Surgical treatment of intracranial Erdheim-Chester disease

Abstract

We review the clinical presentation, radiological and histological characteristics, and the natural history, of intracranial Erdheim-Chester disease (ECD). ECD is a rare form of non-Langerhans histiocytosis that affects multiple organs. It is clinically characterized by leg pain, exophthalmos and diabetes insipidus (DI). Central nervous system involvement is rare, with only 27 patients reported in the international literature. DI and cerebellar signs represent the most common neurological symptoms. Its treatment is controversial. Intracranial surgical procedures for ECD have been reported in 11 patients with a complete surgical resection performed in six, and an intracerebral biopsy performed in five patients. In seven patients the cranial procedures represented the initial diagnostic method. Surgical resection and radiation therapy have been used in the further management of these cerebral lesions.