多發齒源性角化囊腫－病例報告

Multiple Odontogenic Keratocysts-Case Report

黃威雄(Wei-Hsiung Huang) ； 沈宗憲(Tzung-Shiahn Sheen) ； 饒宇東(Yu-Tung Yao)

《中華民國耳鼻喉科醫學會雜誌》 33卷4期 (1998/08) Pp. 384-389

https://doi.org/10.6286/1998.33.4.384

摘要

關鍵字:多發齒源性角化囊腫;痣樣基底細胞癌症候群;哥林氏症候群
多發齒源性角化囊腫（multiple odontogenic keratocysts）相當少見，臨床上它常以某症候群的諸多病變之一而表現，這些症候群包括：痣樣基底細胞癌症候群（nevoid basal-cell carcinoma syndrome）、Noonan氏症候群、Hunter氏症候群及黏多糖貯積症（muco-polysaccharidosis）。當然它也可以是獨立的病變。在此提出1例14歲女性病人，有多發齒源性角化囊腫、多處分歧肋骨（bifid ribs）、大腦鐮鈣化（calcification of falx cerebri）、蝶鞍橋（bridging of sella turcica）、脊柱前曲側彎（kyphoscoliosis）、雞胸（pigeon chest）、腦性麻痺、智能不足及眼距過大（hypertelorism）等等問題，但皮膚並無痣樣的病灶出現。此例疑似痣樣基底細胞癌症候群，可能皮膚病變尚未出現。

關鍵字

多發齒源性角化囊腫 ； 痣樣基底細胞癌症候群 ； 哥林氏症候群

並列摘要

The disease of multiple odontogenic keratocysts is rare. It often presents as one manifestation in some syndromes such as nevoid basal cell carcinoma syndrome, Noonan syndrome, Hunter syndrome and mucopolysaccharidosis. However, sometimes, it is not associated with any syndrome and exists independently. This 14 year-old girl presented with multiple odontogenic deratocysts, bifid ribs, calcification of falx cerebri, bridging of sella turcica, kyphoscoliosis, pigeon chest, cerebral palsy, mental retardation and hypertelorism. No skin lesions such as basal cell carcinoma were found on presentation. She might be a victim of nevoid basal cell carcinoma syndrome.

並列關鍵字

multiple odontogenic keratocysts ； nevoid basal-cell carcinoma syndrome ； Gorlin syndrome