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

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.