纤维发育不良伴动脉瘤样骨囊肿患者的恶性转化

T. Troelsen, P. Jørgensen, Steen Baerentzen, T. Baad-Hansen
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摘要

38岁女性,右股骨单纯性纤维发育不良(MFD)病史,继发性转化为骨肉瘤和动脉瘤样骨囊肿。患者最初接受大范围切除和髋关节假体植入以及术后化疗。3个月后,她复发,进行了半骨盆切除术,随后进行了一系列新的化疗。治疗7年后,患者仍然存活,无转移迹象。目前尚不清楚骨肉瘤是由MFD还是动脉瘤性骨囊肿发展而来。继发性纤维发育不良转化为骨肉瘤是一种罕见的事件,特别是在没有合并mcune - albright综合征的MFD患者或未接受放射治疗的MFD患者中。动脉瘤样骨囊肿的转变是一个更罕见的事件。由于MFD的恶性转化并伴有动脉瘤性骨囊肿的罕见事件,尽管预后较差,但由于预后良好,因此报告了该病例。
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Malignant transformation in a patient with fibrous dysplasia and aneurysmal bone cyst
A 38-year old woman, with a history of monostotic fibrous dysplasia (MFD) of the right femur, developed secondary transformationto both an osteosarcoma and an aneurysmal bone cyst. The patient was initially treated with wide resection and insertion of a hipprosthesis and postoperative chemotherapy. After 3 months she had a recurrence and a hemipelvectomy was performed, followedby a new series of chemotherapy. Seven years after treatment the patient is still alive with no signs of metastasis. It is unknownwhether the osteosarcoma developed from the MFD or from the aneurysmal bone cyst. Secondary transformation of fibrous dysplasia into osteosarcoma is a rare event, especially in MFD without concomitant McCune-Albright syndrome or in patients with MFD without prior radiation therapy. Transformation of an aneurysmal bone cyst is aneven more rare event. This case is reported due to the rare event of malignant transformation of MFD with a concomitant aneurysmal bone cyst and dueto the favorable outcome, in spite of the poor prognosis associated with the condition.
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