Erdheim-Chester Disease Mimicking a Precentral Meningioma: A Case of Rapid Parenchymal Recurrence and Multisystemic Progression

Abstract: Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis that remains a significant diagnostic challenge, particularly when it presents with dural involvement. We describe the clinical course of a 50-year-old male, originally treated for a presumed precentral meningioma based on a dural mass showing intense enhancement and an atypical T2-hypointense signal. Following surgical resection, the patient experienced a multisystemic flare (thoracic, hepatic, and adrenal) alongside a rapid multifocal parenchymal recurrence in the brain. Biopsy of the thoracic lesion confirmed a BRAF V600E-mutated ECD. This report emphasizes that a “black” T2 signal in a dural mass is a major clinical red flag. Radiologists should systematically correlate such findings with the patient’s cardiac history to distinguish ECD from more common dural tumors and avoid unnecessary surgery.