Article record

专家中心治疗的罕见头颈癌的诊疗模式与质量：欧洲罕见成人实体癌参考网络（EURACAN）注册登记的经验

Pattern and quality of care for rare head and neck cancers treated in expert centres: the experience of the registry of the European Reference Network on Rare Adult Solid Cancers (EURACAN).

临床研究鼻科IF 8.8Q1

文献信息

PMID	42772165
原文	在 PubMed 查看原文 ↗
发表日期	2026
作者	A Trama 查看其他 26 位作者 L Licitra；C Bergamini；M Gil Sanjines；S Bonfarnuzzo；E Orlandi；B Vischioni；M Tagliabue；R De Berardinis；M Ansarin；V Manciocco；F Nardozza；G Mercante；A Mirabile；G Mari；F Gaino；P de Franco；E Cavalera；V Gregorc；V Valentini；P Bonomo；M Ferrari；L D Locati；J Galli；L Sacchetto；S Cavalieri；L Botta
作者单位	Evaluative Epidemiology Unit, Department of Epidemiology and Data Science, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. Electronic address: annalisa.trama@istitutotumori.mi.it. 查看其他 19 条单位信息 Head and Neck Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan, Italy; Department of Oncology and Hemato-oncology, University of Milan, Milan, Italy.；Evaluative Epidemiology Unit, Department of Epidemiology and Data Science, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.；Data Science Unit, Department of Epidemiology and Data Science, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.；Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Pavia, Italy; Clinical-Scientific Department, National Center for Oncological Hadrontherapy (CNAO), Pavia, Italy.；Radiotherapy Unit, National Center for Oncological Hadrontherapy (CNAO), Pavia, Italy.；Department of Otorhinolaryngology-Head and Neck Surgery, European Institute of Oncology IRCCS, Milan, Italy.；Otorhinolaryngology Head and Neck Surgical Unit, IRCCS Regina Elena National Cancer Institute, Rome, Italy.；Department of Research and Advanced Technologies, Clinical Trial Center, Biostatistics and Bioinformatics Unit, IRCCS Regina Elena National Cancer Institute, Rome, Italy.；Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum - University of Bologna, Bologna, Italy; Department of Otolaryngology - Head and Neck Surgery, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.；Department of Otolaryngology, IRCCS Ospedale San Raffaele, Milan, Italy.；Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy; Department of Otorhinolaryngology - Head and Neck Surgery, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy.；Radiation Oncology Unit, Department of Onco-Hematology, "Vito Fazzi" Hospital, Lecce, Italy.；Clinical Research and Innovation Department, INOC - Istituto Nazionale Oncologico Candiolo; IRCCS, Candiolo (TO), Italy.；Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Rome, Italy; Department of Maxillofacial Surgery, Azienda Ospedaliero-Universitaria Policlinico Umberto I, Viale del Policlinico 155, 00161 Rome.；Department of Radiation Oncology, Azienda Ospedaliero-Universitaria Careggi, Florence, Italy.；Section of Otorhinolaryngology - Head and Neck Surgery, Department of Neuroscience, University of Padova, Padova, Italy; Unit of Otorhinolaryngology, Department of Surgery, Azienda Ospedale-Università Padova, Padova, Italy.；Department of Internal Medicine and Medical Therapeutics, University of Pavia, Pavia, Italy; Medical Oncology Unit, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy.；Department of Neuroscience, Sense Organs and Thorax, Otorhinolaryngology Unit, Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.；Department of Surgical, Odontostomatological and Maternal-Child Sciences, University of Verona, Verona, Italy.
期刊	ESMO open
SCI 分区	Q1
IF	8.8
研究类型	临床研究 · 临床
所属专科	鼻科

中文摘要

背景： 罕见头颈癌（HNCs），包括鼻腔鼻窦癌（SNC）、鼻咽癌（NPC）和唾液腺癌（SGC），具有生物学多样性且发病率低，导致指导临床管理的高质量证据有限。本研究在欧洲罕见成人实体癌参考网络（EURACAN）注册登记框架内，评估了意大利这些罕见HNCs的真实世界诊疗模式与质量。
患者与方法： 分析了2018年至2024年间在意大利专家中心诊断或管理的795例罕见HNCs成人患者（152例SNC、201例NPC和442例SGC）的数据。评估的关键指标包括诊断完整性、对欧洲临床实践指南的依从性以及治疗的及时性。分析按肿瘤类型和诊疗环境进行分层。
结果： 大多数患者就诊时已为局部晚期疾病（SNC 70%、NPC 74%和SGC 50%）。80%的NPC病例进行了Epstein-Barr病毒检测。治疗方式异质性较大，尤其是SNC，其多模式治疗差异很大。指南偏离很常见：约50%的局部SGC患者和67%的局部NPC患者未接受指南推荐的治疗。治疗延迟频繁发生，48%-77%的患者未能在推荐时间范围内开始治疗。这些延迟主要与院际转诊路径相关，并且在需要多模式治疗的患者中更为明显。
结论： 这项基于注册登记的分析揭示了罕见HNCs治疗策略的显著变异性以及频繁的治疗延迟，即使在专家中心也是如此。改善转诊协调、提高指南依从性以及及时启动治疗仍然至关重要。EURACAN注册登记是基准比较和提升诊疗质量的有价值工具，可为这些罕见且复杂的癌症提供循证治疗决策支持。

英文摘要

BACKGROUND: Rare head and neck cancers (HNCs), including sinonasal cancers (SNC), nasopharyngeal cancers (NPC), and salivary gland cancers (SGC), are biologically diverse and infrequent, resulting in limited high-quality evidence to guide clinical management. This study evaluated real-world patterns and quality of care in Italy for these rare HNCs within the European Reference Network for Rare Adult Solid Cancers (EURACAN) registry.
PATIENTS AND METHODS: Data from 795 adult patients diagnosed or managed at Italian expert centres between 2018 and 2024 with rare HNCs (152 SNC, 201 NPC, and 442 SGC) were analysed. Key indicators assessed included diagnostic completeness, adherence to European clinical practice guidelines, and timeliness of treatment. Analyses were stratified by tumour type and care setting.
RESULTS: Most patients presented with locally advanced disease (70% SNC, 74% NPC, and 50% SGC). Epstein-Barr virus testing was carried out in 80% of NPC cases. Treatment approaches were heterogeneous, especially for SNC, where multimodal therapy varied widely. Guidelines deviations were common: ∼50% of patients with localised SGC and 67% of those with localised NPC did not receive guideline-recommended treatments. Treatment delays were frequent, with 48%-77% of patients failing to initiate treatment within recommended timeframes. These delays were predominantly associated with interhospital referral pathways and were more pronounced in patients requiring multimodal treatments.
CONCLUSIONS: This registry-based analysis reveals substantial variability in treatment strategies and frequent treatment delays for rare HNCs, even within expert centres. Improving referral coordination, adherence to guidelines, and timely initiation of treatment remain critical. The EURACAN registry represents a valuable tool for benchmarking and enhancing care quality, supporting evidence-informed treatment decisions for these rare and complex cancers.