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Spontaneous Fast-growing Osteosarcoma of the Maxilla Arising in Long-standing Polyostotic Fibrous Dysplasia-A Case Report

長期罹患顱顏面多骨性纖維性發育不良導致上顎骨突然演變出生長快速的骨肉瘤形成-病例報告

摘要


一位長期罹患顱顏面多骨性纖維性發育不良的62歲女性,在這次生病之前都沒有接受過任何放射線腫瘤治療的病史,在她的左側上顎骨突然間冒出一個小腫塊因此前往醫院口腔外科求診,不幸的病理切片之後這個小腫塊快速地生長,在兩週之內這個腫瘤就佔據了病人整個口腔,導致病人無法咀嚼,吞嚥與平躺式睡覺,因此前來本院口腔外科求診。這種由顱顏面多骨性纖維性發育不良導致惡性腫瘤轉變的機會很稀少,因此我們安排病人接受顱顏面區核磁共振檢查發現病人多骨性纖維性發育不良侵犯區域很廣泛,包含了左側上顎骨,顴骨,蝶骨與翼板,眼眶,頂骨,顳骨岩狀區等都被侵犯。這個由多骨性纖維性發育不良所轉變出來的骨肉瘤侵犯了左側上顎骨和它鄰近的骨頭,然後再由左上顎骨的齒槽塉竄出口腔,最後佔據整個口腔。由於這個骨肉瘤太廣泛無法明確界定出它的範圍,因此外科切除整個骨肉瘤手術並非治療的首選。我們報告此病例是因為它是一個很特殊的病例,它在臨床上很少見,而且是由顱顏面多骨性纖維性發育不良所轉變出來的骨肉瘤。在文獻上報告此種轉變出來的骨肉瘤其侵犯力才的章,且多半是發生在30到40歲的患者,而我們的病人是62更為少見。同時本篇除了綜整一些相關文獻報導之外,也會強調我們採用部分腫瘤切除手術再加上同步性放射線治療與化學治療,讓病人的骨肉瘤能夠控制發展約一年左右,在治療期間病人日常起居生活仍能夠維持。

並列摘要


A 62-year-old female with long-standing craniofacial polyostotic fibrous dysplasia (PFD) and no history of prior radiation therapy developed a spontaneous, fast-growing osteosarcoma of left maxilla in two weeks right after she had a biopsy for a painless mass at her left maxilla. Because of this huge mass, she was not able to chew, swallow and even sleep supinely. It was rare to have spontaneous malignant transformation of PFD in the craniofacial region. Magnetic resonance imaging (MRI) suggested a huge polyostotic fibrous dysplasia occupied whole left maxilla, zygoma, sphenoid, pterygoid plate, orbital cavity, parietal bone, and petrous temporal bone. This tumor destroyed left maxilla and adjacent bones, and bulged out from the alveolar crest of left maxilla and then occupied whole oral cavity. Because this tumor was too huge to be excised, surgical intervention was not the first choice for this patient. We presented this case because it was a peculiar case in clinical practice, based on the fact of the occurrence of a malignant transformation of PFD on her old age and the aggressive tumor behavior. We also, emphasized that the combination treatments of this case had shown stable disease progress without tumor progression in one year.

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