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Melanotic neuroectodermal tumor of infancy disseminated by a ventriculoperitoneal shunt and diagnosed from the inguinal sac.

Abstract
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 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.
AuthorsIn Hyuk Yoo, Sook Kyung Yum, Se-Jeong Oh, Kyoung-Mee Kim, Dae Chul Jeong
JournalJournal of pediatric hematology/oncology (J Pediatr Hematol Oncol) Vol. 36 Issue 1 Pg. e61-4 (Jan 2014) ISSN: 1536-3678 [Electronic] United States
PMID23823108 (Publication Type: Case Reports, Journal Article)
Topics
  • Brain Neoplasms (pathology, surgery)
  • Child, Preschool
  • Fatal Outcome
  • Female
  • Humans
  • Hydrocephalus (surgery)
  • Inguinal Canal (pathology)
  • Neoplastic Cells, Circulating
  • Neuroectodermal Tumor, Melanotic (pathology, surgery)
  • Nevus (pathology)
  • Ventriculoperitoneal Shunt (adverse effects)

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