Dias Fernando L, Sa Geraldo M, Lima Roberto A, Kligerman Jacob, Leoncio Marlos P, Freitas Emilson Q, Soares Jose Roberto N, Arcuri Roberto Alfonso
Department of Head and Neck Surgery and Surgical Pathology, Hospital do Cancer I-Instituto Nacional de Cancer, Rio de Janeiro, Brazil.
Arch Otolaryngol Head Neck Surg. 2003 Nov;129(11):1186-92. doi: 10.1001/archotol.129.11.1186.
To evaluate the results of standardized treatment of esthesioneuroblastoma (ENB) during a 17-year period and to identify pertinent factors for clinical outcome.
Review of clinical and radiographic data and retrospectively staging ENB according to 3 staging systems: Kadish, Biller, and Dulguerov and Calcaterra.
Hospital do Cancer I-Instituto Nacional de Cancer, Rio de Janeiro, Brazil.
Thirty-six patients with histologically confirmed ENB treated between January 1, 1983, and December 31, 2000; 35 fulfilled study inclusion criteria.
Treatment included gross tumor resection through a transfacial approach with postoperative radiotherapy (RT) in 11 patients, craniofacial resection (CFR) and postoperative RT in 7, exclusive RT in 14, CFR alone in 1, and a combination of chemotherapy and RT in 2. Histopathological slides were reviewed and graded using the Hyams staging system. Analysis of prognostic factors was performed.
Evaluation of survival rates using the Kaplan-Meier method. Analysis of prognostic factors carried out with the Fisher exact test and the log-rank test.
Analysis of survival showed that the Kadish classification best predicted disease-free survival (P =.046). The presence of regional and distant metastases adversely affected prognosis (P<.001 and P =.01, respectively). Craniofacial resection plus postoperative RT provided a better 5-year disease-free survival rate (86%) compared with the other therapeutic options used (P =.05). The 5-year disease-specific survival rate was 64% and 43% for the low- and high-grade tumors, respectively (P =.20). Disease-free survival for this cohort of 35 patient was 46% and 24% at 5 and 10 years, respectively. Overall survival was 55% and 46% at 5 and 10 years of follow-up, respectively.
The development of cervical nodal metastases and distant metastases had a significant adverse impact on prognosis. The value of the Kadish staging system was confirmed in our study, significantly correlating with prognosis. Tumor grade according to the Hyams staging system also seems to be an important factor in determining prognosis for tumor recurrence and survival. Aggressive multimodality therapeutic strategies, particularly CFR and adjuvant RT, yielded the best treatment outcome.
评估17年间嗅神经母细胞瘤（ENB）标准化治疗的结果，并确定影响临床结局的相关因素。
回顾临床和影像学数据，并根据3种分期系统对ENB进行回顾性分期：卡迪什（Kadish）分期、比勒（Biller）分期以及杜尔盖罗夫和卡尔卡特拉（Dulguerov and Calcaterra）分期。
巴西里约热内卢国立癌症研究所第一癌症医院。
1983年1月1日至2000年12月31日期间36例经组织学确诊的ENB患者；35例符合研究纳入标准。
治疗包括11例经经面部入路行大体肿瘤切除术并术后放疗（RT）、7例行颅面切除术（CFR）并术后放疗、14例单纯放疗、1例单纯CFR以及2例化疗联合放疗。使用海姆斯（Hyams）分期系统对组织病理学切片进行复查和分级。进行预后因素分析。
采用Kaplan-Meier法评估生存率。采用Fisher精确检验和对数秩检验进行预后因素分析。
生存分析显示，卡迪什分类法最能预测无病生存期（P = 0.046）。区域和远处转移的存在对预后有不利影响（分别为P<0.001和P = 0.01）。与其他治疗方案相比，颅面切除术加术后放疗的5年无病生存率更高（86%）（P = 0.05）。低级别和高级别肿瘤的5年疾病特异性生存率分别为64%和43%（P = 0.20）。这35例患者的5年和10年无病生存率分别为46%和24%。随访5年和10年时的总生存率分别为55%和46%。
颈部淋巴结转移和远处转移的发生对预后有显著不利影响。本研究证实了卡迪什分期系统的价值，其与预后显著相关。根据海姆斯分期系统确定的肿瘤分级似乎也是决定肿瘤复发和生存预后的一个重要因素。积极的多模式治疗策略，尤其是CFR和辅助放疗，产生了最佳治疗效果。
