Isolated Intramedullary Spinal Rosai–Dorfman Disease in a Child: A Case Report

Abstract

Rosai–Dorfman disease (RDD) is an uncommon benign histiocytic proliferative disorder commonly involving the cervical lymph nodes and, less frequently, extranodal sites. The histological hallmark of RDD is emperipolesis displayed by lesional histiocytes. Central nervous system involvement is rare and usually intracranial; intramedullary spinal involvement is even less common and, to our knowledge, rarely reported in children. Herein, we report a case of RDD with isolated intramedullary spinal involvement in a child, which, to our knowledge, is the first case reported in an infant.