Rosai-Dorfman Disease in the Skull Base: A Case Series Study

Rosai-Dorfman disease (RDD) is a rare, idiopathic, and non-neoplastic histio-proliferative disease that is uncommon in the central nervous system. Hence, reports of management of RDD in the skull base are scarce and only a few studies on skull base RDD are available. The objective of this study was to analyze the diagnosis, treatment, and prognosis of RDD in the skull base and explore an appropriate treatment strategy thereof.

Nine patients with clinical characteristics and follow-up data from our department between 2017 and 2022 were included in this study. From this information, the clinical profiles, imaging, treatment, and prognosis data were collected.

There were 6 male and 3 female patients with skull base RDD. These patients ranged in age from 13 to 61 years, with a median age of 41 years. The locations included 1 anterior skull base orbital apex, 1 parasellar region, 2 sellar regions, 1 petroclivus, and 4 foramen magnum regions. Six patients underwent total resection and 3 underwent subtotal resection. Patient follow-up lasted 11-65 months, with a median duration of 24 months. One patient died, 2 experienced recurrence, and the other patients' lesions were stable. The symptoms worsened and new complications occurred in 5 patients.

Skull base RDDs are intractable diseases with a high rate of complications. Some patients are at risk of recurrence and death. Surgery may be the basic treatment for this disease, and combined therapy including targeted therapy or radiation therapy may also be a valuable therapeutic strategy.