Long-Term Outcome of Spinal Extranodal Rosai-Dorfman Disease: A Report of Two Cases and Systematic Review

Abstract

Background: Rosai-Dorfman disease (RDD) is a rare pathologic entity caused by sinus histiocytosis with massive cervical lymphadenopathy. Isolated spinal involvement is an infrequent presentation of extranodal RDD. The clinical and radiologic appearance of RDD represents a diagnostic challenge. We report 2 patients with paraparesis caused by RDD of the thoracic spine and a PRISMA-style systematic review. Case Description: There were 2 patients with isolated extranodal thoracic spinal RDD without cervical lymphadenopathy. One patient presented with anterior thoracic RDD and a subtotal resection. The small residual disease completely responded to the postoperative course of steroids. The second patient had extradural thoracic spine RDD, which was resected completely. A 6-month postoperative follow-up magnetic resonance imaging (MRI) scan showed local recurrence, which responded to radiation therapy. Five years follow-up of both patients showed normal neurologic functions and no recurrence on MRI scan surveillance. Conclusions: RDD is a rare occurrence and should be considered in the differential diagnosis of extradural or intradural spinal lesions. Gross total resection is recommended, and long-term clinical follow-up with MRI is advised. Residual or recurrent RDD requires steroids or radiation therapy.