上頜之基質增生型釉質母細胞瘤－病例報告

Desmoplastic Ameloblastoma in the Maxilla - Case Report

林長希(Chang-His Lin) ； 林堂烈(Tarng-Lieh Lin) ； 陳正文(Jeng-Weng Chen) ； 林書馨(Hshu-Hshing Lin) ； 婁培人(Pei-Jen Lou)

《中華民國耳鼻喉科醫學雜誌》 34卷5期 (1999/09) Pp. 413-417

https://doi.org/10.6286/1999.34.5.413

摘要

基質增生型釉質母細胞瘤（desmoplastic ameloblastoma）是最近才發表之極為罕見的腫瘤，文獻上僅有48例病例報告。它多發生在東方男性身上，年齡層分布廣（18-68歲）。與一般釉質母細胞瘤不同的是好發於前上頜骨，且X光片上為透光與不透光混和的泡沫狀（soap-bubble like）腫瘤。病理切片可見釉質母細胞瘤在一片緻密的結締組織內。本病例為66歲男性病人，主訴右臉頰部腫痛有2年之久。核磁共振影像顯示右上頜竇前側有一骨蝕性腫瘤，切片報告為鱗狀齒源性腫瘤（squamous odontogenic tumor）。於是病患接受右側部分上頜骨切除術（partial maxillectomy），初步病理報告為齒源性纖維瘤（odontogenic fibroma），但經最後證實為基質增生型釉質母細胞瘤。術後恢復情況良好，追蹤無腫瘤復發的現象。由於此腫瘤病理切片容易與其他低惡性度的腫瘤混淆，尤其是手術方式差異頗大。因此必須獲得足夠的組織切片，並配合臨床及影像的特性，才能有正確的病理診斷。

關鍵字

基質增生型釉質母細胞瘤 ； 上頜 ； 齒源性纖維瘤

並列摘要

Desmoplastic ameloblastoma is a vaiant of ameloblastoma that was described in detail only recently. The incidence rate is low and only 48 cases have ever been reported, with a greater frequency among Asian males in a wide age range (from 18 to 68 years old). Most related tumors occur in the anterior maxilla. Radiologically, desmoplastic ameloblastoma has a mixed radiolucent and radiopaque (“soap-bubble”) appearance. Histo-logically, the tumor is characterized by islands of odontogenic epithelium in abundant collagenous stroma. In this article, we report a case of desmoplas-tic ameloblastoma in the maxilla. The patient was a 66 years old man with the chief complaint of right cheek swelling for 2 years prior to admission to our hospital. Magnetic resonance imaging revealed a right maxillary tumor with bony destruction. The patient received partial maxillectomy under the biopsied diagnosis of squamous odontogenic tumor. Pathology examination of the resected specimen led to an initial diagnosis of odontogenic fibroma and finally diagnosed to desmoplastic ameloblastoma owing to its character-istic histologic and radiological features. His postoperative course was smooth and follow-up examinations revealed no recurrence. As the histo-logic appearance of this tumor is easily confused with other low-grade odotogenic tumors, of which the operative procedures may differ from each other, we suggest that obtaining an adequate biopsy specimen and establishing a close correlation with clinical and radiological findings are essential for a correct pathological diagnosis.

並列關鍵字

desmoplastic ameloblastoma ； maxilla ； odontogenic fibroma