Erdheim–Chester disease: A systematic review

Highlights • CNS involvement occurs in 56% of ECD patients. • Concomitant pituitary involvement, retro-orbital and axial lesions suggest ECD diagnosis. • Kidney infiltration is more frequent in patients with CNS involvement.

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Bibliographic Details
Published in:Critical reviews in oncology/hematology 2015-07, Vol.95 (1), p.1-11
Main Authors: Cives, Mauro, Simone, Valeria, Rizzo, Francesca Maria, Dicuonzo, Franca, Cristallo Lacalamita, Marirosa, Ingravallo, Giuseppe, Silvestris, Franco, Dammacco, Franco
Format: Article
Language:English
Subjects:
Adrenal Cortex Hormones - therapeutic use
Adult
Bone and Bones - pathology
BRAFV600E
Central Nervous System - pathology
CNS involvement
Erdheim-Chester Disease - diagnosis
Erdheim-Chester Disease - drug therapy
Erdheim-Chester Disease - genetics
Erdheim-Chester Disease - pathology
Erdheim–Chester disease
Hematology, Oncology and Palliative Medicine
Histiocytosis
Humans
Kidney - pathology
Male
Myocardium - pathology
Point Mutation
Prognosis
Proto-Oncogene Proteins B-raf - genetics
Vemurafenib
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Description
Summary:Highlights • CNS involvement occurs in 56% of ECD patients. • Concomitant pituitary involvement, retro-orbital and axial lesions suggest ECD diagnosis. • Kidney infiltration is more frequent in patients with CNS involvement.
ISSN:1040-8428
1879-0461
DOI:10.1016/j.critrevonc.2015.02.004