MALIGNANT NEUROEPITHELIOMA (PERIPHERAL NEURO-BLASTOMA) - A CLINICOPATHOLOGIC STUDY OF 15 CASES

MALIGNANT NEUROEPITHELIOMA (PERIPHERAL NEURO-BLASTOMA) - A CLINICOPATHOLOGIC STUDY OF 15 CASES
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DOI:
10.1097/00000478-198306000-00002
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发表时间:
1983-01-01
影响因子:
5.6
通讯作者:
DAIMARU, Y
DAIMARU, Y
中科院分区:
医学1区
文献类型:
--
作者:
HASHIMOTO, H;ENJOJI, M;DAIMARU, Y

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本文报告15例软组织恶性神经上皮瘤(周围神经母细胞瘤)的临床病理研究。患者主要是年轻的日本成年人,中位年龄为21岁。肿瘤主要发生在小腿软组织(7例)和躯干软组织(4例)。显微镜下可见紧密排列的小圆形或椭圆形细胞片,所有病例均可见荷马-赖特型花环,其中一例也有Flexner-型花环。8例中有6例用过氧化物酶-抗过氧化物酶免疫组织化学方法显示神经元特异性烯醇化酶胞浆定位。未见S-100蛋白染色反应。在可获得随访信息的14名患者中,9名患者在最初治疗后2年内死亡,2名患者存活并存活5年以上。本文结合临床病理,简要讨论了与其他软组织圆细胞肉瘤的鉴别诊断,如胚胎性或泡状横纹肌肉瘤、骨外尤文S肉瘤等。
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 yr. The tumors arose mainly in the soft tissues of the lower extremity (7 cases) and the trunk (4 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 was demonstrated in 6 of the 8 cases, using the peroxidase-antiperoxidase method. In no case was there any staining reaction for S-100 protein. Of the 14 patients for whom follow-up information could be obtained, 9 died within a period of 2 yr and 2 were alive and well for over 5 yr 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 clinicopathological basis.