Melanotic neuroectodermal tumor of infancy: extra-axial lesion in the frontal midline of the skull – a case report

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Abstract Introduction Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a rare tumor originating from neural crest cells, most often affecting infants, particularly in the head and neck region. Case Report The patient, a 4-month-old boy, presented with an expansile extra-axial lesion in the frontal midline of the skull. The tumor exhibited small round cells and melanocyte-like cells containing dark brown pigment. Diagnosis was confirmed through histopathological and immunohistochemical exams, showing positivity for HMB-45, cytokeratin AE1/AE3 and synaptophysin, characteristic of MNTI. Early diagnosis is critical due to the tumor’s rapid growth, which can lead to local complications. Conclusion This case report highlights the importance of early diagnosis and monitoring of MNTI, due to its rapid growth and potential for local destruction. Histopathological and immunohistochemical confirmation is essential for accurate diagnosis and differentiation from other tumors.

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