Hashimoto H, Enjoji M, Nakajima T, Kiryu H, Daimaru Y
Am J Surg Pathol. 1983 Jun;7(4):309-18. doi: 10.1097/00000478-198306000-00002.
A clinicopathologic study of 15 cases of malignant neuroepithelioma (peripheral neuroblastoma) of soft tissues is reported. The patients were chiefly young Japanese adults with a median age of 21 years. The tumors arose mainly in the soft tissues of the lower extremity (seven cases) and the trunk (four cases). Microscopically, there were sheets of closely packed, small round or oval cells, and Homer Wright-type rosettes were seen in all cases, one of which also had Flexner-type rosettes. Immunohistochemical cytoplasmic localization of neuron-specific enolase (NSE) was demonstrated in six of the eight cases, using the peroxidase-antiperoxidase (PAP) method. In no case, however, was there any staining reaction for S-100 protein. Of the 14 patients for whom follow-up information could be obtained, nine died within a period of 2 years and two were alive and well for over 5 years after the initial treatment. Differential diagnosis from other soft-tissue round-cell sarcomas, such as embryonal or alveolar rhabdomyosarcoma, extraskeletal Ewing's sarcoma, and others, are briefly discussed, on a clinicopathologic basis.
本文报告了15例软组织恶性神经上皮瘤（外周神经母细胞瘤）的临床病理研究。患者主要为日本年轻成年人，中位年龄21岁。肿瘤主要发生于下肢软组织（7例）和躯干（4例）。显微镜下可见成片紧密排列的小圆形或椭圆形细胞，所有病例均见霍纳·赖特（Homer Wright）型菊形团，其中1例还见弗莱克斯纳（Flexner）型菊形团。采用过氧化物酶-抗过氧化物酶（PAP）法，8例中有6例显示神经元特异性烯醇化酶（NSE）免疫组化胞质定位。然而，无一例S-100蛋白染色反应阳性。在可获得随访信息的14例患者中，9例在2年内死亡，2例在初始治疗后存活且状况良好超过5年。基于临床病理基础，简要讨论了与其他软组织圆形细胞肉瘤，如胚胎性或肺泡性横纹肌肉瘤、骨外尤文肉瘤等的鉴别诊断。
