Surgical Management of Skull Base Rosai-Dorfman Disease

Abstract

Background and importance: 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.

Case description: 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.

Keywords: Rosai–Dorfman disease; Sellar/parasellar tumor; Skull base tumor; Vision loss.