Spinal extranodal Rosai-Dorfman disease: A case report and literature review.

Abstract

IntroductionSpinal extranodal Rosai-Dorfman disease (RDD) is extremely rare. In this paper, we reported successful management of spinal extranodal RDD and reviewed medical literature.Case presentationA 19-year-old male presented with progressive bilateral leg weakness and back pain for two months before admission. He denied weight loss, fever, night sweats, and lymph node enlargement. On examination, his muscle strength of both legs was grade I with hyperreflexia. Magnetic resonance imaging of the spine (MRI) showed a thoracic extradural mass at a level of T6-T9, which was a heterogeneous hyperintense on T2W, STIR, and isointense on T1W and enhanced contrast vividly. We resected the tumor totally and decompressed the spinal cord. Pathology revealed a histiocytic tumor. Immunohistochemical staining was S100 (+), CD68 (+), CD45 (+), and CD1a (−). Postoperatively, his muscle strength improved gradually to grade IV after four months. Postoperative MRI of the spine showed no residual tumor. No further adjuvant therapy was indicated.Clinical discussionSpinal extranodal RDD has no specific symptoms and pathognomonic imaging features. CT and MRI of the spine are still the essential tools for diagnosing RDD, but biopsy is often mandatory for definitive diagnosis. There have not been consensus guidelines for treating RDD of the spine because of its rarity. Surgical resection remained the mainstay of treatment (78.8%), with or without adjuvant therapies.ConclusionSurgery is the treatment of choice for most cases, while steroid therapy, radiotherapy, and chemotherapy should be adjuvant treatment and tailored individually.