单细胞纤维发育不良术后恶性转化1例

Cesar D. Dimayuga
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引用次数: 0

摘要

纤维结构不良(FD)是一种良性病变,其特征是正常骨被异常结缔组织取代。它以单骨或多骨形式发生,具有罕见但已证实的恶性转化潜力。急性疼痛、迅速肿胀或肿块增大的症状应增加对肉瘤改变的怀疑。完全手术切除是治疗的主要方法,建议化疗以提高生存率。病例报告:一名52岁男性,因单侧FD继发病理性骨折接受股骨远端钢板固定2年后,右侧大腿近端出现疼痛、肿大的肿块。诊断影像显示复发征象,核心穿刺活检显示提示恶性转化的侵袭性特征。手术切除和化疗后,4年无复发或转移迹象。结论:单纯性FD的恶性转化是罕见的。症状恶化应增加对肉瘤改变的怀疑,并提示诊断影像学和组织学证实的需要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Malignant Transformation of Monostotic Fibrous Dysplasia after Surgery: A Case Report
Introduction: Fibrous dysplasia (FD) is a benign lesion characterized by replacement of normal bone with abnormal connective tissue. It occurs in monostotic or polyostotic forms, with a rare but proven potential for malignant transformation. Symptoms of acute pain, rapid swelling, or an enlarging mass should increase suspicion for possible sarcomatous change. Complete surgical resection is the mainstay of treatment, and chemotherapy is recommended to improve survival. Case Report: A 52-year-old male presented with a painful, enlarging mass on the right proximal thigh 2 years after undergoing plate fixation of the distal femur for a pathologic fracture secondary to monostotic FD. Diagnostic imaging revealed signs of recurrence, and core needle biopsy revealed aggressive features suggestive of malign ant transformation. Following surgical resection and chemotherapy, the outcome has been uneventful without evidence of recurrence or metastasis at 4-year post-operation. Conclusion: Malignant transformation in monostotic FD is rare. Symptom exacerbation should increase the suspicion for sarcomatous change and prompt the need for diagnostic imaging as well as histologic confirmation.
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