Abstract：
BACKGROUND: High-grade neuroendocrine cancers (NEC) of the head and neck (HN) are rare and aggressive, accounting for ≤1 % of all HN cancers, with a 5-year overall survival (OS) of ≤20 %. This case series examines clinical characteristics, treatments, and outcomes of patients diagnosed at a regional UK HN cancer centre over the last 23 years.
METHODS: A retrospective review of medical records was conducted for all patients diagnosed with NEC HN from 1st January 2000 until 1st March 2023 at Velindre Cancer Centre.
RESULTS: During the study period, 19 cases of NEC HN were identified, primarily affecting males (n = 15, 79 %). Median age of 67 years (range: 44-86). At diagnosis, 32 % of patients (n = 6) were smokers. The most common primary tumour sites were larynx (n = 5, 26.3 %) and sinonasal (n = 5, 26.3 %). Most patients presented with advanced loco-regional disease or distant metastasis, with stage IVA (n = 6, 32 %) and stage IVC (n = 6, 32 %) being the most common. The key pathology marker was synaptophysin, present in 100 % of the tested patients (n = 15). In the study, of the 12 patients with non-metastatic disease, 10 received a combination of treatments that included radiotherapy (RT). Some of these patients also received chemotherapy (CT) at the same time as their radiotherapy. Surgery alone was used in two patients with stage II disease. Seven subjects had complete responses, and one achieved a partial response. Among the seven metastatic patients, three received CT, and one underwent palliative RT, all achieving a partial response. In all cases, the CT used was carboplatin and etoposide. After a median follow-up of 11 months (range: 1-96), the median OS was 27 months for the overall population, 51 months for those treated radically, and three months for metastatic patients with palliative treatment. The 1-year OS for all patients was 54.3 %, the 2-year OS was 46.5 %, and the 5-year OS was 23.3 %. Among patients treated radically, these rates were 65.3 %, 52.2 %, and 26.1 %, respectively. For patients treated palliatively, the 1-year OS was 33.3 %.
CONCLUSIONS: This case series contributes preliminary observations on the characteristics and management of non-metastatic NEC HN, suggesting potential benefits from multimodality treatment strategies. Given the small cohort size, these observations should be interpreted cautiously and seen as a foundation for further research.
METHODS: A retrospective review of medical records was conducted for all patients diagnosed with NEC HN from 1st January 2000 until 1st March 2023 at Velindre Cancer Centre.
RESULTS: During the study period, 19 cases of NEC HN were identified, primarily affecting males (n = 15, 79 %). Median age of 67 years (range: 44-86). At diagnosis, 32 % of patients (n = 6) were smokers. The most common primary tumour sites were larynx (n = 5, 26.3 %) and sinonasal (n = 5, 26.3 %). Most patients presented with advanced loco-regional disease or distant metastasis, with stage IVA (n = 6, 32 %) and stage IVC (n = 6, 32 %) being the most common. The key pathology marker was synaptophysin, present in 100 % of the tested patients (n = 15). In the study, of the 12 patients with non-metastatic disease, 10 received a combination of treatments that included radiotherapy (RT). Some of these patients also received chemotherapy (CT) at the same time as their radiotherapy. Surgery alone was used in two patients with stage II disease. Seven subjects had complete responses, and one achieved a partial response. Among the seven metastatic patients, three received CT, and one underwent palliative RT, all achieving a partial response. In all cases, the CT used was carboplatin and etoposide. After a median follow-up of 11 months (range: 1-96), the median OS was 27 months for the overall population, 51 months for those treated radically, and three months for metastatic patients with palliative treatment. The 1-year OS for all patients was 54.3 %, the 2-year OS was 46.5 %, and the 5-year OS was 23.3 %. Among patients treated radically, these rates were 65.3 %, 52.2 %, and 26.1 %, respectively. For patients treated palliatively, the 1-year OS was 33.3 %.
CONCLUSIONS: This case series contributes preliminary observations on the characteristics and management of non-metastatic NEC HN, suggesting potential benefits from multimodality treatment strategies. Given the small cohort size, these observations should be interpreted cautiously and seen as a foundation for further research.
摘要：
背景：头颈部（HN）的高级神经内分泌癌（NEC）是罕见且具有侵袭性的，占所有HN癌症的≤1%，5年总生存率（OS）≤20％。这个病例系列检查了临床特征，治疗,以及过去23年在英国地区HN癌症中心诊断的患者的结局。
方法：对2000年1月1日至2023年3月1日在Velindre癌症中心诊断为NECHN的所有患者的病历进行回顾性回顾。
结果：在研究期间，确定了19例NECHN，主要影响男性（n=15，79%）。年龄中位数为67岁（范围：44-86）。诊断时,32％的患者（n=6）是吸烟者。最常见的原发肿瘤部位是喉（n=5，26.3％）和鼻窦（n=5，26.3％）。大多数患者表现为晚期局部区域疾病或远处转移，最常见的是IVA阶段(n=6，32%)和IVC阶段(n=6，32%)。关键的病理标记是突触素,存在于100%的测试患者中(n=15)。在研究中,在12例非转移性疾病患者中，10接受了包括放射疗法(RT)的治疗组合。其中一些患者在放疗的同时也接受了化疗（CT）。两名II期患者仅使用手术。七个受试者有完整的回答，一个人得到了部分回应。在7名转移性患者中，三人接受了CT检查,一个人接受了姑息性RT，都实现了部分回应。在所有情况下,使用的CT是卡铂和依托泊苷。经过11个月的中位随访（范围：1-96），总人口的中位OS为27个月，对于那些接受彻底治疗的人来说是51个月，转移性患者接受姑息治疗3个月。所有患者的1年OS为54.3%,两年OS为46.5%，5年OS为23.3%。在接受彻底治疗的患者中，这些比率是65.3%，52.2%,和26.1%，分别。对于姑息治疗的患者，1年OS为33.3%。
结论：本病例系列有助于对非转移性NECHN的特征和治疗的初步观察，提示多模式治疗策略的潜在益处。鉴于队列规模较小，这些观察结果应谨慎解释，并被视为进一步研究的基础。
方法：对2000年1月1日至2023年3月1日在Velindre癌症中心诊断为NECHN的所有患者的病历进行回顾性回顾。
结果：在研究期间，确定了19例NECHN，主要影响男性（n=15，79%）。年龄中位数为67岁（范围：44-86）。诊断时,32％的患者（n=6）是吸烟者。最常见的原发肿瘤部位是喉（n=5，26.3％）和鼻窦（n=5，26.3％）。大多数患者表现为晚期局部区域疾病或远处转移，最常见的是IVA阶段(n=6，32%)和IVC阶段(n=6，32%)。关键的病理标记是突触素,存在于100%的测试患者中(n=15)。在研究中,在12例非转移性疾病患者中，10接受了包括放射疗法(RT)的治疗组合。其中一些患者在放疗的同时也接受了化疗（CT）。两名II期患者仅使用手术。七个受试者有完整的回答，一个人得到了部分回应。在7名转移性患者中，三人接受了CT检查,一个人接受了姑息性RT，都实现了部分回应。在所有情况下,使用的CT是卡铂和依托泊苷。经过11个月的中位随访（范围：1-96），总人口的中位OS为27个月，对于那些接受彻底治疗的人来说是51个月，转移性患者接受姑息治疗3个月。所有患者的1年OS为54.3%,两年OS为46.5%，5年OS为23.3%。在接受彻底治疗的患者中，这些比率是65.3%，52.2%,和26.1%，分别。对于姑息治疗的患者，1年OS为33.3%。
结论：本病例系列有助于对非转移性NECHN的特征和治疗的初步观察，提示多模式治疗策略的潜在益处。鉴于队列规模较小，这些观察结果应谨慎解释，并被视为进一步研究的基础。
