Suh Y L, Chi J G
Department of Pathology, Seoul National University Children's Hospital, Korea.
J Korean Med Sci. 1989 Jun;4(2):97-101. doi: 10.3346/jkms.1989.4.2.97.
Telangiectatic osteosarcoma is a rare and special variant of osteogenic sarcoma with distinct radiologic, gross and microscopic features. This tumor is predominantly lytic, destructive tumor without sclerosis on roentgenogram, and is soft and cystic on gross examination. Histologically aneurysmally dilated spaces lined or traversed by stromal cells producing osteoid are noted. This report concerns a case of telangiectatic osteosarcoma occurring in a 7 years old boy. He presented with pathologic fracture of the right distal tibia, followed by a purely lytic lesion on X-ray examination. This lesion recurred five times during a span of one year. Microscopic features of the biopsy specimen was difficult to differentiate from aneurysmal bone cyst because of prominant blood-filled cyst formation. It was finally identified as osteosarcoma from the below-knee amputation specimen through the close examination for anaplastic osteoid-producing stromal cells in the septa that separate the blood cysts.
毛细血管扩张性骨肉瘤是骨肉瘤中一种罕见且特殊的变异类型,具有独特的放射学、大体和显微镜下特征。该肿瘤在X线片上主要表现为溶骨性、破坏性病变,无硬化,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基质细胞衬里或穿过的动脉瘤样扩张间隙。本报告涉及一名7岁男孩发生的毛细血管扩张性骨肉瘤病例。他因右胫骨远端病理性骨折就诊,X线检查显示为单纯溶骨性病变。该病变在一年时间内复发了5次。活检标本的显微镜下特征因显著的充满血液的囊肿形成而难以与动脉瘤样骨囊肿区分。通过对分隔血囊肿的间隔中间变的产生类骨质的基质细胞进行仔细检查,最终从膝下截肢标本中确诊为骨肉瘤。