Spinal Rosai-Dorfman disease: case report and literature review

Eur Spine J. 2017 May;26(Suppl 1):117-127. doi: 10.1007/s00586-017-4975-0. Epub 2017 Feb 6.


Objective: Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a rare benign disease of dubious etiology that arises predominantly in lymph nodes with generalized fever and malaise. Isolated intraspinal involvement has its unique characteristics. The purpose of this study is to present the largest series of cases in the spinal Rosai-Dorfman disease literature to increase familiarity with its clinicopathologic features, diagnosis, and treatment of RDD from spine.

Methods: We present the case of a 34-year-old man who presented with paraplegia secondary to an isolated thoracic vertebral lesion. On physical exam, the patient displayed progressive weakness and tendon hyperreflexia of the lower limbs. After a totally section, symptoms of the patient were obviously relieved and the patient remained asymptomatic and no signs of recurrences were observed after follow-up for 5 months. We also retrospectively analyzed 60 cases of patients with spinal RDD published in English since 1969. Clinical date, histopathology, and radiological feature were retrospectively analyzed.

Results: Spinal RDD should no longer be considered rare and it may occupy an increasingly prominent place in the list of differential diagnoses for intraspinal lesions. Only elaborate histopathology was diagnostic for RDD. Most of the patients were surgically treated and marked improvements were observed in their clinical conditions.

Conclusions: RDD with spinal involvement is uncommon and it is challengeable in making a certain diagnosis. Histopathologic characteristics and immunohistochemical findings are considered as the key points for the diagnosis of this disease. The optimal treatment remains controversial, and more efforts should be focused on the investigation of etiology and adjuvant therapy for relapsing cases or subresected lesions.

Keywords: Rosai–Dorfman disease; Sinus histiocytosis with massive lymphadenopathy; Spinal involvement.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Diagnosis, Differential
  • Histiocytosis, Sinus / complications
  • Histiocytosis, Sinus / diagnostic imaging*
  • Histiocytosis, Sinus / surgery
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Paraplegia / etiology
  • Recurrence
  • Reflex, Abnormal
  • Retrospective Studies
  • Spinal Cord Compression / diagnostic imaging*
  • Spinal Cord Compression / etiology
  • Spinal Cord Compression / surgery
  • Spinal Diseases / diagnostic imaging*
  • Spinal Diseases / surgery
  • Thoracic Vertebrae / diagnostic imaging*
  • Thoracic Vertebrae / surgery