title: Surgical Management of Skull Base Rosai-Dorfman Disease.
abstract: BACKGROUND
  Rosai-Dorfman disease is a rare benign histiocytic proliferative disorder with a self-limiting clinical course. Skull base Rosai-Dorfman disease presents with intracranial lesions that often mimic meningiomas and other benign skull base tumors. The disease is difficult to diagnose radiographically, and tissue diagnosis exposes patients to significant perioperative risk. Surgical resection may require a large skull base exposure that risks significant surgical morbidity. Aggressive surgical resection, although often attempted, is of unproven efficacy. Our objective was to determine the optimal surgical management of skull base Rosai-Dorfman disease.
  
  
  METHODS
  We present 2 cases of skull base Rosai-Dorfman disease: a 26-year-old man with a middle fossa tumor and a 15-year-old teenage girl with a hypothalamic tumor. In addition, we reviewed 39 cases of skull base Rosai-Dorfman disease reported in the literature.
  
  
  CONCLUSIONS
  Tumors commonly occur in the sellar/parasellar region and result in loss of vision. Regardless of extent of resection, the majority of patients (>78%) have subsequent tumor regression or stable disease. Steroids and/or radiation are effective treatments for tumor recurrence. Tumor biopsy followed by observation, steroids, and/or radiation may be the most appropriate surgical management of skull base Rosai-Dorfman disease.
authors: Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan, USA.
authors: Todd
authors: T
authors: Hollon
authors: Department of Pathology, University of Michigan, Ann Arbor, Michigan, USA.
authors: Sandra I
authors: SI
authors: Camelo-Piragua
authors: Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan, USA.
authors: Erin L
authors: EL
authors: McKean
authors: Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan, USA.
authors: Stephen E
authors: SE
authors: Sullivan
authors: Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan, USA. Electronic address: hgarton@med.umich.edu.
authors: Hugh J L
authors: HJ
authors: Garton
mesh_terms: D000293:Adolescent; D000328:Adult; D000818:Animals; D001706:Biopsy; D035262:Cranial Fossa, Anterior / Q000473:pathology / Q000601:surgery; D015618:Histiocytosis, Sinus / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D020249:Hormone Replacement Therapy; D006801:Humans; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods*; D010027:Osteotomy; D011183:Postoperative Complications / Q000188:drug therapy; D011817:Rabbits; D019291:Skull Base / Q000601:surgery*; D016896:Treatment Outcome; D015050:Zygoma / Q000601:surgery
keywords: Rosai–Dorfman disease; Sellar/parasellar tumor; Skull base tumor; Vision loss