Dabska M, Huvos A G
Virchows Arch A Pathol Anat Histopathol. 1983;399(1):89-104. doi: 10.1007/BF00666221.
It has been almost a quarter of a century that this rare, still poorly understood and to date insufficiently studied, cartilage neoplasm was described. Based on 19 cases in the young representing 26 percent of chondrosarcoma patients under the age of 21 years, this study found equal sex distribution with the youngest patient being a 6-year old boy. Twelve lesions occurred between the ages of 16 and 21 years. All but one of the tumors arose in the skeleton with nearly half of them involving the lower extremity. Pain was inconsistent and rare at presentation in contrast to the regularity of swelling or a painless mass. Survival analysis revealed a 46 percent 2-year and a 35 percent 5-year survival rate, whereas at 10 years only 20 percent of the patients were still alive. This study attempts to establish the likeliest evolutionary pathway of neoplastic cell differentiation and traces the origin of this tumor to a neoplastic caricature of embryonal endochondral osteogenesis.
这种罕见的软骨肿瘤被描述出来已有近四分之一个世纪了,它至今仍鲜为人知且研究不足。基于19例年轻患者的病例(占21岁以下软骨肉瘤患者的26%),本研究发现该肿瘤在性别上分布均等,最年轻的患者是一名6岁男孩。12个病灶出现在16至21岁之间。除1例肿瘤外,所有肿瘤均发生于骨骼,其中近一半累及下肢。与肿胀的规律性或无痛性肿块相比,疼痛在就诊时并不一致且少见。生存分析显示,2年生存率为46%,5年生存率为35%,而到10年时,只有20%的患者仍存活。本研究试图确定肿瘤细胞分化最可能的进化途径,并将该肿瘤的起源追溯至胚胎性软骨内成骨的肿瘤性模拟。