Abstract：
BACKGROUND: Rare cancers constitute less than 10% of head and neck cancers and lack sufficient evidence for standardized care. The French Rare Head and Neck Cancer Expert Network (REFCOR) as established a national database to collect data on these rare cancers. This study aims to describe patient and tumour characteristics in this database.
METHODS: Prospective data collection was conducted across multiple centers. Survival analyses were performed using Kaplan Meier method and Log Rank test. Odds ratios were used for comparing proportions.
RESULTS: A total of 7208 patients were included over a period of 10 years. The most frequent histologies were: Not Otherwise Specified (NOS) adenocarcinoma 13 %, adenoid cystic carcinoma 12 %, squamous cell carcinoma of rare locations 10 %, mucoepidermoid carcinoma 9 %, intestinal-type adenocarcinoma (8 %). Tumours were located in sinonasal area (38 %); salivary glands (32 %); oral cavity / oropharynx / nasopharynx (16 %); larynx / hypopharynx (3 %); ears (1 %); others (3 %). Tumours were predominantly classified as T4 (23 %), N0 (54 %), and M0 (62 %). Primary treatment approach involved tumour resection (78 %) and / or radiotherapy (63 %). Patients with salivary gland cancers exhibited better 5-year overall survival (OS) rates (p < 0.05), and lower recurrence rates compared to patients with sinonasal, laryngeal/ hypopharyngeal cancers. No significant differences were observed in the other comparisons. Acinar cell carcinoma demonstrated the best OS while mucous melanoma had the poorest prognosis.
CONCLUSIONS: Melanoma, carcinoma NOS, and sinonasal undifferenciated carcinoma still have poor prognoses. Efforts are being made, including training and guidelines, to expand network coverage (REFCOR, EURACAN), improve data collection and contribute to personalized therapies.
METHODS: Prospective data collection was conducted across multiple centers. Survival analyses were performed using Kaplan Meier method and Log Rank test. Odds ratios were used for comparing proportions.
RESULTS: A total of 7208 patients were included over a period of 10 years. The most frequent histologies were: Not Otherwise Specified (NOS) adenocarcinoma 13 %, adenoid cystic carcinoma 12 %, squamous cell carcinoma of rare locations 10 %, mucoepidermoid carcinoma 9 %, intestinal-type adenocarcinoma (8 %). Tumours were located in sinonasal area (38 %); salivary glands (32 %); oral cavity / oropharynx / nasopharynx (16 %); larynx / hypopharynx (3 %); ears (1 %); others (3 %). Tumours were predominantly classified as T4 (23 %), N0 (54 %), and M0 (62 %). Primary treatment approach involved tumour resection (78 %) and / or radiotherapy (63 %). Patients with salivary gland cancers exhibited better 5-year overall survival (OS) rates (p < 0.05), and lower recurrence rates compared to patients with sinonasal, laryngeal/ hypopharyngeal cancers. No significant differences were observed in the other comparisons. Acinar cell carcinoma demonstrated the best OS while mucous melanoma had the poorest prognosis.
CONCLUSIONS: Melanoma, carcinoma NOS, and sinonasal undifferenciated carcinoma still have poor prognoses. Efforts are being made, including training and guidelines, to expand network coverage (REFCOR, EURACAN), improve data collection and contribute to personalized therapies.
摘要：
背景：罕见癌症占头颈部癌症的比例不到10%，并且缺乏足够的标准化护理证据。法国罕见头颈部癌症专家网络（REFCOR）建立了一个国家数据库来收集这些罕见癌症的数据。本研究旨在描述该数据库中的患者和肿瘤特征。
方法：在多个中心进行前瞻性数据收集。使用KaplanMeier方法和LogRank检验进行生存分析。赔率用于比较比例。
结果：在10年的时间里，共纳入7208例患者。最常见的组织学是：非其他特定（NOS）腺癌13％，腺样囊性癌12%，罕见部位的鳞状细胞癌10%，粘液表皮样癌9%，肠型腺癌（8%）。肿瘤位于鼻窦区域（38％）;唾液腺（32％）;口腔/口咽/鼻咽（16％）;喉/下咽（3％）;耳朵（1％）;其他（3％）。肿瘤主要分类为T4（23％），N0（54%），和M0（62%）。主要治疗方法包括肿瘤切除（78％）和/或放疗（63％）。唾液腺癌患者表现出更好的5年总生存率（OS）（p<0.05），与鼻窦患者相比，复发率较低，喉/下咽癌。在其他比较中没有观察到显著差异。腺泡细胞癌表现出最好的OS，而粘液黑色素瘤预后最差。
结论：黑色素瘤，癌NOS,鼻窦无分化癌的预后仍然很差。正在努力,包括培训和指导方针，扩大网络覆盖(REFCOR，EURACAN)，改善数据收集并促进个性化治疗。
方法：在多个中心进行前瞻性数据收集。使用KaplanMeier方法和LogRank检验进行生存分析。赔率用于比较比例。
结果：在10年的时间里，共纳入7208例患者。最常见的组织学是：非其他特定（NOS）腺癌13％，腺样囊性癌12%，罕见部位的鳞状细胞癌10%，粘液表皮样癌9%，肠型腺癌（8%）。肿瘤位于鼻窦区域（38％）;唾液腺（32％）;口腔/口咽/鼻咽（16％）;喉/下咽（3％）;耳朵（1％）;其他（3％）。肿瘤主要分类为T4（23％），N0（54%），和M0（62%）。主要治疗方法包括肿瘤切除（78％）和/或放疗（63％）。唾液腺癌患者表现出更好的5年总生存率（OS）（p<0.05），与鼻窦患者相比，复发率较低，喉/下咽癌。在其他比较中没有观察到显著差异。腺泡细胞癌表现出最好的OS，而粘液黑色素瘤预后最差。
结论：黑色素瘤，癌NOS,鼻窦无分化癌的预后仍然很差。正在努力,包括培训和指导方针，扩大网络覆盖(REFCOR，EURACAN)，改善数据收集并促进个性化治疗。
