Long-term follow-up in two cases of intracranial Rosai–Dorfman Disease complicated by incomplete resection and recurrence
A 22-year-old male presented with progressive headache, focal convulsions, and monoparesis of his right arm and loss of vision of the right eye due to papillary congestion. In addition, he had noted a left supraclavicular swelling. Imaging showed a large extracranial space-occupying dura-based mass []. Lymph node and intracranial biopsy findings were compatible with RDD showing the emperipolesis phenomenon [] and a positive S-100 reaction. On operation, the tumor could be removed completely. Nineteen months later, a right submaxillary lymph node was extirpated confirming the diagnosis. After further 10 months, a left temporo-basal and a parietal tumor and after another year, a left retrobulbar lesion appeared and was operated as well. Because of this relapsing course, the patient received chemotherapy consisting out of 8 cycles of cyclophosphamide (1 g), vincristine (2 mg), doxorubicin (50 mg), and prednisone (50 mg) for 5 days every 3 weeks according to a modified CHOP regimen.[] Since then, no recurrences have been noted over a follow-up period of 7 years.