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内生软骨瘤、内生软骨瘤病及继发软骨肉瘤风险的相关见解。文献复习,重点关注临床行为、放射学、恶变及随访。

Insights into Enchondroma, Enchondromatosis and the risk of secondary Chondrosarcoma. Review of the literature with an emphasis on the clinical behaviour, radiology, malignant transformation and the follow up.

出版信息

Neoplasma. 2014;61(4):365-78. doi: 10.4149/neo_2014_046.

DOI:10.4149/neo_2014_046
PMID:24645839
Abstract

The Enchondroma is a common, benign, cartilage forming tumour. They usually occur as a single, asymptomatic lesion. Occasionally patients present with multiple enchondromas which is generally defined as enchondromatosis. This entity encompasses several different subtypes including Ollier disease and Maffucci syndrome (enchondromatosis associated with soft tissue haemangiomas) as the most commons. Some of them have a complicated clinical course when malignant transformation occurs. This malignant progression is a well known fact especially in enchondromatosis, but up to now there is still a lack of recommendations concerning the follow up. The aim of this article is to review the clinical and imaging features of patients with solitary enchondroma and enchondromatosis focusing on the development of secondary chondrosarcoma and the follow up.

摘要

内生软骨瘤是一种常见的良性软骨组织肿瘤。它们通常表现为单个无症状病变。偶尔,患者会出现多发性内生软骨瘤,通常被定义为内生软骨瘤病。该病症包括几种不同的亚型,最常见的是 Ollier 病和 Maffucci 综合征(内生软骨瘤病伴软组织血管瘤)。当发生恶性转化时,其中一些会出现复杂的临床病程。这种恶性进展是一个众所周知的事实,尤其是在内生软骨瘤病中,但迄今为止,关于随访仍缺乏建议。本文的目的是回顾单发内生软骨瘤和内生软骨瘤病患者的临床和影像学特征,重点关注继发软骨肉瘤的发生和随访。

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