Department of Otolaryngology, Head and Neck Surgery, University Medical Center Groningen, University of Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands.
Eur Arch Otorhinolaryngol. 2013 Sep;270(9):2565-8. doi: 10.1007/s00405-013-2554-5. Epub 2013 Jun 6.
Sinonasal carcinoma with neuroendocrine differentiation (SCND) is a rare group of tumors known for their aggressive behavior and poor response to treatment. The data in the literature are sparse and cover a wide range of therapeutic approaches over a protracted timeline. Therefore, it is important that institutions report on their experience with these rare neoplasms. Clinical data, such as age at diagnosis, gender, tumor subtype and stage, treatment intention and modality, recurrence, salvage treatment, and survival of patients with a SCND, diagnosed at our department between 1980 and 2010, were retrospectively analyzed. Fifteen patients were available for analysis; eight with sinonasal undifferentiated carcinoma (SNUC), five with sinonasal neuroendocrine carcinoma (SNEC), and two with small cell neuroendocrine carcinoma (SmCC). The median age at the time of diagnosis was 68 years (range 28-87). Treatment consisted of surgery (2), radiotherapy (4), a combination of these modalities (6) and palliation (3). The estimated 5-year overall survival was 60 % for SNEC, 44 % for SNUC and 0 % for SmCC. According to our institutional experience an aggressive multi-modality approach incorporating (neoadjuvant) chemoradiotherapy, radical surgery and elective treatment of the neck is the best treatment strategy for SCND. The high propensity for distant metastasis and poor prognosis of SmCC warrants consideration of the impact of treatment on the remaining quality of life in these patients.
具有神经内分泌分化的鼻腔鼻窦癌（SCND）是一组罕见的肿瘤，以其侵袭性行为和对治疗的反应差而闻名。文献中的数据稀少，涵盖了广泛的治疗方法和漫长的时间线。因此，机构报告这些罕见肿瘤的经验非常重要。回顾性分析了 1980 年至 2010 年间在我科诊断为 SCND 的患者的临床数据，如诊断时的年龄、性别、肿瘤亚型和分期、治疗意图和方式、复发、挽救治疗和生存情况。共有 15 名患者可供分析；8 名患有未分化型鼻窦癌（SNUC），5 名患有鼻窦神经内分泌癌（SNEC），2 名患有小细胞神经内分泌癌（SmCC）。诊断时的中位年龄为 68 岁（范围 28-87）。治疗包括手术（2 例）、放疗（4 例）、这些方法的联合治疗（6 例）和姑息治疗（3 例）。SNEC 的 5 年总生存率估计为 60%，SNUC 为 44%，SmCC 为 0%。根据我们机构的经验，包括（新辅助）放化疗、根治性手术和选择性颈部治疗在内的积极多模态方法是治疗 SCND 的最佳策略。SmCC 具有远处转移的高倾向和预后不良，需要考虑治疗对这些患者剩余生活质量的影响。
