ（Ｈ－Ｗ）菊形团的排列；免疫组化检查Ｏ１３阳性并至少含２种以上不同神经标记的表达，如ＮＳＥ、ＳＹ３８、ＣｇＡ等，或经电镜检查细胞内含有神经内分泌颗粒为ＰＮＥＴ诊断标准。结果５例均行术后化疗，３例同时行放疗。３例因肺转移分别于发病后２０、１３、５个月死亡；２例带瘤生存。结论（１）Ｏ１３阳性，光镜下Ｈ－Ｗ菊形团，免疫组化检查至少含２种以上不同神经标记的表达，如ＮＳＥ、ＳＹ３８、ＣｇＡ等，对于与其它骨和软组织的小圆细胞类肿瘤的鉴别具有一定意义；（２）临床上应注意ＰＮＥＴ与Ｅｗｉｎｇ瘤的区分和鉴别；（３）肿瘤远处转移是导致ＰＮＥＴ治疗失败的主要原因，更有效的治疗方法仍有待于探讨。

Objective To study the pathological characteristics of primitive neuroectodermal tumor(PNET) of bone and soft tissue. Method Five patients with bone and soft tissue of PNET (2 men and 3 women) were studied by light microscope and immunohistochemistry.The age of the patients was 18,18,43,14,32 years and the primary sites of involvement were the ilium( n =2),axillary fossa( n =1),condyle of femur( n =1),and diploe( n =1).X ray films showed bone osteolytic destruction,and no periosteal reaction or calcification. Surgery was performed in the patients. Result All patients received combined chemotherapy and 3 received radiotherapy. Three patients died of metastatic tumor of lung 20,13,and 5 months after invasion.1 patient metastatic tumor of lung 20 months after invasion, and 1 patient bone extensive metastasis 1 5 months after surgery. Conclusion The treatment of PNET of bone and soft tissued awaits further study.