OUTCOME AND PROGNOSTIC FACTORS IN OLFACTORY NEUROBLASTOMA: A RARE CANCER NETWORK STUDY
OUTCOME AND PROGNOSTIC FACTORS IN OLFACTORY NEUROBLASTOMA: A RARE CANCER NETWORK STUDY
复制标题
DOI：
10.1016/j.ijrobp.2009.09.019
复制
发表时间：
2010-11-15
影响因子：
7
通讯作者：
Zouhair, Abderrahim
中科院分区：
文献类型：
--
作者：
Ozsahin, Mahmut;Gruber, Guenther;Zouhair, Abderrahim
目的：评估嗅神经母细胞瘤 (ONB) 患者的预后。方法和材料：纳入 1971 年至 2004 年间接受非转移性 ONB 治疗的 77 名患者。根据Kadish分型，A期11例，B期29例，C期37例。T分期包括T1期9例，T2期26例，T3期16例，T4a期15例，T4b期11例。 68 例患者出现 N0 (88%) 疾病。 结果：大多数患者 (n = 56, 73%) 受益于手术 (S)，44 例患者可以完全切除（R0 32 例，R1 13 例，R2 11 例）。除 5 名患者外，所有患者均从放疗中受益，21 名患者（27%）接受了化疗。中位随访期为 72 个月（范围 6-315）。 5年总生存率（OS）、无病生存率（DES）、局部控制率和局部控制率分别为64%、57%、62%和70%。在单变量分析中，有利因素是 Kadish A 或 B 病、T1 T3 肿瘤、无淋巴结受累、根治性手术、R0/R1 切除以及 RT 剂量 54 Gy 或更高。多变量分析显示，预测结果的最佳独立因素是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手术切除随后至少54 Gy术后放疗的ONB患者具有最佳结果。对于这种局部失败仍然是一个问题的罕见疾病，应前瞻性地研究包括联合化疗和/或更高剂量放疗在内的新策略。 (C) 2010 爱思唯尔公司。
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 (DES), 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. (C) 2010 Elsevier Inc.