Department of Radiation Oncology, Centre Hospitalier Universitaire Vaudois and University of Lausanne, Lausanne, Switzerland.
Int J Radiat Oncol Biol Phys. 2010 Nov 15;78(4):992-7. doi: 10.1016/j.ijrobp.2009.09.019. Epub 2010 Mar 16.
PURPOSE: To assess the outcome in patients with olfactory neuroblastoma (ONB). METHODS AND MATERIALS: Seventy-seven patients treated for nonmetastatic ONB between 1971 and 2004 were included. According to Kadish classification, there were 11 patients with Stage A, 29 with Stage B, and 37 with Stage C. T-classification included 9 patients with T1, 26 with T2, 16 with T3, 15 with T4a, and 11 with T4b tumors. Sixty-eight patients presented with N0 (88%) disease. RESULTS: Most of the patients (n = 56, 73%) benefited from surgery (S), and total excision was possible in 44 patients (R0 in 32, R1 in 13, R2 in 11). All but five patients benefited from RT, and chemotherapy was given in 21 (27%). Median follow-up period was 72 months (range, 6-315). The 5-year overall survival (OS), disease-free survival (DFS), locoregional control, and local control were 64%, 57%, 62%, and 70%, respectively. In univariate analyses, favorable factors were Kadish A or B disease, T1-T3 tumors, no nodal involvement, curative surgery, R0/R1 resection, and RT-dose 54 Gy or higher. Multivariate analysis revealed that the best independent factors predicting the outcome were T1-T3, N0, R0/R1 resection, and total RT dose (54 Gy or higher). CONCLUSION: In this multicenter retrospective study, patients with ONB treated with R0 or R1 surgical resection followed by at least 54-Gy postoperative RT had the best outcome. Novel strategies including concomitant chemotherapy and/or higher dose RT should be prospectively investigated in this rare disease for which local failure remains a problem.
目的:评估嗅神经母细胞瘤(ONB)患者的预后。
方法和材料:纳入了 1971 年至 2004 年间治疗的 77 例非转移性 ONB 患者。根据 Kadish 分期,11 例为 A 期,29 例为 B 期,37 例为 C 期。T 分期包括 9 例 T1 期、26 例 T2 期、16 例 T3 期、15 例 T4a 期和 11 例 T4b 期肿瘤。68 例患者表现为 N0(88%)疾病。
结果:大多数患者(n=56,73%)受益于手术(S),44 例患者可完全切除(R0 切除 32 例,R1 切除 13 例,R2 切除 11 例)。除 5 例患者外,所有患者均受益于放疗,21 例患者接受化疗(27%)。中位随访时间为 72 个月(范围为 6-315)。5 年总生存率(OS)、无病生存率(DFS)、局部区域控制率和局部控制率分别为 64%、57%、62%和 70%。单因素分析表明,有利因素为 Kadish A 或 B 期疾病、T1-T3 肿瘤、无淋巴结受累、根治性手术、R0/R1 切除和放疗剂量 54 Gy 或更高。多因素分析显示,预测预后的最佳独立因素是 T1-T3、N0、R0/R1 切除和总放疗剂量(54 Gy 或更高)。
结论:在这项多中心回顾性研究中,接受 R0 或 R1 手术切除后接受至少 54 Gy 术后放疗的 ONB 患者预后最佳。对于局部复发仍然是一个问题的这种罕见疾病,应前瞻性研究包括同时化疗和/或更高剂量放疗的新策略。
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