Rare Head and Neck Neuroendocrine Neoplasms: A Retrospective Study of Prognosis and Treatment Outcomes.

Cancer diagnosis & prognosis Pub Date : 2025-06-30 eCollection Date: 2025-07-01 DOI:10.21873/cdp.10460
Mioko Matsuo, Kenji Tsuchihashi, Yusuke Miyamoto, Kazuki Hashimoto, Ryunosuke Kogo, Noritaka Komune, Masanobu Sato, Shogo Masuda, Takashi Nakagawa
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Abstract

Background/aim: Neuroendocrine neoplasms (NEN) of the head and neck (HN) region are rare, with limited reported cases. NENs are classified into neuroendocrine tumors (NET) grades G1, G2, and G3, and neuroendocrine carcinomas (NECs), with varying treatment strategies. This study investigated patient outcomes of HN-NENs and proposed a treatment algorithm based on pathological classification.

Patients and methods: This retrospective study analyzed 24 HN-NEN cases treated at Kyushu University Hospital (2007-2023). Tumors were classified using the 2022 WHO criteria, and overall survival rates were evaluated using the Kaplan-Meier method.

Results: Among the 24 patients, 29% had NETs and 71% had NECs. The most common primary sites were the sinonasal cavity (42%) and larynx (29%). Seven-year survival rates were 100% for NET G1 and G2, 50% for NET G3, and 43% for NEC. Two NET cases treated with somatostatin analogs (SSA) and radionuclide therapy (PRRT) showed tumor reduction.

Conclusion: Prognosis of HN-NENs varies significantly by pathological grade. While NET G1 and G2 showed favorable outcomes, NET G3 and NEC had poorer survival. SSA and PRRT may be effective options for selected HN-NETs. A preliminary treatment algorithm is proposed to guide management, warranting validation in larger studies.

罕见头颈部神经内分泌肿瘤:预后和治疗结果的回顾性研究。
背景/目的:头颈部(HN)区域的神经内分泌肿瘤(NEN)是罕见的,报道病例有限。NENs分为神经内分泌肿瘤(NET) G1、G2、G3级和神经内分泌癌(NECs),治疗策略各不相同。本研究调查了HN-NENs患者的预后,并提出了一种基于病理分类的治疗算法。患者和方法:回顾性分析2007-2023年在九州大学医院治疗的24例HN-NEN病例。肿瘤采用2022年WHO标准进行分类,总体生存率采用Kaplan-Meier法进行评估。结果:24例患者中net占29%,nec占71%。最常见的原发部位是鼻窦(42%)和喉(29%)。NET G1和G2的7年生存率为100%,NET G3为50%,NEC为43%。经生长抑素类似物(SSA)和放射性核素治疗(PRRT)治疗的2例NET病例显示肿瘤缩小。结论:不同病理分级HN-NENs预后差异显著。NET G1和G2的预后良好,而NET G3和NEC的生存率较差。SSA和PRRT可能是某些HN-NETs的有效选择。提出了一种初步的治疗算法来指导管理,需要在更大规模的研究中进行验证。
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