Xanthomatous Inflammatory Infiltrate Involving the Spleen: An Unusual Presentation of Erdheim-Chester Disease and Review of the Literature

[b]BACKGROUND[/b] Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by foamy histiocytes, Touton-like giant cells, and fibrosis, typically affecting the diaphyseal metaphyseal region long bones but that can involve any organ or tissue. ECD usually associated with BRAF V600E mutation other molecular mutations inserted in MAPK cascade. [b]CASE REPORT[/b] We present case 63-year-old man previous history myocardial infarction who underwent an emergency splenectomy for splenic rupture after accidental fall. Histological examination spleen showed diffuse xanthogranulomatous proliferation (CD68+, CD163+, S100-, CD1a-) cells red pulp. Based on histologic findings, diagnosis was made. However, skeletal involvement were not detected. [b]CONCLUSIONS[/b] Cases are histologically consistent unusual sites have been increasingly described. There also anecdotal evidence cases being besides no genetic alteration involvement. Likewise, spectrum clinical features be broader than previously considered. Furthermore, there various phases show different presentations distinct prognostic impact, according to mutational spectrum. Recognizing at early stage allows more effective patient management, pathologists clinicians should aware this condition.