Askin F B, Rosai J, Sibley R K, Dehner L P, McAlister W H
Cancer. 1979 Jun;43(6):2438-51. doi: 10.1002/1097-0142(197906)43:6<2438::aid-cncr2820430640>3.0.co;2-9.
This report describes a unique clinicopathologic entity characterized as a malignant small cell tumor of the thoracopulmonary region in 20 children and adolescents (average age 14.5 years). There was a female predilection (75%) for this tumor which appeared to originate in the soft tissues of the chest wall or the peripheral lung. The neoplasm tended to recur locally and did not seem to disseminate as widely as some of the other small cell tumors of childhood (rhabdomyosarcoma, Ewing's sarcoma, neuroblastoma and malignant lymphoma). However, the median survival was only 8 months. Electron microscopy of 3 cases suggested a neuroepithelial derivation, but, at the present, the histogeneis remains a subject for further investigation.
本报告描述了一种独特的临床病理实体，其特征为20例儿童和青少年（平均年龄14.5岁）胸肺区域的恶性小细胞肿瘤。该肿瘤女性多见（75%），似乎起源于胸壁软组织或肺外周。肿瘤倾向于局部复发，且不像儿童期的一些其他小细胞肿瘤（横纹肌肉瘤、尤因肉瘤、神经母细胞瘤和恶性淋巴瘤）那样广泛播散。然而，中位生存期仅8个月。3例的电子显微镜检查提示神经上皮起源，但目前组织发生仍有待进一步研究。
