Abstract：
Among the 16,000 new cases of malignant tumors of the head and neck diagnosed in France each year, 10% are not conventional squamous cell carcinomas. These so-called rare cancers are distinguished by their presentation and patterns of failure, which is important to recognize in order to offer specific adapted management and maximize the chances of tumor control. These cancers can be rare by their histology as well as their anatomical location when arising from the paranasal sinuses, salivary glands and ear. The management of these heterogeneous rare diseases of complex treatment has considerably been structured over the last 15 years, in particular via the French ENT Cancer Expertise Network (REFCOR) and international networks and registries (EURACAN, etc.). Structuration also favors research with identification of new entities and setting up of specific therapeutic trials. A first article (part 1) discusses the diagnostic and therapeutic specificities of these rare cancers, and develops the recommendations of the REFCOR concerning rare epithelial tumors, i.e., salivary tumors, sinonasal tumors, variants of conventional squamous cell carcinomas, neuroendocrine carcinomas, malignant odontogenic tumors, and ear tumors. This second article (part 2) is focused on non-epithelial tumors (sarcomas, mucosal melanomas, lymphomas, tumors of uncertain or undetermined malignancy) and describes the organization and missions of the REFCOR.
摘要：
在法国每年诊断的16,000例新的头颈部恶性肿瘤病例中，10%不是常规鳞状细胞癌。这些所谓的罕见癌症的特点是它们的表现和失败模式，为了提供特定的适应性管理并最大化肿瘤控制的机会，认识到这一点很重要。这些癌症可能是罕见的组织学以及它们的解剖位置时，从鼻旁窦。唾液腺和耳朵。在过去的15年中，这些复杂治疗的异质性罕见疾病的管理已经有了相当大的结构，特别是通过法国耳鼻喉科癌症专家网络(REFCOR)和国际网络和注册机构(EURACAN，等。).结构化也有利于识别新实体和建立特定治疗试验的研究。第一篇文章（第1部分）讨论了这些罕见癌症的诊断和治疗特异性，并制定了REFCOR关于罕见上皮肿瘤的建议，即，唾液肿瘤，鼻窦肿瘤,常规鳞状细胞癌的变体，神经内分泌癌，恶性牙源性肿瘤，还有耳部肿瘤.这第二篇文章（第2部分）专注于非上皮性肿瘤（肉瘤，粘膜黑素瘤，淋巴瘤,不确定或不确定的恶性肿瘤），并描述了REFCOR的组织和任务。
