Witkin G B, Guilford W B, Siegal G P
Clin Orthop Relat Res. 1986 Mar(204):245-52.
A 41-year-old man with recognized polyostotic fibrous dysplasia since late childhood developed fibroblastic osteogenic sarcoma in the left tibia. Four months after the initial diagnosis, an intramuscular myxoma was discovered in the left thigh. Twenty years previously he had been found to be heterozygous for hemoglobins JBaltimore and S. Malignant transformation in fibrous dysplasia is unusual and may be associated in some individuals with prior irradiation. Soft tissue myxomas associated with fibrous dysplasia are even rarer. To the best of the authors' knowledge the occurrence of both of these lesions in a patient with fibrous dysplasia has been reported only once before. Patients with both fibrous dysplasia and myxomas may be at greater risk for malignant transformation than are individuals with only one of these lesions. There is no well-recognized association between hemoglobinopathies and either fibrous dysplasia or bone tumors. It is therefore probable that the rare constellation of findings is in this patient a stochastic event.
一名自童年后期就被确诊为多骨型纤维发育不良的41岁男性,左胫骨发生了成纤维细胞性骨肉瘤。初次诊断四个月后,在左大腿发现了一个肌内黏液瘤。20年前,他被发现血红蛋白JBaltimore和S呈杂合状态。纤维发育不良的恶性转化并不常见,在一些个体中可能与既往放疗有关。与纤维发育不良相关的软组织黏液瘤更为罕见。据作者所知,纤维发育不良患者同时出现这两种病变此前仅报道过一次。患有纤维发育不良和黏液瘤的患者可能比仅患有其中一种病变的个体发生恶性转化的风险更高。血红蛋白病与纤维发育不良或骨肿瘤之间没有公认的关联。因此,该患者出现这种罕见的症状组合很可能是一个随机事件。