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骨软骨瘤病或马富西综合征中的颅底软骨肉瘤——三例报告及文献复习

Chondrosarcomas of the base of the skull in Ollier's disease or Maffucci's syndrome--three case reports and review of the literature.

作者信息

Noël Georges, Feuvret Loïc, Calugaru Valentin, Hadadi Khalid, Baillet François, Mazeron Jean-Jacques, Habrand Jean-Louis

机构信息

Centre de Protonthérapie d'Orsay--Institut Curie, Orsay cedex Paris, France.

出版信息

Acta Oncol. 2004;43(8):705-10. doi: 10.1080/02841860410002770.

Abstract

Ollier's disease is a rare disease characterized by constitutional bone dysplasia with multiple enchondromas. The combination of haemangioma and chondromatoses is known as Maffucci's syndrome. Malignant degeneration of bone dysplasia into chondrosarcoma is a well-known complication, but a lesion of the base of the skull is exceptional. It is a slowly growing low-grade malignant tumour. Three new cases of chondrosarcoma of the base of the skull occurring in one patient with Marfucci's syndrome and two patients with Ollier's disease are reported and these cases are discussed in the light of the literature. A multidisciplinary approach, comprising surgery and radiotherapy, achieved good results.

摘要

奥利尔病是一种罕见疾病,其特征为伴有多发性内生软骨瘤的全身性骨发育异常。血管瘤与软骨瘤病并存被称为马富西综合征。骨发育异常恶变为软骨肉瘤是一种众所周知的并发症,但发生于颅底的病变则较为罕见。它是一种生长缓慢的低度恶性肿瘤。本文报告了1例马富西综合征患者和2例奥利尔病患者发生颅底软骨肉瘤的3例新病例,并结合文献对这些病例进行了讨论。采用包括手术和放疗在内的多学科方法取得了良好效果。

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