Mantsopoulos Konstantinos, Koch Michael, Iro Heinrich, Constantinidis Jannis
Department of Otorhinolaryngology, Head and Neck Surgery, University of Erlangen-Nürnberg, 91054 Erlangen, Germany.
1st Department of Otolaryngology, Head & Neck Surgery, Aristotle University of Thessaloniki, 54636 Thessaloniki, Greece.
J Clin Med. 2022 Apr 20;11(9):2288. doi: 10.3390/jcm11092288.
The aim of this study was to investigate the long-term oncologic outcome and review the state of the art in the management of olfactory neuroblastomas.
The records of all patients treated for olfactory neuroblastomas in two academic departments between 1975 and 2012 were evaluated retrospectively. Data on epidemiological parameters were collected (age, gender), along with staging (Kadish, Morita), histologic grading (Hyams), time and form of treatment, locoregional control, and disease-specific and overall survival. Patients with other malignant diseases, distant metastases of olfactory neuroblastomas at the time of initial diagnosis, a follow-up time of less than 5 years, or insufficient clinical-pathological data were excluded from further analysis.
In total, 53 cases made up our final study sample (26 men, 27 women; male-female ratio 0.96:1). Their mean age was 48.6 years (range: 10-84 years). The mean follow-up time was 137.5 months (4-336 months, SD: 85.0). A total of 5 out of 53 study cases (9.4%) showed metastatic involvement of the neck at the time of initial presentation. Local recurrence was detected in 8/53 (15.1%) and regional recurrence in 7/53 of our study cases (13.2%). Three patients (42.8%) from the group of cases with surgery as the sole form of management (7/53, 13.2%) died due to the disease. The cumulative disease-specific survival and overall survivalfor the whole group of patients were 88.6% and 63.6%, respectively. The cumulative disease-specific survival stratified by Kadish A/B vs. Kadish C/D as well as Hyams I/II vs. Hyams III/IV showed superior results for limited tumors, albeit without significance, and low-grade tumors (highly significant difference).
Craniofacial or sometimes solely endoscopically controlled resection can warrant resection of the olfactory neuroblastoma with wide margins. However, locoregional failures and distant metastases can occur after a long period of time. The non-negligible incidence of regional recurrences, partly in unusual localizations, leads us to consider the need to identify the "recurrence-friendly" cases and to perform individualized elective irradiation of the neck in cases with high-risk features.
本研究旨在调查嗅神经母细胞瘤的长期肿瘤学结局，并回顾其治疗的最新进展。
回顾性评估1975年至2012年期间两个学术科室治疗的所有嗅神经母细胞瘤患者的记录。收集流行病学参数（年龄、性别）数据，以及分期（卡迪什分期、森田分期）、组织学分级（海姆斯分级）、治疗时间和方式、局部区域控制情况以及疾病特异性生存率和总生存率。排除患有其他恶性疾病、初诊时存在嗅神经母细胞瘤远处转移、随访时间少于5年或临床病理数据不充分的患者，进行进一步分析。
最终研究样本共53例（男性26例，女性27例；男女比例为0.96:1）。他们的平均年龄为48.6岁（范围：10 - 84岁）。平均随访时间为137.5个月（4 - 336个月，标准差：85.0）。53例研究病例中有5例（9.4%）在初次就诊时出现颈部转移。在我们的研究病例中，8/53（15.1%）检测到局部复发，7/53（13.2%）检测到区域复发。在仅接受手术治疗的病例组（7/53，13.2%）中，有3例患者（42.8%）死于该疾病。整个患者组的累积疾病特异性生存率和总生存率分别为88.6%和63.6%。按卡迪什A/B期与卡迪什C/D期以及海姆斯I/II级与海姆斯III/IV级分层的累积疾病特异性生存率显示，局限性肿瘤和低级别肿瘤的结果较好，尽管无统计学意义，但低级别肿瘤有高度显著差异。
颅面手术或有时仅通过内镜控制的切除术可保证广泛切除嗅神经母细胞瘤。然而，长时间后可能会发生局部区域复发和远处转移。区域复发的发生率不可忽视，部分发生在不寻常的部位，这使我们考虑需要识别“易于复发”的病例，并对具有高危特征的病例进行个体化的选择性颈部放疗。
