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Introduction <p>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.</p> Case Report <p>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.</p> Conclusion <p>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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年6月20日

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

摘要

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.