Rosai-Dorfman Disease

FACTS

  • connective tissue disorder with sinus histiocytosis
  • massive painless lymphadenopathy
  • path: dense fibro-collagenous CT with spindle cells and lymphocytic infiltration: S100/CD68
  • part of a group of pertinent histiocytic tumors: Langerhans cell histiocytosis, Erdheim-Chester disease, juvenile xanthogranuloma, histiocytic sarcoma

HPI

universal ROS
  • hx autoimmune disease
  • constitutional symptoms: fever, night sweats, weight loss

Pertinent work-up so far:
- ESR/CRP
- Syphillis, TB, HIV
- APLS, cardiolipin, LAC
- any other lesions / biopsies undertaken yet

PHYSICAL EXAM

universal neuro exam
  • evaluate for massive painless lymphadenopathy
  • check vision

IMAGING

can look deceptively similar to meningiomas: enhancing dural-based masses sometimes with a dural tail. most common locations:
  • convexities
  • parasagittal
  • supra-sellar
  • cavernous sinus
A/P
  • Neurosurgery role is for a biopsy to confirm diagnosis if needed.
  • Neuro-oncology consulatation
    • cobimetinib (PO kinase inhibitor) has been employed.
    • per Greenberg: low-dose XRT usually best treatment, surgery and immunosuppression NOT effective.
 
middle aged female with hx autoimmune comorbidities who presented with headaches, vision changes (found to have anterior uveitis) found to have 5 total dural-based lesions on MRI T1+c.
middle aged female with hx autoimmune comorbidities who presented with headaches, vision changes (found to have anterior uveitis) found to have 5 total dural-based lesions on MRI T1+c.