Erdheim-Chester Disease.

Autor: Benson JC; From the Departments of Radiology (J.C.B., I.T.M.) benson.john3@mayo.edu., Vaubel R; Laboratory Medicine and Pathology (R.V., B.A.E., C.G.)., Ebne BA; Laboratory Medicine and Pathology (R.V., B.A.E., C.G.)., Mark IT; From the Departments of Radiology (J.C.B., I.T.M.)., Peris Celda M; Neurosurgery (M.P.C.)., Hook CC; Hematology and Oncology (C.C.H.)., Tobin WO; Neurology (W.O.T.), Mayo Clinic, Rochester, Minnesota., Giannini C; Laboratory Medicine and Pathology (R.V., B.A.E., C.G.).
Jazyk: angličtina
Zdroj: AJNR. American journal of neuroradiology [AJNR Am J Neuroradiol] 2023 May; Vol. 44 (5), pp. 505-510. Date of Electronic Publication: 2023 Mar 30.
DOI: 10.3174/ajnr.A7832
Abstrakt: Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis. The disease is widely variable in its severity, ranging from incidental findings in asymptomatic patients to a fatal multisystem illness. CNS involvement occurs in up to one-half of patients, most often leading to diabetes insipidus and cerebellar dysfunction. Imaging findings in neurologic Erdheim-Chester disease are often nonspecific, and the disease is commonly mistaken for close mimickers. Nevertheless, there are many imaging manifestations of Erdheim-Chester disease that are highly suggestive of the disease, which an astute radiologist could use to accurately indicate this diagnosis. This article discusses the imaging appearance, histologic features, clinical manifestations, and management of Erdheim-Chester disease.
(© 2023 by American Journal of Neuroradiology.)
Databáze: MEDLINE