Brennan Bernadette
Royal Manchester Children's Hospital, Hospital Road, M27 4HA Manchester, UK.
Orphanet J Rare Dis. 2006 Jun 26;1:23. doi: 10.1186/1750-1172-1-23.
Nasopharyngeal carcinoma (NPC) is a tumor arising from the epithelial cells that cover the surface and line the nasopharynx. The annual incidence of NPC in the UK is 0.3 per million at age 0-14 years, and 1 to 2 per million at age 15-19 years. Incidence is higher in the Chinese and Tunisian populations. Although rare, NPC accounts for about one third of childhood nasopharyngeal neoplasms. Three subtypes of NPC are recognized in the World Health Organization (WHO) classification: 1) squamous cell carcinoma, typically found in the older adult population; 2) non-keratinizing carcinoma; 3) undifferentiated carcinoma. The tumor can extend within or out of the nasopharynx to the other lateral wall and/or posterosuperiorly to the base of the skull or the palate, nasal cavity or oropharynx. It then typically metastases to cervical lymph nodes. Cervical lymphadenopathy is the initial presentation in many patients, and the diagnosis of NPC is often made by lymph node biopsy. Symptoms related to the primary tumor include trismus, pain, otitis media, nasal regurgitation due to paresis of the soft palate, hearing loss and cranial nerve palsies. Larger growths may produce nasal obstruction or bleeding and a "nasal twang". Etiological factors include Epstein-Barr virus (EBV), genetic susceptibility and consumption of food with possible carcinogens--volatile nitrosamines. The recommended treatment schedule consists of three courses of neoadjuvant chemotherapy, irradiation, and adjuvant interferon (IFN)-beta therapy.
鼻咽癌(NPC)是一种起源于覆盖鼻咽表面和衬里的上皮细胞的肿瘤。在英国,0至14岁人群中鼻咽癌的年发病率为百万分之0.3,15至19岁人群中为百万分之1至2。在中国和突尼斯人群中发病率更高。尽管罕见,但鼻咽癌约占儿童鼻咽肿瘤的三分之一。世界卫生组织(WHO)分类中确认了鼻咽癌的三种亚型:1)鳞状细胞癌,通常见于老年人群;2)非角化癌;3)未分化癌。肿瘤可在鼻咽内或向外扩展至其他侧壁和/或向后上方扩展至颅底、腭部、鼻腔或口咽。然后通常转移至颈部淋巴结。颈部淋巴结病是许多患者的首发表现,鼻咽癌的诊断通常通过淋巴结活检做出。与原发肿瘤相关的症状包括牙关紧闭、疼痛中耳炎、由于软腭麻痹导致的鼻反流、听力丧失和颅神经麻痹。较大的肿物可能导致鼻塞或出血以及“鼻音”。病因包括爱泼斯坦-巴尔病毒(EBV)、遗传易感性以及食用含有可能致癌物——挥发性亚硝胺的食物。推荐的治疗方案包括三个疗程的新辅助化疗、放疗和辅助干扰素(IFN)-β治疗。