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[Melanotic neuroectodermal tumor in an infant]

C Chossegros1, F Cheynet, J C Gentet

  • 1Clinique de stomatologie et de chirurgie maxillofaciale, CHU Timone, Marseille, France.

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|June 1, 1995
PubMed
Summary

Melanotic neuroectodermal tumor, a rare childhood condition, can recur after initial treatment. Early surgical intervention, like maxillectomy, is crucial for managing this odontogenic tumor.

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Area of Science:

  • Pediatric Oncology
  • Oral and Maxillofacial Surgery
  • Dermatopathology

Background:

  • Melanotic neuroectodermal tumor (MNT) is a rare, predominantly benign neoplasm.
  • Typically affects the head and neck region, with a predilection for infants and young children.

Observation:

  • A case of a rapidly growing, cystic premaxillary tumor in a two-month-old girl is presented.
  • The tumor recurred with an osteogenic appearance after initial enucleation, despite normal catecholamine levels and negative MIBG scintigraphy.
  • Histopathological examination revealed melanocytes, confirming the diagnosis of melanotic neuroectodermal tumor.

Findings:

  • This case highlights the potential for recurrence of melanotic neuroectodermal tumors.
  • Diagnostic imaging (CT scan) demonstrated changes in tumor appearance post-excision.

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  • Electron microscopy was crucial in identifying melanocytic origin.

    • Implications:

    • Early and complete surgical excision is vital for managing MNT.
    • An oral surgical approach can yield favorable cosmetic outcomes.
    • Further research into MNT recurrence factors may be warranted.