Ow Thomas J, Hanna Ehab Y, Roberts Dianna B, Levine Nicholas B, El-Naggar Adel K, Rosenthal David I, DeMonte Franco, Kupferman Michael E
Department of Head and Neck Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas.
Head Neck. 2014 Apr;36(4):524-30. doi: 10.1002/hed.23327. Epub 2013 Jun 18.
Esthesioneuroblastoma is a rare cancer of the anterior cranial base that arises in the region of the olfactory rootlets. The purpose of this study was to review the long-term outcomes of patients diagnosed with esthesioneuroblastoma (ENB) treated at a single institution to determine factors associated with improved disease control and survival.
A retrospective review of 70 patients with ENB treated at the University of Texas MD Anderson Cancer Center between 1992 and 2007 was undertaken. Survival and recurrence was analyzed and compared using the Kaplan-Meier method and log-rank statistics.
Seventy patients were reviewed. The majority (77%) had T3 or T4 disease at presentation, 38% identified as modified Kadish stage C or D. Ninety percent of patients received surgical resection as part of their treatment, and 66% received postoperative radiation or chemoradiation. The median follow-up was 91.4 months (7.6 years). Forty-eight percent of patients developed recurrent disease and the median time to recurrence was 6.9 years. Overall and disease-specific median survival was 10.5 and 11.6 years, respectively. Patients who were treated with surgery alone had a median disease-specific survival of 87.9 months, whereas those who were treated with surgery and postoperative radiation had a median disease-specific survival of 218.5 months (p = .047).
Patients with ENB can achieve favorable long-term survival, even if disease is locally advanced. Survival is improved considerably when surgical resection is followed by postoperative radiation. However, recurrence rates and mortality remain high, and therefore long-term observation in these patients is warranted.
嗅神经母细胞瘤是一种罕见的前颅底癌症，起源于嗅丝区域。本研究的目的是回顾在单一机构接受治疗的嗅神经母细胞瘤（ENB）患者的长期预后，以确定与改善疾病控制和生存相关的因素。
对1992年至2007年间在德克萨斯大学MD安德森癌症中心接受治疗的70例ENB患者进行回顾性研究。使用Kaplan-Meier方法和对数秩统计分析并比较生存率和复发率。
共回顾了70例患者。大多数（77%）患者初诊时为T3或T4期疾病，38%为改良Kadish C期或D期。90%的患者接受了手术切除作为治疗的一部分，66%的患者接受了术后放疗或放化疗。中位随访时间为91.4个月（7.6年）。48%的患者出现复发性疾病，复发的中位时间为6.9年。总体中位生存期和疾病特异性中位生存期分别为10.5年和11.6年。单纯接受手术治疗的患者疾病特异性中位生存期为87.9个月，而接受手术和术后放疗的患者疾病特异性中位生存期为218.5个月（p = 0.047）。
ENB患者即使疾病局部进展，也能获得良好的长期生存。手术切除后进行术后放疗可显著提高生存率。然而，复发率和死亡率仍然很高，因此对这些患者进行长期观察是必要的。
