Intracerebral multifocal Rosai-Dorfman disease.
We report a rare occurrence of intracranial Rosai-Dorfman disease (RDD), in a young patient with multiple bilateral intracerebral lesions, and discuss her management. RDD is an histiocytic proliferative disorder, which seldom presents with intracranial pathology. Intraparenchymal RDD without concurrent systemic features is rare. This 31-year old woman presented with two focal seizures, but no peripheral lymphadenopathy, and no significant systemic abnormalities. Preoperative imaging showed intrinsic space-occupying lesions in the left frontal lobe, and right parietal and right inferior frontal white matter. She underwent excision of the peripherally located left frontal lesion, and aside from a five-day course of postoperative dexamethasone, received no further active treatment. Histopathological analysis revealed the characteristic features of RDD. Postoperative imaging, taken at 10 weeks and 12 months, demonstrated resolution of all three lesions. This patient had a rare multifocal, bilateral, intracerebral RDD, illustrating that although a histological diagnosis is necessary, further treatment may not be required.
Department of Neurosurgery, Imperial College, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UK. scamp@doctors.org.uk
Sophie J
SJ
Camp
Federico
F
Roncaroli
Vasileios
V
Apostolopoulos
Mark
M
Weatherall
Siokping
S
Lim
Dipankar
D
Nandi
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001921:Brain / Q000473:pathology* / Q000601:surgery; D003907:Dexamethasone / Q000627:therapeutic use; D004828:Epilepsies, Partial / Q000209:etiology; D005260:Female; D015618:Histiocytosis, Sinus / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D008206:Lymphatic Diseases / Q000473:pathology; D008279:Magnetic Resonance Imaging; D009460:Neurologic Examination; D019635:Neurosurgical Procedures
D000893:Anti-Inflammatory Agents; D003907:Dexamethasone