Isolated Cervical Lymphadenopathy in Pediatric Rosai-Dorfman Disease: A Case Report and Literature Review

Rosai-Dorfman disease (RDD) is a rare, benign histiocytic disorder that typically presents with bilateral cervical lymphadenopathy. Pediatric cases with isolated, unilateral involvement are uncommon and may pose diagnostic challenges. We report the case of an 11-year-old boy presenting with isolated, unilateral cervical lymphadenopathy without systemic symptoms. Biological and radiological investigations were nonspecific. Histopathological examination of the excised lymph node revealed characteristic large histiocytes with emperipolesis and an immunohistochemical profile consistent with RDD (CD163+, CD1a-, and CD20-). The patient was successfully managed with surgical excision alone, with no recurrence or need for additional therapy. This case highlights that RDD may present as isolated, unilateral cervical lymphadenopathy in children and can be effectively managed with surgery alone in selected cases. Awareness of this atypical presentation is essential to ensure accurate diagnosis and avoid unnecessary medical treatment.