Pontine Rosai-Dorfman disease in a child

Rosai-Dorfman Disease (RDD) is a benign histiocytic disorder that commonly presents with massive lymphadenopathy. Central nervous system (CNS) involvement is only 5%; isolated CNS involvement is much rarer. The patient presented here has been diagnosed with isolated pontine RDD.

A 5-year-old child was admitted to the hospital with the history of double vision and ptosis of the left eye. Magnetic resonance imaging revealed presence of a mass at the pontomesencephalic junction. Subtotal tumor excision was performed. Microscopic examination of the lesion showed histologic features consistent with RDD. The patient was followed up without medication, with a small residue.

RDD is rare in childhood, and isolated CNS involvement is also quite rare. The tumor is located in pons in our case. The treatment of choice is gross total resection. Even total excision is not possible, follow up without medication may be an option because of the indolent and self-limited course of the disease.