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섬유이형성증/맥쿤-알브라이트 증후군이 있는 환자의 하악에서 발생한 이차성 골육종의 증례보고 및 문헌고찰 KCI 등재
Secondary Osteosarcoma of the Mandible Arising in a Patient with Fibrous Dysplasia/Mccune-Albright Syndrome (FD/MAS): A Case Report and Review of the Literature
pp.201-209
Sarcomatous transformation of fibrous dysplasia (FD) is rare and can occur in patients with McCune-Albright syndrome (MAS). To date, there have been several cases of malignant transformation of FD in the craniofacial area of patients with MAS. Here, we report an additional case of secondary osteosarcoma arising from FD in the mandible of a 41-year-old woman with MAS. The patient complained of rapid swelling in the right facial area, which was initially misdiagnosed as soft tissue sarcoma at another hospital. After neoadjuvant concurrent chemoradiotherapy resulting in poor response, the lesion was surgically resected in our hospital, and the final diagnosis of secondary osteosarcoma was rendered. Currently, post-operative adjuvant chemotherapy is in progress. As a result of our review of 17 reported cases showing malignant transformation in FD/MAS, the M/F ratio was 1:1.1, and the median age at onset of malignancy was 28.6 years. The most commonly affected site was the craniofacial bones (n=13; 76%), and the most common histopathologic type of malignancy was osteosarcoma (n=14; 82%). More than half of the patients (8/15; 53.3%) died within 1 year, mainly due to lung metastasis (6/8; 75%). Taken together, since MAS patients with malignant transformation of FD have a relatively poor prognosis, accurate diagnosis based on histopathologic findings as well as clinical and radiographic information is important to select optimal treatment.
Ⅰ. INTRODUCTION
Ⅱ. Case report
Ⅲ. Discussion
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