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[胸壁周围神经外胚层肿瘤。病例描述]

[Peripheral neuroectodermal tumors of the chest wall. Description of a case].

作者信息

Inserra A, Zangari A, Ciprandi G, Boldrini R, Boglino C

机构信息

Divisione di Chirurgia Pediatrica, Ospedale Pediatrico Bambino Gesù, Roma.

出版信息

Minerva Chir. 1998 Apr;53(4):299-303.

PMID:9701985
Abstract

The authors discuss a case of malignant small round cell tumor of the thoracopulmonary region (Askin tumor) in which the initial pathological diagnosis was "sarcoma". Only a review of the histological specimens and the clinical features and evolution revealed the correct diagnosis of "Askin tumor". A 15 year old girl with fever, dyspnoea, cough underwent thoracentesis, which revealed hemothorax. At surgery, a large mass not involving the lung, with pleural thickening, was found in the left hemithorax and excised, without rib resection. No metastasis was observed at that time. Histological findings revealed features of malignant small round cell tumors, with occasional pseudo-rosettes. Chemotherapy was performed. 16 months after clinical presentation the girl died with abdomino-thoracic metastatic disease.

摘要

作者讨论了一例胸肺区域恶性小圆细胞肿瘤(阿斯金瘤),最初的病理诊断为“肉瘤”。仅通过对组织学标本、临床特征及病情演变的回顾才得出“阿斯金瘤”的正确诊断。一名15岁发热、呼吸困难、咳嗽的女孩接受了胸腔穿刺,结果显示为血胸。手术时,在左半胸发现一个不涉及肺部、伴有胸膜增厚的大肿块并予以切除,未进行肋骨切除。当时未观察到转移情况。组织学检查发现具有恶性小圆细胞肿瘤的特征,偶见假菊形团。进行了化疗。临床表现16个月后,该女孩死于腹胸转移性疾病。

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