Melanotic Neuroectodermal Tumor of Infancy Disseminated by a Ventriculoperitoneal Shunt and Diagnosed from the Inguinal Sac

In Hyuk Yoo, Sook Kyung Yum, Se-Jeong Oh, Kyoung-Mee Kim, Dae Chul Jeong
2014 Journal of pediatric hematology/oncology  
Melanotic neuroectodermal tumor of infancy (MNTI) is a rare congenital neoplasm that originates from the neural crest cells, which give rise to the melanocytes of the skin and leptomeninges. We report a case of MNTI with neurocutaneous melanosis of a 28-month-old girl. She was born with hydrocephalus and several large congenital giant nevi. There were no findings except for hydrocephalus, after a ventriculoperitoneal (VP) shunt operation performed when she was 6 months old. She was operated on
more » ... he was operated on for a growing inguinal mass at 8 months. The specimen from the inguinal sac was positive for HMB45, vimentin, chromogranin, and neuron-specific enolase. Brain magnetic resonance imaging showed an extensive enhancing extra-axial mass with high signal intensity, along the cerebral spinal fluid space. We report a rare case of MNTI, diagnosed from an inguinal hernia sac, with a disseminated clinical manifestation.
doi:10.1097/mph.0b013e31829dd114 pmid:23823108 fatcat:b2iznpev4javzjcistcb5kjynm