CLEAR CELL CHONDROSARCOMA OF BONE - OBSERVATIONS IN 47 CASES

CLEAR CELL CHONDROSARCOMA OF BONE - OBSERVATIONS IN 47 CASES
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DOI:
10.1097/00000478-198403000-00009
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发表时间:
1984-01-01
影响因子:
5.6
通讯作者:
SIM, FH
SIM, FH
中科院分区:
医学1区
文献类型:
--
作者:
BJORNSSON, J;UNNI, KK;SIM, FH

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骨透明细胞软骨肉瘤是一种低度恶性肿瘤,诊断上常有困难。它的临床、X线和病理特征与传统的软骨肉瘤不同,更重要的是,它与几个经常被混淆的良性骨肿瘤不同。这份报告分析了在[美国明尼苏达州罗切斯特市]梅奥诊所就诊的47例病例。男性多于女性(2.6:1),好发于长骨末端,尤其是股骨近端。年龄范围很广;大多数患者都在3岁和4岁。X线片上,病变通常是单纯的松解性和轻微的膨胀性,在肿瘤和邻近的正常骨之间有一个锐利的边缘。病理上,透明细胞排列成模糊的小叶图案是肿瘤的特征。通常,存在与其他原发骨肿瘤相似的区域,无论是良性的还是恶性的,往往以至于掩盖了该过程的真实身份。本组病例的总死亡率为15%。整块切除,包括边缘的正常骨和软组织,似乎是首选的治疗方法。
Clear cell chondrosarcoma of bone, a low-grade malignant tumor, frequently presents diagnostic difficulties. Its clinical, roentgenographic and pathologic chracteristics separate it from conventional chondrosarcoma and, more importantly, from several benign bone tumors with which it is often confused. This report analyzes 47 cases seen at the Mayo Clinic [Rochester, Minnesota, USA] and in consultation. The lesion is more common in males than females (2.6:1) and has a predilection for the end of long bones, particularly the proximal femur. The age range is wide; most patients are in the 3rd and 4th decades of life. Roentgenographically, the lesion most often is purely lytic and slightly expansile, with a sharp margin between the tumor and the adjacent normal bone. Pathologically, clear cells arranged in an indistinct lobular pattern characterize the tumor. Frequently, areas mimicking other primary bone tumors, benign and malignant, are present, often to such an extent as to obscure the true identity of the process. The overall mortality in the present series was 15%. En bloc resection, including a margin of normal bone and soft tissue, appears to be the treatment of choice.