König Marton, Osnes Terje, Jebsen Peter, Evensen Jan Folkvard, Meling Torstein R
Department of Neurosurgery, Oslo University Hospital-Rikshospitalet, PB4950 Nydalen, 0424, Oslo, Norway.
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway.
Neurosurg Rev. 2018 Jan;41(1):323-331. doi: 10.1007/s10143-017-0859-3. Epub 2017 May 25.
Olfactory neuroblastoma (ONB) is a potentially curable disease, despite being an aggressive malignancy with a poor natural history. Our goal was to evaluate management outcomes for patients with ONB treated at our institution. Our prospective database for brain tumors and the pathology registry of head and neck cancers at Oslo University Hospital were searched to identify all patients treated for ONB between 1998 and 2016. Variables extracted from these databases, supplemented by retrospective chart reviews, underwent thorough analysis. All cases were formally re-examined by a dedicated head and neck pathologist. Twenty patients were identified. Follow-up was 100%. Mean follow-up was 81.5 months for the entire cohort and 120.3 months for patients with no evidence of disease. Fourteen patients underwent treatment of choice including craniofacial resection (CFR) with or without radiotherapy (XRT). Six patients could only receive less extensive treatment; three patients underwent lateral rhinotomy (LR) with or without XRT after being deemed medically unsuitable for CFR, while another three patients received only supportive, non-surgical treatment (due to positive lymph node status in two and to extensive tumor size in one case). Overall and disease-specific survival rates were 100% after 10 years of follow-up when negative surgical margins were achieved by CFR. Positive margins were associated with poorer outcome with no patients surviving longer than 44 months. Long-term survival was also achieved in two cases among patients not eligible for CFR: one case after radical LR and one case after radio-chemotherapy. Advanced disease at presentation (tumor size ≥40 mm, Kadish grades C and D, or TNM IVa and IVb) and positive surgical margins were correlated to significantly dismal survival. Our study suggests that CFR with or without adjuvant XRT is safe and leads to excellent long-time overall and disease-specific survival. Negative surgical margins, tumor size <40 mm, Kadish stage A/B, and TNM stages I-III are independent prognostic predictors of outcome.
嗅神经母细胞瘤（ONB）尽管是一种侵袭性恶性肿瘤且自然病程不佳，但却是一种有潜在治愈可能的疾病。我们的目标是评估在我们机构接受治疗的ONB患者的治疗效果。我们检索了奥斯陆大学医院的脑肿瘤前瞻性数据库以及头颈癌病理登记处，以确定1998年至2016年间所有接受ONB治疗的患者。从这些数据库中提取的变量，辅以回顾性病历审查，进行了全面分析。所有病例均由一位专门的头颈病理学家进行正式复查。共识别出20例患者。随访率为100%。整个队列的平均随访时间为81.5个月，无疾病证据的患者平均随访时间为120.3个月。14例患者接受了包括有或无放疗（XRT）的颅面切除术（CFR）在内的首选治疗。6例患者只能接受范围较小的治疗；3例患者在被认为因医学原因不适合CFR后接受了有或无XRT的外侧鼻切开术（LR），而另外3例患者仅接受了支持性非手术治疗（2例为淋巴结阳性，1例为肿瘤体积过大）。当通过CFR实现手术切缘阴性时，10年随访后的总生存率和疾病特异性生存率均为100%。切缘阳性与较差的预后相关，没有患者存活超过44个月。在不符合CFR条件的患者中，也有2例实现了长期生存：1例在根治性LR后，1例在放化疗后。就诊时的晚期疾病（肿瘤大小≥40mm、卡迪什分级C和D或TNM IVa和IVb）以及手术切缘阳性与显著较差的生存率相关。我们的研究表明，有或无辅助XRT的CFR是安全的，并能带来出色的长期总生存率和疾病特异性生存率。手术切缘阴性、肿瘤大小<40mm、卡迪什分期A/B以及TNM分期I-III是预后的独立预测指标。
