Meningeal Melanocytoma Extruded From the Skull of a Neonate : Case Report

概要

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Ultrasonography performed at 21 weeks of gestation and magnetic resonance imaging at 23 weeks identified a huge tumor in the occipital region. Premature rupture of the membrane occurred at 28 weeks of gestation, so this baby boy was delivered by emergency cesarean section. Anemia and low blood pressure due to hemorrhage from the surface of the tumor required massive blood transfusion and hemostasis. The tumor was conservatively managed. He manifested symptoms of high output heart failure and persistent infection, necrotizing enterocolitis, and septicemia, and died 51 days after birth. Autopsy examination found the black tumor, weighing 885 g, with a clear boundary with the brain parenchyma. Histological examination showed the tumor was contiguous with the thickened dura mater. The tumor consisted of small, spindle-shaped, round, and polygonal cells without characteristic organized pattern, and positive for S-100 protein and neuron-specific enolase, and negative for HMB-45 and glial fibrillary acidic protein. Ultrastructural study revealed melanin granules within the tumor cells. The diagnosis was meningeal melanocytoma. His skin and spinal cord were unremarkable. Complete tumor resection is considered the best treatment option, followed by incomplete resection with postoperative radiotherapy, but we could not deliver aggressive treatment in our patient because of hemorrhage from the tumor surface and his poor general condition.
2010-03-15