Isolated Intraventricular Rosai-Dorfman Disease

Area of Science:

• Neuropathology
• Histopathology
• Oncology

Background:

• Rosai-Dorfman disease (RDD) is a rare benign histioproliferative disorder.
• It typically presents with lymphadenopathy and can involve extranodal sites.
• Central nervous system (CNS) involvement is infrequent, usually occurring in older individuals.

Observation:

• This report details an exceptionally rare case of isolated intraventricular Rosai-Dorfman disease.
• The lesion was located in the region of the atria of the lateral ventricles.
• This presentation is distinct from typical dural or parenchymal CNS involvement.

Findings:

• The study describes a unique intraventricular mass consistent with Rosai-Dorfman disease.
• This finding expands the spectrum of CNS manifestations of RDD.
• Diagnostic challenges in differentiating from other intraventricular tumors are noted.

Implications:

• This case underscores the importance of considering RDD in the differential diagnosis of intraventricular masses.
• Further research may elucidate the pathogenesis of RDD within the CNS.
• Enhanced understanding aids in accurate diagnosis and management of rare neurological conditions.