Zhang Jian-guo, Zhao Rui-jiao, Kong Ling-fei
Department of Neurosurgery, Henan Provincial People's Hospital, Zhengzhou, Henan, People's Republic of China.
Department of Pathology, Henan Provincial People's Hospital, Zhengzhou, Henan, People's Republic of China.
J Child Neurol. 2015 Apr;30(5):631-4. doi: 10.1177/0883073813494477. Epub 2013 Aug 20.
Melanotic neuroectodermal tumor of infancy is a rare melanin-containing neoplasm with locally aggressive and rapid expansile growth, usually involving the maxilla, skull, and mandible of early infancy. Radical surgery is critical for a long-term outcome. We present a case of 14-month-old girl with rapid-growing subcutaneous mass arising in the right temporal bone and extending intracranially on computed tomographic scan. Radical surgery was performed. A brownish-black tumor composed of large pigmented epithelioid cells, positive for cytokeratins and HMB-45, and nests of small neuroblast-like cells positive for neuron-specific enolase and synaptophysin, was diagnosed as melanotic neuroectodermal tumor of infancy. The patient remained well without evidence of recurrence for 1 year after surgery. Clinicopathological features, management alternatives and outcome were discussed.
婴儿黑色素性神经外胚层肿瘤是一种罕见的含黑色素肿瘤，具有局部侵袭性和快速扩展性生长，通常累及婴儿早期的上颌骨、颅骨和下颌骨。根治性手术对于长期预后至关重要。我们报告一例14个月大女童，其右侧颞骨出现快速生长的皮下肿块，计算机断层扫描显示肿块向颅内延伸。实施了根治性手术。一个由大的色素沉着上皮样细胞组成的棕黑色肿瘤，细胞角蛋白和HMB-45呈阳性，以及小神经母细胞样细胞巢，神经元特异性烯醇化酶和突触素呈阳性，被诊断为婴儿黑色素性神经外胚层肿瘤。术后1年患者情况良好，无复发迹象。讨论了临床病理特征、治疗选择和预后。
