Wei Julie L, Scheithauer Bernd W, Smith Justin S, Jenkins Robert B, Passe Sandra M, Arndt Carola A S, Strome Scott E
Department of Otorhinolaryngology, c/o Section of Scientific Publications, Mayo Clinic, Rochester, MN, USA.
Int J Pediatr Otorhinolaryngol. 2002 Apr 25;63(2):155-62. doi: 10.1016/s0165-5876(01)00650-4.
Although neuroblastoma is the most common of extracranial solid tumors of childhood and infancy, we report the first case of an isolated neuroblastoma of a paranasal sinus. A 15-year-old girl with a right maxillary sinus mass was asymptomatic except for persistent epiphora. Computed tomography and magnetic resonance imaging scans showed that the mass extended into the nasal cavity, encroached on the lamina papyracea, and obstructed the nasofrontal duct. An extensive workup revealed no evidence of systemic disease. The patient underwent right craniofacial resection. Immunohistochemistry and electronmicroscopic findings were consistent with conventional neuroblastoma. Fluorescence in situ hybridization analysis was performed with probes selected to demonstrate genetic alterations associated with neuroblastoma. Studies revealed deletion of chromosome arm 1p, gain of chromosome 17, and normal N-myc gene copy number. In summary, the tumor exhibited morphologic features and genetic alterations more consistent with those of neuroblastoma than with those of esthesioneuroblastoma.
尽管神经母细胞瘤是儿童期和婴儿期最常见的颅外实体瘤，但我们报告了首例孤立性鼻窦神经母细胞瘤病例。一名15岁女孩，右侧上颌窦有肿物，除持续溢泪外无其他症状。计算机断层扫描和磁共振成像扫描显示肿物延伸至鼻腔，侵犯纸样板，并阻塞鼻额管。全面检查未发现全身疾病的证据。患者接受了右侧颅面切除术。免疫组织化学和电子显微镜检查结果与传统神经母细胞瘤一致。使用选择的探针进行荧光原位杂交分析，以显示与神经母细胞瘤相关的基因改变。研究显示1号染色体短臂缺失、17号染色体增加以及N-myc基因拷贝数正常。总之，该肿瘤的形态学特征和基因改变与神经母细胞瘤更为一致，而非嗅神经母细胞瘤。
