第二型神經纖維瘤病併鼻部神經纖維瘤

Neurofibromatosis Type 2 Associated with Nasal Neurofibroma

賴正軒(Cheng-Hsuan Lai) ； 賴仁淙(Jen-Tsung Lai)

《台灣耳鼻喉頭頸外科雜誌》 40卷2期 (2005/04) Pp. 70-73

https://doi.org/10.6286/2005.40.2.70

摘要

第二型神經纖維瘤病爲顯性染患體遺傳之疾病，在第22對染患體上缺乏抑制腫瘤生長的基因，特色爲雙側聽神經瘤合併其他顱內腫瘤或脊髓神經的侵犯，又稱爲中樞型神經纖維瘤病。而鼻部神經纖維瘤相當罕見，多由三叉神經或自主神經長出來，大部份病例與von Recklinghausen氏病（第一型神經纖維瘤病）有關。本科於長期追蹤1名20歲的第二型神經纖維瘤病患者時，患者抱怨他的鼻子最近幾個月來有越來越寬的樣子，檢查發現在右側上鼻道有一紅色腫塊，內視鏡切片檢查確定爲神經纖維瘤，爲罕見第二型神經纖維瘤病併鼻部神經纖維瘤病例。由於目前並沒有任何因爲鼻部神經纖維瘤造成的不適，所以患者拒絕手術治療，目前就比照他雙側聽神經瘤觀察等待的方式，持續於門診追蹤。

關鍵字

鼻部神經纖維瘤 ； 第二型神經纖維瘤病 ； 神經鞘瘤

並列摘要

Neurofibromatosis Type 2 (NF2) is an autosomal dominant disorder, characteristic as bilateral acoustic neuroma with or without other brain or spinal lesion, also called central type neurofibromatosis. Nasal neurofibroma is a very rare disease, most cases are derived from the trigeminal nerve or the autonomic nerve and combined with von Recklinghausen's disease (neurofibromatosis type 1). We have follow up a 20 years-old man, who is a victim of neurofibromatosis type 2, complained of that his nose had been getting wider over the past few months. A mass was found in the right nasal superior meatus and ths diagnosis of neurofibroma was established by endoscopic biopsy. Due to the fact that theie was no obvious discomfort caused by the nasal neuinfibroma at present, the patient refused surgical treatment and was placed on OPD follow up under a ”watchful waiting” policy as the treatment of his bilateral acoustic neuroma.

並列關鍵字

nasal neurofibroma ； neurofibromatosis type 2 ； schwannoma