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Rosai-Dorfman disease (RDD) involving the central nervous system (CNS) is rare and observed in 5% of all patients with extranodal RDD. According to a previous report, gross total resection is curative; however, we encountered a case of recurrence following gross total resection. We discuss our case and review previous reports on recurrent RDD. A 68-year-old woman came to the hospital complaining of left parietal mass. A tumor that had partially eroded the frontal bone was found. As the lesion was suspected to be malignant, we performed a total resection. Pathology results were indicative of an RDD. We did not prescribe adjuvant therapy because total resection was performed. However, after a year, abnormal accumulation in the left parietal bone was observed on FDG-PET. This was considered as recurrence, and re-excision was performed. Pathological assessments confirmed the recurrence of RDD. Our case demonstrated the recurrence of RDD following total resection. Future reports should assess these peculiarities. This will facilitate discussions on the risk factors and the effectiveness of treatment methods.

Early recurrence of Rosai-Doefman disease after total removal resection: a case report