Olfactory Neuroblastoma: A Clinical Analysis of 13 Cases

嗅神經母細胞瘤：十三例病例臨床報告

李淞維(Sung-Wei Lee) ； 連熙隆(Shi-Long Lian) ； 蔡志仁(Chee-Yin Chai) ； 黃志仁(Chi-Jen Huang) ； 黃旼儀(Ming-Yii Huang)

《放射治療與腫瘤學》 14卷3期 (2007/09) Pp. 207-217

https://doi.org/10.6316/TRO/200714(3)207

摘要

目的：研究嗅神經母細胞瘤患者之治療結果與預後因子。
材料與方法：我們回溯分析自1994年3月到2005年10月，17位於本院診斷為嗅神經母細胞瘤的病人。藉由臨床病歷記錄、病理報告、醫學影像與放射線記錄來收集病人資料、治療方式細節與追蹤治療結果。所有病人根據Kadish，Dulguerov及6th AJCC三種方式重定疾病分期，同時也依Hyams分級方式重定腫瘤分化程度。
結果：病患包括9位男性與8位女性，年齡分布自14至74歲。依據Kadish分期，3位病患為A期，3位為B期，10位為C期。最常見的症狀為流鼻血。其中13位病患於本院接受治療，在經過40.3月的中位追蹤時間（平均48.6個月），6位病人存活且無疾病，另有4位病患存活但有腫瘤殘存。有5位病人局部週邊疾病控制失敗，遠處轉移發生在2位病患身上。整體五年存活率為52.5%，5年無疾病存活率為42%。在單變數分析中，唯有手術後腫瘤殘存程度為有意義之預後因子(p=0.021)。
結論：在本研究中，腫瘤完全摘除是最有意義的預後因子。對於即使接受廣泛切除手術仍有殘留的晚期疾病患者，放射線治療等輔助治療是必需的。手術切除合併放射線治療是一有效的治療組合，可以提高局部控制率。大部份的腫瘤在接受55-65 Gy的劑量後均有良好的反應。預防性的頸部照射並非常規必需。局部再發可以在治療結束五年後發生，因此長期的追蹤是需要的。

關鍵字

嗅神經母細胞瘤 ； 知覺神經芽細胞瘤 ； 放射線治療

並列摘要

Purpose: To analyze treatment outcome and prognostic factors of patients with olfactory neuroblastoma.
Materials and Methods: Seventeen patients with pathology-proven olfactory neuroblastoma diagnosed between March 1994 and October 2005 were analyzed retrospectively. Clinical charts, pathology reports, radiology images and radiotherapy records were reviewed. Patients' characteristics, details of treatment modalities and clinical outcome were collected. Sixteen patients were re-staged with three staging system including: Kadish, Dulguerov and the 6th AJCC staging system for nasal cavity and paranasal sinuses. Histo-pathological specimens of 17 patients were reviewed to define the tumor grading using Hyams' four-tier system.
Results: There were 9 male and 8 female, age ranged from 14 to 74 years old. According to Kadish's classification, there were 3 stage A, 3 stage B and 10 stage C diseases. The most common symptom was epistaxis. Thirteen patients received treatment at our institute. A median follow-up time of 40.3 months (mean: 48.6 months), 6 patients were alive without disease and 4 were alive with disease. Locoregional failure occurred in 5 patients and distant metastases were noted in 2 patients. Five-year overall survival rate was 52.5% and 5-year disease free survival rate was 42%. Degree of surgery was the only significant prognostic factor in univariate analysis (p=0.021).
Conclusion: In this study, surgical intervention to total removal of the tumor was the most significant prognostic factor. Adjuvant treatment such as radiotherapy was needed for advanced diseases, in which residual tumor was notified even after radical surgery. Radiotherapy combined with surgical resection was an effective modality for treatment of olfactory neuroblastoma; this combination could provide excellent local control. Most tumors were response to the initial radiotherapy at the range of 55 to 65 Gy. Elective neck irradiation was not needed routinely. Local recurrence might happen even 5 years later, and long-term follow-up should be mandated.

並列關鍵字

Olfactory Neuroblastoma ； Esthesioneuroblastoma ； Radiotherapy