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

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. 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. 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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Publication Details

Journal
Surgical and Experimental Pathology
Published
2025-06-19
DOI
https://doi.org/10.1186/s42047-025-00189-1
Primary Topic
Oral and Craniofacial Lesions
Type
article
Field-Weighted Citation Impact
0.00

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article

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

Bruna Louise Silva, Paulo Henrique Condeixa de França, Rafael Roesler, Karina Munhoz de Paula Alves Coelho et al.
Surgical and Experimental Pathology
Oral and Craniofacial Lesions
article

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

Bruna Louise Silva, Paulo Henrique Condeixa de França, Rafael Roesler, Karina Munhoz de Paula Alves Coelho, Hercílio Fronza, Camila Barbosa, Francis Rossetti Pedack, José Guilherme Jasper Pickler, Cindielly Ribeiro Dzevieski
article en

Abstract

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. 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. 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.

Surgical and Experimental PathologyVol. 8(1)
Universidade Federal do Rio Grande do Sul (BR), Universidade do Estado de Santa Catarina (BR), Hospital de Clínicas de Porto Alegre (BR), Laboratório Nacional de Ciência e Tecnologia do Bioetanol (BR), Centro Técnico de Automação (Brazil) (BR), Universidade da Região de Joinville (BR), Hospital Municipal São José (BR)
Ministério da Ciência, Tecnologia e Inovação, Conselho Nacional de Desenvolvimento Científico e Tecnológico
Openalex Percentile: Top 81%
Oral and Craniofacial Lesions
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