Cuvelier A, L'Her P, Schill H, Jancovici R, Bassoulet J, Vauterin G, Allard P
Service de Pneumologie, Hôpital d'Instruction des Armées Percy, Clamart.
Rev Pneumol Clin. 1990;46(3):116-22.
The authors report the case of a 20-year old male patient presenting with a lateral thoracic tumour which at pathological examination looked like an extra-osseous Ewing's sarcoma. Strongly positive NSE immunolabeling suggested a recently described variant of this lesion: Askin's tumour. In 1979, F. B. Askin described a tumour made of round small cells, located on the side of the thorax and possibly derived from differentiated neuroectodermal tissue. Since that time, the ever wider use of electron microscopy and immunohistochemical methods has made it possible to demonstrate the existence of peripheral neuroectodermal tumours (PNETs) distinct from neuroblastomas. Askin's tumour being only, as it turned out, one of their clinical forms. Following a brief presentation of the clinical, pathological and therapeutic features of PNETs, the authors underline the great similarity between these tumours and Ewing's sarcoma. Owing to the successive discoveries of a cytogenetic abnormality common to these tumour--t(11;22)(q24;q12)--, of a similar expression of proto-oncogens and of identical neuroenzymatic characteristics, Ewing's syndrome can now be regarded as the most undifferentiated of all PNETs, probably arising from the postganglionic neuron of the parasympathetic system.
作者报告了一例20岁男性患者，其胸壁外侧出现肿瘤，病理检查显示类似骨外尤因肉瘤。NSE免疫标记强阳性提示为该病变最近描述的一种变体：阿斯基恩瘤。1979年，F. B. 阿斯基恩描述了一种由圆形小细胞构成的肿瘤，位于胸壁侧面，可能源自分化的神经外胚层组织。从那时起，电子显微镜和免疫组化方法的广泛应用使得有可能证实存在与神经母细胞瘤不同的外周神经外胚层肿瘤（PNETs）。结果表明，阿斯基恩瘤只是其临床形式之一。在简要介绍PNETs的临床、病理和治疗特征后，作者强调了这些肿瘤与尤因肉瘤之间的高度相似性。由于相继发现这些肿瘤共有的细胞遗传学异常——t(11;22)(q24;q12)——、原癌基因的相似表达以及相同的神经酶学特征，尤因综合征现在可被视为所有PNETs中分化程度最低的，可能起源于副交感神经系统的节后神经元。
