Lesão granulomatosa intracraniana mimetizando meningioma: Relato de um caso da doença de Erdheim-Chester / Granulomatous lesion mimicking intracranial meningioma: Erdheim-Chester Disease case report

RESUMO

Erdheim-Chester disease (ECD) is a rare non-Langerhans cells histiocytosis witch affects multiple organs with variable clinicalpresentation. It has a challenging diagnosis most often done after extesnsive investigation. There are no pathognomonicradiological or histological patterns, that is why the diagnosis is based on clinical suspect when all findings areput together andthe immunohistochemistry differentiates from others histiocytosis.The central nervous system involvement is even more rare and the presentation may vary from nodular lesion, white matterinfiltration, to expansive mass effect tumors that may need surgical intervention.In this article we present a case report of ECD and discuss diagnostic and terapeutic options.