Wallin Jordan L, Tanna Neil, Misra Sasmita, Puri Puja K, Sadeghi Nader
The George Washington University School of Medicine, Washington DC, USA.
Am J Otolaryngol. 2007 Sep-Oct;28(5):360-2. doi: 10.1016/j.amjoto.2006.10.006.
Nevoid basal cell carcinoma syndrome (NBCCS) is associated with multiple basal cell carcinomas, odontogenic cysts, craniofacial anomalies, and childhood medulloblastomas. In addition, it has been associated with irradiation-induced neoplasms including, meningiomas, sarcomas, and gliomas.
We present a 19-year-old man with NBCCS who presented with a sinonasal carcinoma 17 years after receiving craniospinal irradiation for treatment of medulloblastoma.
To our knowledge, this is the first report of a sinonasal tumor after irradiation in a patient with NBCCS.
With this case, the authors examine the genotype of NBCCS patients and their propensity for radiation-induced tumors. In addition, the management of neoplasms in these tumor-sensitive patients is reviewed.
痣样基底细胞癌综合征(NBCCS)与多发性基底细胞癌、牙源性囊肿、颅面畸形及儿童髓母细胞瘤相关。此外,它还与辐射诱发的肿瘤有关,包括脑膜瘤、肉瘤和胶质瘤。
我们报告一名19岁患有NBCCS的男性,其在接受颅脊髓照射治疗髓母细胞瘤17年后出现鼻窦癌。
据我们所知,这是首例关于NBCCS患者放疗后发生鼻窦肿瘤的报告。
通过该病例,作者研究了NBCCS患者的基因型及其发生辐射诱发肿瘤的倾向。此外,还对这些肿瘤敏感患者的肿瘤管理进行了综述。