Intracranial Rosai-Dorfman Disease

Abstract

Rosai-Dorfman disease (RDD) is a rare, benign pseudolymphomatous condition, predominantly involving lymph nodes. Rosai-Dorfman disease (RDD) (sinus histiocytes with massive lymphadenopathy) rarely affects the intracranial region without involvement of other sites. It is a rare and idiopathic histoproliferation disorder characterized by painless lymphadenopathy. We report a case of 43-year-old male who presented with unconsciousness; MRI was done and right temporofrontal mass was found. Excision was done, and on histopathology it confirmed RDD.

Bifocal Intracanial Rosai-Dorfman Disease Mimicking Lymphoplasmacyte-Rich Meningioma: Diagnostic Pitfalls About a Case Report

“…Extranodal involvement is documented in 43% of the patients. 2 The CNS can be involved in less than 5% of the Rosai-Dorfman disease. In 90% of these cases the leptomeninges are affected.…”

“…1 Extranodal involvement is documented in 43% of the patients. Primary central nervous involvement is extremely rare (less than 5%); 2 , 3 in such location a wide morphologic spectrum is encountered. This complicates the diagnosis and delays the therapeutic management.…”

Imaging findings of head and neck and intracranial sinus histiocytosis with massive lymphadenopathy (Rosai–dorfman disease): A rare case report

“…Rosai-Dorfman disease (RDD) is a benign lymphohistiocytosis characterized by multiple enlarged cervical lymph nodes. RDD was first described by Destombes in 1965 and was first reported by Juan Rosai and Roland Dorfman as sinus histiocytosis with massive lymphadenopathy in 1969 (1). It is a very rare disease, with a prevalence of 1:200,000 with unknown etiology (2).…”

“…RDD tends to affect children and young people (1)(2)(3). Most of the patients (83%-95%) present with enlarged cervical lymph nodes (2).…”

“…Up to 43% of patients show extranodal involvement including the soft tissues of the head and neck, paranasal sinuses, and nasal cavity (3). The central nervous system is involved in less than 5% of the cases, predominantly with extra-axial dural-based masses (1,4).…”