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首页> 外文期刊>Oral surgery >Basal cell naevoid syndrome with nasopharyngeal carcinoma: a case report
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Basal cell naevoid syndrome with nasopharyngeal carcinoma: a case report

机译：基底细胞痣样综合征伴鼻咽癌：病例报告

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摘要

Basal cell naevoid syndrome is a rare autosomal dominant multisystem disorder characterised by multiple basal cell naevi, keratocystic odontogenic tumours, a variety of developmental anomalies and a predisposition to neoplasms, in particular basal cell carcinomas. A 15-year-old female patient with basal cell naevoid syndrome presented with severe headaches waking her at night, spontaneous nosebleeds, fatigue, increased sleep and decreased appetite. A CT head showed a large right-sided nasopharyngeal mass. The patient underwent nasopharyngoscopy with biopsy which confirmed a nasopharyngeal carcinoma. MRI and a contrast-enhanced CT scan of the head and neck suggested a nasopharyngeal carcinoma with skull base involvement. The carcinoma was treated successfully with chemotherapy and radiotherapy. She remains in remission at 10-month review. To our knowledge, this is the first report of a basal cell naevoid syndrome patient diagnosed with nasopharyngeal carcinoma. Clinicians should be aware of the possibility of head and neck neoplasms in patients with basal cell naevoid syndrome.
机译：基底细胞痣样综合征是一种罕见的常染色体显性遗传多系统疾病，其特征是多发性基底细胞痣、角化囊性牙源性肿瘤、多种发育异常和肿瘤易感性，尤其是基底细胞癌。一名 15 岁女性基底细胞痣状综合征患者出现剧烈头痛，夜间醒来，自发性流鼻血，乏力，睡眠增加和食欲下降。头部 CT 显示右侧鼻咽部肿块较大。患者行鼻咽镜活检，确诊为鼻咽癌。MRI 和头颈部增强 CT 扫描提示颅底受累的鼻咽癌。该癌通过化疗和放疗成功治疗。在 10 个月的复查中，她仍处于缓解状态。据我们所知，这是基底细胞痣样综合征患者被诊断患有鼻咽癌的第一份报告。临床医生应意识到基底细胞痣综合征患者发生头颈部肿瘤的可能性。

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