嗅神经母细胞瘤的预后因素和治疗结果分析

目的 探讨嗅神经母细胞瘤（ENB）的临床特点、预后因素及适宜治疗方案。方法 回顾性分析49例经病理确诊的FNB患者,改良Kadish分期为A期3例,B期15例,C期22例,D期9例。单纯手术治疗4例,单纯放射治疗11例．手术＋放射治疗19例,放射治疗＋化疗8例,手术＋放射治疗＋化疗7例。应用SPSS10．0软件进行统计分析,Kaplan-Meier法计算生存率（OS）和无病生存率（DFS）,Logrank检验生俘曲线之间的差异。结果 49例患者的5年OS为60．7％,5年DFS为41．9％。A、B期和C、D期患者的5年OS分别为78．4％和49．7％（X^2=2．10,P=0．15）,5年DFS分别为47．1％和38．4％（X^2=0．08,P=0．78）。有颈部淋巴结转移和无颈部淋巴结转移的5年OS分别为17．8％和70．8％（X^2=2．32,P=0．13）,5年DFS分别为O和53．4％（X^2=11．67,P〈0．01）。KPS≥80和KPS〈80患者的5年OS分别为69．0％和30．1％（X^2=7．01,P〈0．01）,5年DFS分别为46．7％和24．9％（X^2=6．37,P=0．01）。综合治疗组及单纯放疗和（或）单纯手术组的5年OS分别为69．7％和46．3％（X^2=3．49,P=0．06）,5年DF、S分别为52．2％和21．8％（X^2=7．03,P〈0．01）。无颈部淋巴结转移患者,接受手术治疗苦和未接受手术治疗者的5年OS分别为71．1％和44．6％（X^2=7．99,P〈0．01）,5年DFS分别为54．0％和24．1％（X^2=6．41,P=0．01）。11例单纯放疗患者5年OS为47．7％,5年DFS为30．7％。结论 FNB患者发病年龄较早,男性较多,就诊时多已是局部晚期,一般状况评分及颈部淋巴结转移是预后的影响因素,治疗以包括手术在内的综合治疗疗效较好,放射治疗在ENB的治疗中占有重要地位。

Prognostic factors and outcome of esthesioneuroblastoma

OBJECTIVE: To investigate the clinical feature, prognostic factors and the appropriate treatment modality of esthesioneuroblastoma (ENB). METHODS: The data of 49 patients with ENB treated from Dec. 1978 to Dec. 2001 were retrospectively reviewed and analyzed. In this series, 3 patients had modified Kadish stage A disease, 15 stage B, 22 stage C, and 9 stage D lesion. The treatment modalities included surgery alone in 4 patients, and radiotherapy alone in 11, surgery plus radiotherapy in 19, radiotherapy plus chemotherapy in 8, surgery plus radiotherapy plus chemotherapy in 7. Statistic analysis was performed using software SPSS 10.0. Overall survival (OS) and disease free survival (DFS) were calculated using Kaplan-Meier method. Differences between survival curves were tested by Log rank method. RESULTS: The 5-year OS and DFS of the whole group was 60.5% and 41.9%, respectively. The 5-year OS of patients with modified Kadish stage A or B disease and those with stage C or D was 78.4% and 49.7% (chi(2) = 2.10, P = 0.15), and the 5-year DFS was 47.1% and 38.4% (chi(2) = 0.08, P = 0.78), respectively. The 5-year OS of patients with or without neck lymph nodal metastasis was 17.8% and 70.8% (chi(2) = 2.32, P = 0.13), and the 5-year DFS was 0 and 53.4% (chi(2) = 11.67, P < 0.01), respectively. For patients with kfs > or = 80 and those with kfs < 80, the 5-year OS was 69.0% and 30.1% (chi(2) = 7.01, P < 0.01), and 5-year DFS was 46.7% and 24.9% (chi(2) = 6.37, P = 0.01), respectively. As regard to the treatment modality, The 5-year OS was 69.7% for the patients treated with combined modalities and 46.3% for those with surgery alone or radiotherapy alone (chi(2) = 3.49, P = 0.06), and the 5-year DFS were 52.2% and 21.8% (chi(2) = 7.03, P < 0.01), respectively. The 5-year OS was 71.1% for patients who received surgical treatment and 44.6% for those without it (chi(2) = 7.99, P < 0.01), and 5-year DFS was 54.0% and 24.1% (chi(2) = 6.41, P = 0.01), respectively. The 5-year OS and DFS of 11 patients who received radiotherapy alone were 47.7% and 30.7%, respectively. For 19 patients treated by radiotherapy with radical purpose (including patients who received combined modality with radiotherapy plus chemotherapy), the 5-year OS were 33.9% for < 70 Gy patients and 48.0% for > or = 70 Gy (chi(2) = 0.89, P = 0.35), and the 5-year DFS was 13.3% and 33.3% (chi (2) = 4.48, P = 0.03), respectively. For those who received chemotherapy or not, the 5-year OS was 50.0% and 64.9% (chi(2) = 0.91, P = 0.34), and the 5-year DFS was 38.9% and 43.1% (chi(2) = 0.01, P = 0.91), respectively. CONCLUSION: Esthesioneuroblastoma is more prevalent in the young male adults than female, usually with locally advanced stage lesion when first diagnosed. Performance status and neck lymph node metastasis are significantly correlated with the prognosis. Combined treatment modality consisting of surgery may help to gain more favorable result. Radiotherapy plays an important role in the management of the disease.