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高级神经内分泌头颈部癌:病例系列及文献回顾。

High-grade neuroendocrine head and neck cancer: Case series and review of the literature.

机构信息

Velindre Cancer Centre, Cardiff, United Kingdom; Medical Oncology, Centro Integral Oncologico Clara Campal (CIOCC), Hospital Universitario HM Sanchinarro, Madrid, Spain.

Velindre Cancer Centre, Cardiff, United Kingdom.

出版信息

Curr Probl Cancer. 2024 Aug;51:101105. doi: 10.1016/j.currproblcancer.2024.101105. Epub 2024 May 31.

DOI:10.1016/j.currproblcancer.2024.101105
PMID:38823286
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 %.

CONCLUSION

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)高级别神经内分泌癌(NEC)较为罕见且侵袭性强,占所有 HN 癌症的比例≤1%,5 年总生存率(OS)≤20%。本病例系列研究旨在探讨过去 23 年在英国某区域 HN 癌症中心诊断的患者的临床特征、治疗方法和结局。

方法

对 2000 年 1 月 1 日至 2023 年 3 月 1 日期间在 Velindre 癌症中心诊断为 NEC HN 的所有患者的病历进行回顾性分析。

结果

研究期间共发现 19 例 NEC HN,主要影响男性(n=15,79%),中位年龄 67 岁(范围:44-86 岁)。诊断时,32%的患者(n=6)为吸烟者。最常见的原发肿瘤部位为喉(n=5,26.3%)和鼻腔鼻窦(n=5,26.3%)。大多数患者存在晚期局部区域疾病或远处转移,IV 期 A(n=6,32%)和 IV 期 C(n=6,32%)最为常见。关键的病理标志物是突触素,100%的检测患者(n=15)呈阳性。在这项研究中,12 例非转移性疾病患者中,10 例接受了包括放疗(RT)在内的联合治疗。其中一些患者在接受 RT 的同时还接受了化疗(CT)。2 例 II 期疾病患者仅接受手术治疗。7 例患者达到完全缓解,1 例患者达到部分缓解。7 例转移性患者中,3 例接受 CT 治疗,1 例接受姑息性 RT 治疗,均达到部分缓解。所有患者均使用卡铂和依托泊苷进行 CT 治疗。中位随访 11 个月(范围:1-96 个月)后,全组人群的中位 OS 为 27 个月,根治性治疗组为 51 个月,姑息性治疗组转移性患者为 3 个月。所有患者的 1 年 OS 为 54.3%,2 年 OS 为 46.5%,5 年 OS 为 23.3%。根治性治疗组的这些比例分别为 65.3%、52.2%和 26.1%。姑息性治疗组的 1 年 OS 为 33.3%。

结论

本病例系列研究初步观察了非转移性 NEC HN 的特征和治疗方法,提示多模式治疗策略可能具有潜在获益。鉴于该队列规模较小,这些观察结果应谨慎解读,作为进一步研究的基础。

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