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先天性颅内间叶性软骨肉瘤:病例报告及小儿患者文献综述

Congenital intracranial mesenchymal chondrosarcoma: case report and review of the literature in pediatric patients.

作者信息

De Cecio Rossella, Migliaccio Ilenia, Falleti Jessica, Del Basso De Caro Marialaura, Pettinato Guido

机构信息

Dipartimento di Scienze Biomorfologiche e Funzionali, Università degli Studi di Napoli Federico II, Naples, Italy.

出版信息

Pediatr Dev Pathol. 2008 Jul-Aug;11(4):309-13. doi: 10.2350/07-05-0279.1. Epub 2007 Aug 22.

Abstract

In this paper we report the 1st case of a congenital intracranial mesenchymal chondrosarcoma in a 2-month-old infant, apparently present at birth. A magnetic resonance image showed a large left parietal solid mass, while microscopy revealed a mixture of undifferentiated small cells and mature hyaline cartilage islands, positive for vimentin, S-100, and CD99. A surgical excision was performed but the patient died after a few weeks as a result of a rapid relapse of the tumor. We also review the pediatric cases (in patients less than 20 years old) of extraskeletal (intracranial) mesenchymal chondrosarcomas of the literature, with a focus on the most recent cytogenetic and immunohistochemical studies.

摘要

在本文中,我们报告了首例2个月大婴儿先天性颅内间叶性软骨肉瘤病例,该肿瘤显然在出生时就已存在。磁共振成像显示左顶叶有一个大的实性肿块,而显微镜检查显示有未分化的小细胞和成熟透明软骨岛的混合物,波形蛋白、S-100和CD99呈阳性。进行了手术切除,但患者在几周后因肿瘤迅速复发而死亡。我们还回顾了文献中儿童(年龄小于20岁)的颅外(颅内)间叶性软骨肉瘤病例,重点关注最新的细胞遗传学和免疫组织化学研究。

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