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Background <p>Neurocutaneous melanosis (NCM) is a rare congenital syndrome involving melanocytic tumors in the central nervous system (CNS) in association with giant melanocytic nevi.</p> Case presentation <p>This case details a 10-year-old male with NCM, presenting with an intraspinal hamartoma. Computed tomography revealed spinal cord compression. Pathology confirmed that the cutaneous lesions were nevi and that the hamartoma exhibited pigmentation, with positive staining for S100, HMB-45, and Fontana-Masson.</p> Conclusion <p>This case illustrates the embryological link between CNS and skin melanocytic proliferation.</p>

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Neurocutaneous melanosis in a pediatric patient with intraspinal hamartoma presentation

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年6月10日

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  • Camila Barbosa,
  • Hercilio Fronza Junior,
  • Simone Muller,
  • Thiago Traesel,
  • Bruna Louise Silva,
  • Paulo Henrique Condeixa de França,
  • Karina Munhoz de Paula Alves Coelho

摘要

Background

Neurocutaneous melanosis (NCM) is a rare congenital syndrome involving melanocytic tumors in the central nervous system (CNS) in association with giant melanocytic nevi.

Case presentation

This case details a 10-year-old male with NCM, presenting with an intraspinal hamartoma. Computed tomography revealed spinal cord compression. Pathology confirmed that the cutaneous lesions were nevi and that the hamartoma exhibited pigmentation, with positive staining for S100, HMB-45, and Fontana-Masson.

Conclusion

This case illustrates the embryological link between CNS and skin melanocytic proliferation.