Case Report: Coexistence of Giant Congenital Melanocytic Nevus and Holocord Spinal Nerve Sheath Tumor

Bilici M., Yilmaz M. C., Bilgic B., Saglam M., Ince H., Albayrak D., ...Daha Fazla

Fetal and Pediatric Pathology, 2025 (SCI-Expanded, Scopus) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Basım Tarihi: 2025
  • Doi Numarası: 10.1080/15513815.2025.2585053
  • Dergi Adı: Fetal and Pediatric Pathology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, BIOSIS, EMBASE, MEDLINE
  • Anahtar Kelimeler: Giant congenital melanocytic nevus, inoperable benign tumor, respiratory failure, spinal nerve sheath tumor, spinal surgery
  • İstanbul Üniversitesi Adresli: Evet

Özet

Background: Melanocytes and Schwann cells share a neural crest origin. Giant congenital melanocytic nevi (CMN) are linked to neurocutaneous melanocytosis (NCM), melanoma risk, and CNS anomalies. We report a case of giant CMN with a holocord intradural nerve sheath tumor. Case Presentation: A 19-month-old male with CMN presented for spinal mass treatment. History included recurrent pulmonary infections, severe neurological impairment, and developmental delay. Spinal MRI revealed an intradural-intramedullary mass extending from C5 to L1 (up to 2 cm thick), compressing nerve roots. Partial resection was performed, though the thoracic portion adhered irreversibly to the cord and roots. Pathology identified a hybrid nerve sheath tumor. Conclusion: To our knowledge, this is the first reported case of a spinal nerve sheat tumor associated with CMN in a pediatric patient. The surgery and pathology of the case demonstrated distinctive features. This unique case and its management are shared with the literature.