目的：鼻咽部的惡性腫瘤診斷上大多是非鱗狀上皮細胞癌或未分化癌，其他病理型態極為少見。我們探討本院 25 年期間接受治療的罕見鼻咽部惡性腫瘤病人的臨床表現與治療結果。材料與方法：從 1983 年 1 月開始至 2008 年 12 月為止，總共有 2113 個鼻咽部惡性腫瘤的病人登錄在本院的放射治療資料庫，僅有 7 位病人符合本篇文章的研究目的。我們回顧性的檢視這幾位病人的病歷、放射治療紀錄與影像檢查。我們分析了這些病人的臨床病程與治療結果。我們比較了這群病人的存活與另一群傳統鼻咽癌（期別 II-IVb）接受同步化學放射治療的病人的存活。結果：本院罕見鼻咽部惡性腫瘤的發生率只有 0.33% 。病理診斷共有 3 位上皮性惡性腫瘤（腺癌、黏膜性上皮癌、小細胞癌），3 位淋巴癌，1 位橫紋肌惡性肉瘤。這些病人的初期臨床表現與傳統鼻咽癌相似。有兩位病人一開始被診斷為傳統鼻咽癌（未分化癌），直至發生遠端轉移後，診斷更正為淋巴癌與橫紋肌惡性肉瘤。研究組病人的整體存活率與疾病無惡化存活率比傳統鼻咽癌差，具有統計顯著差異（分別為 42.9% vs. 74.8% ，P = 0.027; 42.9% vs. 71.7% ，P = 0.016）。
結論：罕見的鼻咽部惡性腫瘤包括多種不同的病理型態。因此要正確診斷這些病人必須仰賴病理科醫師的高度懷疑。這類病人的治療成果低於傳統鼻咽癌的病人。

Purpose : Nasopharyngeal malignancies, other than squamous cell carcinoma and undifferentiated carcinoma, are rare. We investigated the clinical manifestations and treatment outcomes of patients with rare nasopharyngeal malignancies over a 25-year period.Materials and Methods : From January 1983 to December 2008, a total of 2113 patients with nasopharyngeal malignancies were registered in our radiotherapy database. Only seven eligible patients with rare malignancies of the nasopharynx were found. We retrospectively reviewed hospital charts, radiotherapy records, and imaging studies. The clinical courses and final outcomes were analyzed. Survival was compared with another group of patients with stage II – IVb conventional nasopharyngeal carcinoma (NPC), treated by concomitant chemoradiotherapy. Results : The incidence of rare nasopharyngeal malignancies was only 0.33%. The pathologic diagnoses were three rare carcinomas (adenocarcinoma, mucoepidermoid carcinoma, and small cell carcinoma), three lymphomas and one rhabdomyosarcoma.
The initial manifestations were similar to conventional NPC. Two patients were misdiagnosed with conventional NPC (undifferentiated carcinoma), but this was revised to rhabdomyosarcoma and malignant lymphoma when distant sites relapsed. The overall survival and progression-free survival of the study group were significantly worse than conventional NPC (42.9% vs. 74.8%, P = 0.027; 42.9% vs. 71.7%, P = 0.016, respectively). Conclusion : Rare malignancies arising from the nasopharynx consist of several different pathologies. The accurate diagnosis of rare malignancies of the nasopharynx depends on high index of suspicious of pathologist. The treatment outcome is worse than conventional NPC.