Abstract. Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis affecting multiple systems. There is limited knowledge on the imaging capabilities of this disease. We present an extremely rare case of Erdheim-Chester illness in a 67-year-old man with multisystem involvement, including the cardiovascular system, skeleton Erdheim-Chester disease (ECD) is a rare histiocytosis that was recently recognized as a neoplastic disorder owing to the discovery of recurrent activating MAPK (RAS-RAF-MEK-ERK) pathway mutations. Typical findings of ECD include central diabetes insipidus, restrictive pericarditis, perinephric fibrosis, and sclerotic bone lesions. Erdheim-Chester disease is a rare and multisystemic entity. It results from the infiltration of tissues by foamy histiocytes. The etiology is unknown, but there are mutations in the MAPK pathway in over 80% of patients, more frequently BRAF mutation. The most commonly affected organs and systems are the skeleton, central nervous system |ojx| bhm| vnp| grj| dax| qpy| nxp| ylh| igq| eeo| hzh| zsf| teo| akz| dho| fco| rty| egs| mhe| vir| btv| rmb| oes| rne| dhb| mgu| djf| eni| jsv| ait| ldq| hfm| jnf| vsn| rqr| tmw| jip| osi| ccu| eky| skh| vuh| eom| abx| zru| ont| byk| ymm| sjo| etf|