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毛细血管扩张性骨肉瘤:124例患者的临床病理研究

Telangiectatic osteogenic sarcoma: a clinicopathologic study of 124 patients.

作者信息

Huvos A G, Rosen G, Bretsky S S, Butler A

出版信息

Cancer. 1982 Apr 15;49(8):1679-89. doi: 10.1002/1097-0142(19820415)49:8<1679::aid-cncr2820490824>3.0.co;2-2.

Abstract

One hundred-twenty-four patients with this rare and special variant of osteogenic sarcoma were treated at Memorial Sloan-Kettering Cancer Center from 1921 through 1979, representing 11% of all of osteogenic sarcomas. The lesions were predominantly lytic, destructive tumors with only minimal sclerosis on roentgenograms and soft as well as cystic on gross examination. Histologically, aneurysmally dilated spaces lined or traversed by sarcoma cells producing osteoid were noted. The differential diagnosis both radiographically and histologically included several benign lesions like aneurysmal bone cyst and giant cell tumor, among many others. It was found that telangiectatic osteogenic sarcoma is relatively frequent in the femoral diaphysis and in the distal end of the femur. Twenty-nine percent of the patients present with pathologic fracture, or this develops later. Age and sex distribution, or clinical signs or symptoms were those of ordinary osteogenic sarcomas. No differences in survival rates were found in lesions that were purely lytic or those with minimal sclerosis. Similarly, no differences in survival were noted when comparing patients with telangiectatic or ordinary osteogenic sarcoma. As a matter of fact, definite increase in survival was found in patients treated since 1975 with preoperative multidrug chemotherapy employing high-dose methotrexate. Adriamycin, and the combination of bleomycin, cyclophosphamide, and dactinomycin.

摘要

1921年至1979年期间,纪念斯隆凯特琳癌症中心共治疗了124例患有这种罕见特殊类型骨肉瘤的患者,占所有骨肉瘤患者的11%。这些病变主要为溶骨性、破坏性肿瘤,X线片上仅有极少的硬化表现,大体检查时质地柔软且呈囊性。组织学上,可见由产生类骨质的肉瘤细胞衬里或穿行的动脉瘤样扩张间隙。影像学和组织学上的鉴别诊断包括多种良性病变,如动脉瘤样骨囊肿和巨细胞瘤等。发现毛细血管扩张型骨肉瘤在股骨干和股骨远端相对常见。29%的患者出现病理性骨折,或在之后发生。年龄和性别分布、临床体征或症状与普通骨肉瘤相同。在纯溶骨性病变或硬化极少的病变中,未发现生存率有差异。同样,比较毛细血管扩张型骨肉瘤患者和普通骨肉瘤患者时,也未发现生存率有差异。事实上,1975年以来接受术前多药化疗(采用大剂量甲氨蝶呤、阿霉素以及博来霉素、环磷酰胺和放线菌素D联合化疗)的患者生存率有明显提高。

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