Rosai-Dorfman-Destombes disease: isolated intracranial without nodal involvement presentation. Illustrative case.

Background: Rosai-Dorfman-Destombes disease is a rare and heterogeneous entity that has been associated with autoimmune, hereditary, and malignant diseases. There is controversy about its etiopathogenesis, clinical course, and therapeutic management.

Observations: The authors report a case of a 61-year-old man with a history of progressive headache without any other symptoms. Magnetic resonance imaging of the brain revealed multiple irregular lesions with an initial diagnostic impression of meningiomatosis. An excisional biopsy was performed, and the pathology report stated the finding was Rosai-Dorfman-Destombes disease.

Lessons: The uniqueness of this case is its rarity. The isolated intracranial location presents many diagnostic and therapeutic challenges, with radiological and clinical characteristics similar to those of other central nervous system tumors. There is currently no clear evidence of the pathogenesis and therapeutic management of this condition. Follow-up of these patients will help elucidate the natural history of this condition and the benefits of various treatment modalities.