Rosai-Dorfman

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Definition

Rosai-Dorfman disease (RDD) is a systemic histioproliferative disorder with massive lymphadenopathy. Central nervous system involvement is rare. The etiology of this disease is unknown, with some evidence supporting dysfunction of the immune system or an autoimmune disorder.

Epidemiology

First described in 1969, there have been over 1000 cases reported since then. Patients most often present with massive, painless lymphadenopathy involving the cervical region. Only about 50 cases have been reported involving the central nervous system. In 25-40% of cases, RDD may occur in the skin, orbits, respiratory tract, bones, and CNS.

Imaging Studies

RDD lesions appear as dural based lesions on T1 weighted MRI with contrast. As such, they may mimic meningioma, Langerhans cell histiocytosis, sarcoidosis, infectious processes (e.g. tuberculoma), lymphoproliferative disorders, plasma cell granuloma, and inflammatory pseudotumor.

Pathology

The key features in RDD are S100 protein-positive histiocytes that are polyclonal in nature. Microscopically, a mixture of lymphocytes and large histioyctes will be seen with some histiocytes having engulfed other lymphocytes (emperipolesis - a hallmark of RDD).

Treatment

Treatment of RDD has traditionally been surgical. However, more recent literature has supported the use of radiosurgery with long term (>1 year) local control. Glucocorticoid (Prednisone) administration has also been used (sometimes in combination with cytotoxic therapy for orbital involvement) with long term results as well (~11 months).

References

  1. J Neurosurg. 2006 May;104(5):840-4.
  2. Childs Nerv Syst. 2006 Sep;22(9):1194-200.
  3. J Neurosurg. 2003 Jan;98(1):165-8.
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