Extranodal Intracranial Rosai-Dorfman-Destombes Disease in Children: a Literature Review

Rosai-Dorfman-Destombes (RDD) disease also known as sinus histiocytosis with massive lymphadenopathy is a rare disease characterized by disorder of histiocytes within lymph nodes or other affected tissues. Extranodal forms of disease are even more uncommon. Only small percent of patients present with intracranial extranodal form of disease. Lesion biopsy and characteristic histopathological findings are crucial for definitive diagnosis. Histological findings typically show emperipolesis of affected histiocytes or hyperplastic lymph node sinuses containing enlarged histiocytes with phagocyted intact lymphocytes with specific immunoassay findings of the characteristic histiocytic non-Langerhans cells. Therapy includes various modalities such as surgery, steroids, radiotherapy, and/or chemotherapy. In case of extranodal intracranial form of disease, resection (single or combined with other treatment) was reported as effective. There are only few cases of extranodal intracranial disease in pediatric population described in the world so far. We aimed to provide brief comprehensive review of published cases referring on pediatric extranodal intracranial RDD.