Eich Hans Theodor, Müller Rolf-Peter, Micke Oliver, Kocher Martin, Berthold Frank, Hero Barbara
Department of Radiation Oncology, University of Cologne, Cologne, Germany.
Strahlenther Onkol. 2005 Jun;181(6):378-84. doi: 10.1007/s00066-005-1362-2.
Only 3% of all malignant intranasal tumors are esthesioneuroblastomas (ENB) and only 20% of these rare neuroectodermal tumors are diagnosed up to 20 years of age. Radiotherapy and surgery are established treatment modalities for these patients, but the role of chemotherapy, especially in a multimodal approach, is not well defined. To investigate the influence of radio- and chemotherapy, the treatment and course of the disease in children and adolescents with ENB were analyzed retrospectively.
19 unselected patients (nine male and ten female) diagnosed with ENB < or = 20 years of age were included in this analysis. Median age at diagnosis was 14.0 years (range, 5-20 years). The tumors were Kadish stage B in 4/19 patients and stage C in 15/19 patients. 17 patients underwent surgery, either without further therapy (n = 4), followed by radiotherapy (n = 1) or as part of multimodal regimens (n = 12). Two patients received radio- and chemotherapy without surgery. Complete resection (R0) was achieved in 15 out of 17 patients with surgery including all five patients with preoperative chemotherapy due to unresectable primary at diagnosis.
The 5-year overall survival (OS) for the whole group was 73% +/- 12% and the 5-year event-free survival (EFS) 55% +/- 13%. None of the four patients with stage B experienced tumor progression so far, whereas seven out of 15 patients with stage C did (5-year EFS 47% +/- 14%; not significant). Patients with Kadish stage C and multimodal treatment strategies combing surgery, chemo- and radiotherapy had a significantly better outcome than patients with stage C and less than three treatment modalities (65% +/- 17% vs. 20% +/- 18%; p = 0.02).
These data indicate a benefit of multimodal treatment regimens combining surgery, chemo- and radiotherapy for pediatric patients with ENB Kadish stage C. Chemotherapy appears to improve resectability, EFS, and OS. Radiotherapy is an integral part in the management of children and young adolescents with ENB in Kadish stage B and C.
在所有鼻腔恶性肿瘤中，嗅神经母细胞瘤（ENB）仅占3%，且在这些罕见的神经外胚层肿瘤中，仅有20%在20岁之前被诊断出来。放疗和手术是这些患者既定的治疗方式，但化疗的作用，尤其是在多模式治疗方法中的作用，尚未明确界定。为了研究放疗和化疗的影响，我们对儿童和青少年ENB患者的疾病治疗及病程进行了回顾性分析。
本分析纳入了19例年龄小于或等于20岁、未经筛选诊断为ENB的患者（9例男性，10例女性）。诊断时的中位年龄为14.0岁（范围5 - 20岁）。19例患者中，4例为卡迪什B期肿瘤，15例为C期肿瘤。17例患者接受了手术，其中4例未接受进一步治疗，1例术后接受放疗，12例作为多模式治疗方案的一部分。2例患者未手术，接受了放疗和化疗。17例接受手术的患者中，15例实现了完全切除（R0），其中包括因诊断时原发性肿瘤无法切除而术前接受化疗的所有5例患者。
全组患者的5年总生存率（OS）为73%±12%，5年无事件生存率（EFS）为55%±13%。4例B期患者目前均未出现肿瘤进展，而15例C期患者中有7例出现肿瘤进展（5年EFS为47%±14%；无显著性差异）。采用手术、化疗和放疗联合的多模式治疗策略的卡迪什C期患者，其预后明显优于采用少于三种治疗模式的C期患者（65%±17%对20%±18%；p = 0.02）。
这些数据表明，对于卡迪什C期的儿童ENB患者，手术、化疗和放疗联合的多模式治疗方案有益。化疗似乎能提高可切除性、EFS和OS。放疗是卡迪什B期和C期儿童及青少年ENB治疗中不可或缺的一部分。
