Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Copenhagen, Denmark.
Department of Pathology, Rigshospitalet, Copenhagen, Denmark.
APMIS. 2018 Aug;126(8):663-666. doi: 10.1111/apm.12859.
The aim of this study was to report incidence and patient characteristics of olfactory neuroblastoma (ONB) at a tertiary cancer institution during a 16-year period. A retrospective review was conducted on patients with ONB treated at Rigshospitalet, Copenhagen from 2000 to 2016 covering Eastern Denmark. Patient demographics, symptoms, stage, pathology-reports, treatment, and outcome were extracted from the patient records and the Danish pathology register. The tumours were graded both histologically and clinically using Hyam's and Kadish classifications, respectively. We identified a total of 14 patients (ten males, four females, median age 57 years, range 17-81 years). Four patients were in Kadish stage A, one stage B, and seven stage C. According to Hyam's classification, two tumours were grade I, nine grade II, and three grade III. All patients were treated with surgery, eight in combination with radiotherapy, where one received proton therapy, and one a combination with chemotherapy. At a median follow-up time of 58 months, the 5-year overall survival was 90% (95% CI 61; 99). ONB is a rare disease; complete radical surgery alone or combined with radiotherapy offered good oncologic control and outcome. Long-term follow-up of ONB should be mandatory.
本研究旨在报告一家三级癌症中心在 16 年期间嗅神经母细胞瘤（ONB）的发病情况和患者特征。对 2000 年至 2016 年期间在哥本哈根 Rigshospitalet 接受治疗的丹麦东部地区 ONB 患者的病历和丹麦病理登记处进行了回顾性分析。从患者记录和丹麦病理登记处提取了患者人口统计学、症状、分期、病理报告、治疗和结局等信息。肿瘤的组织学和临床分级分别采用 Hyam 分级和 Kadish 分级。共确定了 14 例患者（男性 10 例，女性 4 例，中位年龄 57 岁，范围 17-81 岁）。4 例患者处于 Kadish A 期，1 例处于 B 期，7 例处于 C 期。根据 Hyam 分级，2 例为 I 级，9 例为 II 级，3 例为 III 级。所有患者均接受手术治疗，8 例联合放疗，其中 1 例接受质子治疗，1 例联合化疗。中位随访时间为 58 个月，5 年总生存率为 90%（95%CI 61%；99%）。ONB 是一种罕见疾病；单纯完全根治性手术或联合放疗可提供良好的肿瘤控制和结局。对 ONB 应进行长期随访。
