Melanotic Neuroectodermal Tumors in Pediatric Oncology

Melanotic neuroectodermal tumours of infancy (MNTI) are rare, pigmented neoplasms derived from neural crest cells. They typically present within the first year of life, most often affecting the anterior maxilla but occasionally arising in the mandible, calvarium or other soft tissues. Clinically, MNTI manifest as rapidly expanding, painless masses that can displace tooth germs and cause local bone erosion. Histologically, lesions display a biphasic cellular pattern comprising small, neuroblast-like cells and larger, melanin-producing epithelial cells, reflecting dual neural and melanocytic differentiation. Diagnosis relies on imaging—ultrasonography, computed tomography and magnetic resonance imaging—to delineate tumour extent, coupled with immunohistochemical staining for markers such as HMB-45, Melan-A and synaptophysin. Surgical excision remains the primary treatment, with margin size carefully balanced to reduce recurrence risk while preserving facial form and function. Recurrence rates range from 20–60%, prompting exploration of adjuvant therapies and underscoring the need for long-term surveillance to detect late relapses and guide reconstructive efforts.

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Melanotic Neuroectodermal Tumors in Pediatric Oncology publication trend

The graph below shows the total number of articles in melanotic neuroectodermal tumors in pediatric oncology across all publications each year (not limited to Nature Index journals).

Technical terms

Neural crest cells: Embryonic cells that migrate to form diverse tissues, including melanocytes and peripheral nervous system components.

DNA methylation profiling: A technique to assess genome-wide methylation patterns, enabling tumour classification by epigenetic signature.

Copy number variation (CNV): Gains or losses of genomic regions that can contribute to oncogenesis and influence prognosis.

Immunohistochemistry: A laboratory method using labelled antibodies to detect specific antigens in tissue sections for diagnostic confirmation.

References

1. Melanotic Neuroectodermal Tumor of Infancy (MNTI) and Pineal Anlage Tumor (PAT) Harbor A Medulloblastoma Signature by DNA Methylation Profiling. Cancers (2021).
2. Clinical and imaging features of melanotic neuro-ectodermal tumor of infancy of the maxillary bone: report of four cases and review of the literature. Oral Radiology (2022).
3. Clinical and immunohistochemical study of melanotic neuroectodermal tumor of infancy in the maxilla. Einstein (São Paulo) (2018).
4. Melanotic neuroectodermal tumor of infancy: A case report and review of the surgical treatment. Oncology Letters (2014).