Sinus histiocytosis with massive lymphadenopathy, or Rosai–Dorfman disease (RDD),
was first described in 1969 as a reactive condition of unknown etiology that is characterized
by a proliferation of histiocytes exhibiting emperipolesis of both lymphocytes and
plasma cells. It usually presents with painless cervical lymphadenopathy either with
or without extranodal manifestations. Intracranial involvement of this disease is
extremely rare. Intracranial RDD occurs in <5% of all patients with extranodal disease.
Here, we report a case of RDD with isolated intracranial involvement. A 67-year-old
male presented with a long-standing headache, retro-ocular pain, and progressive visual
loss of the left eye. Magnetic resonance imaging showed features of optic nerve meningioma.
The histopathology revealed sheets of histiocytes displaying emperipolesis. These
histiocytes were S100 positive; however, a CD1a and epithelial membrane antigen were
negative.
Key-words:
Intracranial tumor - optic nerve meningioma - Rosai–Dorfman disease - sinus histiocytosis
