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 pathological entity due to sinus histiocytosis with massive cervical lymphadenopathy (SHML). Isolated spinal involvement is an infrequent presentation of extranodal RDD. The clinical and radiological appearance of RDD represents a diagnostic challenge. We aimed to present two patients with paraparesis due to RDD of the thoracic spine and a PRISMA-style systematic review. Case Description There were two cases of isolated extra nodal 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 case had extradural thoracic spine RDD, which was resected completely. A 6-month postoperative follow-up magnetic resonance imaging (MRI) scan revealed local recurrence, which responded to radiation therapy. A 5-year follow-up of both patients revealed normal neurological functions and no recurrence on MRI scan surveillance. Conclusions RDD is a rare occurrence and should be considered in the differential diagnosis of extra- 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.