Pigmented epithelioid melanocytoma: a rare lytic bone lesion involving intradural extension and subtotal resection in a 14-month-old girl

J Neurosurg Pediatr. 2020 Mar 13:1-4. doi: 10.3171/2020.1.PEDS19359. Online ahead of print.

Abstract

Pigmented epithelioid melanocytomas (PEMs) are low-grade, intermediate-type borderline melanocytic tumors with limited metastatic potential. To date, PEMs have been treated via gross-total resections. Postoperative recurrence and mortality are rare. This case highlights a unique presentation of a PEM that involved bone destruction and intradural infiltration, which required a subtotal resection. To the authors' knowledge, this is the first report of a PEM extending through the dura and necessitating subtotal resection, which is contrary to the standard of care, gross-total resection. Surveillance imaging 10 months after resection remained negative for clinical and radiological recurrence.

Keywords: NGS = next-generation sequencing; PEM = pigmented epithelioid melanocytoma; oncology; pediatrics; pigmented epithelioid melanocytoma; subtotal resection.

Publication types

  • Case Reports