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.
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Authors | In Hyuk Yoo, Sook Kyung Yum, Se-Jeong Oh, Kyoung-Mee Kim, Dae Chul Jeong |
Journal | Journal of pediatric hematology/oncology
(J Pediatr Hematol Oncol)
Vol. 36
Issue 1
Pg. e61-4
(Jan 2014)
ISSN: 1536-3678 [Electronic] United States |
PMID | 23823108
(Publication Type: Case Reports, Journal Article)
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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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