Nonresectable Thoracic Rosai-Dorfman Disease: A Case Report and Review of the Literature

World Neurosurg. 2019 Dec:132:309-313. doi: 10.1016/j.wneu.2019.09.034. Epub 2019 Sep 13.

Abstract

Introduction: Rosai-Dorfman disease (RDD) is a rare disease that can be triggered by either viral or bacterial infection. Several parts of the body can be involved, from the central nervous system to the pelvic regions had been reported. At present, there is a serious lack of guidelines as to how to treat cases of RDD involving the spine. Current trends show that surgery remains the first method of choice to cure this disease, but in refractory or recurrent RDD, repeat surgery cannot guarantee total resection. Under such circumstances, adjuvant therapy can be very useful. Here, we share our experience of treating recurrent spinal RDD.

Case description: Our patient was a 32-year-old male patient with hepatitis B virus infection. He suffered from lower back pain with lower limb weakness after being hit by a metal pipe. Laminectomy of T11-12 was performed to decompress the spinal cord. The final pathological results demonstrated chronic inflammation. After surgery, the lower limbs improved and the patient recovered quite well. However, he returned 3 years later with the same complaints and was diagnosed with RDD. Durectomy was performed and repaired with an artificial dura. Thalidomide therapy was initiated. Currently, 9 years later, the patient has not experienced recurrence or any form of neurological deficit.

Conclusions: RDD is a rare disease and can be misdiagnosed easily. Although it may resolve spontaneously, recurrence is possible. Hence, extensive follow-up is necessary. Surgery remained the first choice of surgery, however, when encountered recurrent or nonresectable RDD, adjuvant therapy such as corticosteroids, thalidomide, and radiotherapy could help. In this article, we shared our experience using thalidomide in treating nonresectable RDD.

Keywords: Adjuvant therapy; Recurrent; Rosai-Dorfman disease.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Histiocytosis, Sinus / complications
  • Histiocytosis, Sinus / diagnostic imaging
  • Histiocytosis, Sinus / pathology
  • Histiocytosis, Sinus / therapy*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Low Back Pain / etiology
  • Lower Extremity
  • Male
  • Muscle Weakness / etiology
  • Recurrence
  • Spinal Diseases / complications
  • Spinal Diseases / diagnostic imaging
  • Spinal Diseases / pathology
  • Spinal Diseases / therapy*
  • Thalidomide / therapeutic use*
  • Thoracic Vertebrae / surgery*

Substances

  • Immunosuppressive Agents
  • Thalidomide