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鼻及鼻竇區之少年型沙樣狀骨化纖維瘤

Juvenile Psammomatoid Ossifying Fibroma of Sinonasal Region

摘要


少年型沙樣狀骨化纖維瘤(juvenile psammomatoid ossifying fibroma, JPOF)為一頭頸部罕見之良性纖維骨性腫瘤,通常發生於兒童和年輕人,無性別上之差異,好發於鼻及鼻竇區。有其特殊的組織型態及臨床特徵,雖生長緩慢但常造成外觀上的變形並有易復發的傾向,治療一般是以外科切除為主。本院於2005年10月經歷一名19歲男性患者,主訴左側鼻塞及凸眼已有多年,經理學檢查及電腦斷層檢查,發現左側鼻腔鼻竇中,有一鈣化性腫塊。病人接受鼻竇內視鏡手術,病理組織切片證實為少年型沙樣狀骨化纖維瘤。但腫瘤於2007年1月復發,並且較前次手術前更廣泛;症狀仍以左側鼻塞及凸眼表現。病患於本院接受側鼻切開術(lateral rhinotomy),病理組織切片同上次手術結果。術後鼻塞及凸眼症狀大幅改善,病患於門診追蹤4個月無復發情形,因此病例極為罕見,故提出報告說明之。

並列摘要


Juvenile psammomatoid ossifying fibroma (JPOF) is a rare benign fibro-osseous tumor of the head and neck. It occurs predominantly in children and young adults without significant sexual predilection. It typically involves the sinonasal region, and has distinctive histomophological and clinical features. Although it is a slowly progressing lesion, it causes cosmetic disfigurement and has a tendency to recur. The main treatment is surgical excision. We report on a 19-year-old male bothered by left nasal obstruction and proptosis for many years. On first admission in October 2005, a calcified lesion was found within the left nasal cavity and paranasal sinus, based on the findings of a physical examination and computed tomography (CT) scan. The tumor was resected by endoscopic surgery. The pathology report identified juvenile psammomatoid ossifying fibroma. In January 2007, the tumor recurred and was larger. It still presented as left nasal obstruction and proptosis. A lateral rhinotomy was performed. The pathology report corresponded to the last report. Postoperatively, the symptoms of proptosis and nasal obstruction were improved. The patient has been followed up for 4 months without any evidence of recurrence.

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