外周原始神经外胚层肿瘤形态学免疫表型及临床预后研究

目的：探讨外周原始神经外胚层肿瘤(PNET)的临床表现，病理及免疫组织化学特点及预后。方法：对15例外周原始神经外胚层肿瘤进行临床资料及预后，病理组织形态学和超微结构特点及免疫组化表型研究。结果：患者以男性为主，男女之比约为6．5：1，年龄6～35岁，年龄中位数为16，发生部位较多，可发生在直肠，腹膜后，腹股沟，淋巴结，胸壁，睾丸，鼻腔及骨组织等；免疫组化：肿瘤均弥漫表达CD99，并不同程度地表达NSE，SYN，CgA等，但不表达CK，Desmin，LCA等，其中1例表达组织化学染色PAS，随访最长时间为24月。结论：PNET是一种发生在年轻男性，进展迅速，预后非常差的恶性小圆细胞肿瘤，认识其临床病理特点及免疫组化表型对于该恶性肿瘤的诊断及临床治疗意义重大。

A Morphological, Clinical Prognostic and Immunohistochemical Study in Primitive Neuroectodermal Tumor

Objective:To investigate the clinical,histomorphological and immunohistochemi-cal features in primitive neuroectodermal tumor (PNET).Methods:Fifteen cases of small round cell tumors are studied by histopathological,ultrastructural and immunohistochemical methods and clinical data.Results:There is prodominat male bias with male:female ratio of6.57:1,ages are ranged from6years to32years and median age is16years.The location of tumors are rectum,retroperitoneum,inguina,lymph node,chest wall,testis,nasal cavity and bone.Almost all tumor cells are strong and diffused staining for CD99,but weak or strong positive for NSE,Syn and CgA.All the tumor cells are negative for CK,Desmin and LCA.Only one case is positive for PAS histochemically.The follow-up period is about 4to24months.Conclusion:PNET is a kind of malignant small round cell tumors with rapid aggressive clinical course.The clinical and histopathological features are special and help for the diagnosis and treatment of PNET.