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毛细血管扩张性骨肉瘤

Telangiectatic osteogenic sarcoma.

作者信息

Matsuno T, Unni K K, McLeod R A, Dahlin D C

出版信息

Cancer. 1976 Dec;38(6):2538-47. doi: 10.1002/1097-0142(197612)38:6<2538::aid-cncr2820380643>3.0.co;2-1.

Abstract

Of approximately 1,999 cases of osteogenic sarcomas at the Mayo Clinic, 25 were diagnosed as telangiectatic osteogenic sarcomas. Of the 25 patients involved, 16 were males and 9 were females, and their ages ranged from 6 to 49 years. Six patients had had pathologic fracture. The lesions were typically located centrally and usually in the distal femur or proximal humerus and roentgenographically were large and purely lytic with destruction of cortex. Grossly, the lesions were cystic and contained clotted blood. Histologically, cystic spaces that contained blood were lined with anaplastic spindle cells and benign giant cells; sometimes, there were so few malignant cells that diagnosis was difficult. Usually, fine, lacelike osteoid was present. Of the 25 patients, 23 have died of metastatic disease, and another has developed pulmonary metastasis 11 months after amputation. Only one patient has survived for more than five years; however, he has developed pneumothorax. Data from this series suggest that the outlook in telangiectatic osteogenic sarcoma is more bleak than in conventional osteosarcoma.

摘要

在梅奥诊所大约1999例骨肉瘤病例中,有25例被诊断为毛细血管扩张型骨肉瘤。在这25例患者中,男性16例,女性9例,年龄在6岁至49岁之间。6例患者发生了病理性骨折。病变通常位于中央,多位于股骨远端或肱骨近端,X线表现为较大的纯溶骨性病变,伴有皮质破坏。大体上,病变呈囊性,内含凝血块。组织学上,含血的囊腔衬以间变的梭形细胞和良性巨细胞;有时,恶性细胞极少,诊断困难。通常可见纤细的花边样骨样组织。25例患者中,23例死于转移性疾病,另1例在截肢后11个月发生了肺转移。只有1例患者存活超过5年;然而,他发生了气胸。该系列的数据表明,毛细血管扩张型骨肉瘤的预后比传统骨肉瘤更差。

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