Imagerie des localisations osseuses et extra-osseuses de la maladie d'Erdheim-Chester

O. Adib; E. Baroth; L. Perard; J. Y. Scoazec; L. Vervueren; C. Aubé; S. Willoteaux

doi:10.1016/j.jradio.2011.04.013

Imagerie des localisations osseuses et extra-osseuses de la maladie d'Erdheim-Chester

Translated title of the contribution: Imaging features of osseous and extra-osseous involvement in Erdheim-Chester disease

O. Adib, E. Baroth, L. Perard, J. Y. Scoazec, L. Vervueren, C. Aubé, S. Willoteaux

Research output: Contribution to journal › Review article › peer-review

1 Citation (Scopus)

Abstract

Erdheim-Chester disease is a rare form of systemic non-Langerhans cell histiocytosis characterized by infiltration by lipid-laden or foamy histiocytes. Osseous involvement, major diagnostic criteria, is constant and characteristic. It presents as metaphyseal and diaphyseal osteosclerosis, mainly affecting the long bones of the lower limbs. A few cases with axial skeleton involvement have been reported. Extra-osseous lesions may affect the retroperitoneum, lungs, skin, heart, brain and orbits. Prognosis depends mainly on the extra-osseous disease, mainly heart and lung involvement. Diagnosis is based on the combination of radiographic features, nuclear medicine features and nearly pathognomonic immunohistochemical profile.

Translated title of the contribution	Imaging features of osseous and extra-osseous involvement in Erdheim-Chester disease
Original language	French
Pages (from-to)	671-680
Number of pages	10
Journal	Journal de Radiologie
Volume	92
Issue number	7-8
DOIs	https://doi.org/10.1016/j.jradio.2011.04.013
Publication status	Published - 1 Jul 2011
Externally published	Yes

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10.1016/j.jradio.2011.04.013

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title = "Imagerie des localisations osseuses et extra-osseuses de la maladie d'Erdheim-Chester",

abstract = "Erdheim-Chester disease is a rare form of systemic non-Langerhans cell histiocytosis characterized by infiltration by lipid-laden or foamy histiocytes. Osseous involvement, major diagnostic criteria, is constant and characteristic. It presents as metaphyseal and diaphyseal osteosclerosis, mainly affecting the long bones of the lower limbs. A few cases with axial skeleton involvement have been reported. Extra-osseous lesions may affect the retroperitoneum, lungs, skin, heart, brain and orbits. Prognosis depends mainly on the extra-osseous disease, mainly heart and lung involvement. Diagnosis is based on the combination of radiographic features, nuclear medicine features and nearly pathognomonic immunohistochemical profile.",

keywords = "Erdheim-Chester, Imaging, Interferon-α, Non-Langerhans cell histiocytosis, Scintigraphy",

author = "O. Adib and E. Baroth and L. Perard and Scoazec, {J. Y.} and L. Vervueren and C. Aub{\'e} and S. Willoteaux",

year = "2011",

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doi = "10.1016/j.jradio.2011.04.013",

language = "Fran{\c c}ais",

volume = "92",

pages = "671--680",

journal = "Journal de Radiologie",

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T1 - Imagerie des localisations osseuses et extra-osseuses de la maladie d'Erdheim-Chester

AU - Adib, O.

AU - Baroth, E.

AU - Perard, L.

AU - Scoazec, J. Y.

AU - Vervueren, L.

AU - Aubé, C.

AU - Willoteaux, S.

PY - 2011/7/1

Y1 - 2011/7/1

N2 - Erdheim-Chester disease is a rare form of systemic non-Langerhans cell histiocytosis characterized by infiltration by lipid-laden or foamy histiocytes. Osseous involvement, major diagnostic criteria, is constant and characteristic. It presents as metaphyseal and diaphyseal osteosclerosis, mainly affecting the long bones of the lower limbs. A few cases with axial skeleton involvement have been reported. Extra-osseous lesions may affect the retroperitoneum, lungs, skin, heart, brain and orbits. Prognosis depends mainly on the extra-osseous disease, mainly heart and lung involvement. Diagnosis is based on the combination of radiographic features, nuclear medicine features and nearly pathognomonic immunohistochemical profile.

AB - Erdheim-Chester disease is a rare form of systemic non-Langerhans cell histiocytosis characterized by infiltration by lipid-laden or foamy histiocytes. Osseous involvement, major diagnostic criteria, is constant and characteristic. It presents as metaphyseal and diaphyseal osteosclerosis, mainly affecting the long bones of the lower limbs. A few cases with axial skeleton involvement have been reported. Extra-osseous lesions may affect the retroperitoneum, lungs, skin, heart, brain and orbits. Prognosis depends mainly on the extra-osseous disease, mainly heart and lung involvement. Diagnosis is based on the combination of radiographic features, nuclear medicine features and nearly pathognomonic immunohistochemical profile.

KW - Erdheim-Chester

KW - Imaging

KW - Interferon-α

KW - Non-Langerhans cell histiocytosis

KW - Scintigraphy

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U2 - 10.1016/j.jradio.2011.04.013

DO - 10.1016/j.jradio.2011.04.013

M3 - Article 'review'

C2 - 21819909

AN - SCOPUS:80052023658

SN - 0221-0363

VL - 92

SP - 671

EP - 680

JO - Journal de Radiologie

JF - Journal de Radiologie

IS - 7-8

ER -

Imagerie des localisations osseuses et extra-osseuses de la maladie d'Erdheim-Chester

Abstract

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