AJR 2004; 183:1253-1260
© American Roentgen Ray Society
Imaging of Thoracoabdominal Involvement in Erdheim-Chester Disease
Elisabeth Dion1,
Claire Graef1,
Julien Haroche2,
Raphaële Renard-Penna1,
Philippe Cluzel1,
Bertrand Wechsler2,
Jean-Charles Piette2 and
Philippe A. Grenier1
1 Department of Radiology, Hôpital Pitié-Salpêtrière,
Assistance Publique-Hôpitaux de Paris-Université
Pierre-et-Marie-Curie, 47-83 Boulevard de l'Hôpital, 75651 Paris Cedex
13, France.
2 Department of Internal Medicine, Hôpital
Pitié-Salpêtrière, Assistance Publique-Hôpitaux de
Paris-Université Pierre-et-Marie-Curie, 75651 Paris Cedex 13,
France.
Received February 26, 2004;
accepted after revision April 21, 2004.
Address correspondence to E. Dion.
Introduction
Erdheim-Chester disease is a rare form of non-Langerhans cell
histiocytosis of unknown origin, belonging to the category of acquired
overload histiocytosis. Erdheim-Chester disease is characterized by tissue
infiltration by foamy histiocytes (xanthogranulomatosis) with
immunohistochemical characteristics different from those of Langerhans cell
histiocytosis. Immunohistochemical stains are constantly positive for CD68 and
negative for CD1a, and ultrastructural studies show Birbeck granules in less
than 20% of histiocytes. Staining of all histiocytic cells is negative or very
weakly positive for S-100 protein.
Erdheim-Chester disease most often occurs after the age of 40 years with a
slight male predominance. Pediatric cases are extremely rare. Bone involvement
consists of symmetric osteosclerosis of the long tubular bones, predominating
in the metaphysis and diaphysis of the lower limbs. It is the most frequent
abnormality present radiologically in almost all patients reported in the
literature and is asymptomatic in at least 60% of cases
[1]. Moreover, Erdheim-Chester
disease is clinically polymorphous and can represent a true systemic disease
with potentially life-threatening organ and, in most cases, extraosseous
involvement [1].
Pathologically, Erdheim-Chester disease is diffuse and infiltrative; can
affect many organs; and shows a tropism for connective, adipose, and
perivascular tissue. This involvement can extend throughout the length of the
aorta (up to the heart) and invade the retroperitoneum and mediastinum, with
potentially life-threatening complications, such as heart failure, tamponade,
and renal failure. The involvement of the liver, pancreas, and mesentery is
extremely rare.
This pictorial essay presents the clinical and imaging features
characterizing retroperitoneal, mediastinal, cardiovascular, and lung
involvement in Erdheim-Chester disease.
Renal and Perirenal Involvement
Renal and perirenal involvement is relatively frequent, found in 29%
(17/59) of patients in the series reported by Veyssier-Belot et al.
[1] and may be seen as an
isolated location of the disease
[2]. This involvement is
usually asymptomatic and is revealed on CT, which classically shows
hypoattenuated homogeneous tissue infiltration with weak contrast enhancement
in the renal fossae (Figs. 1A,
1B). The perirenal infiltration
may extend to the fat of the anterior or posterior pararenal spaces or both.
The "hairy kidney" appearance, due to symmetric and bilateral
infiltration of both the perirenal and posterior pararenal space, is highly
suggestive of the diagnosis (Figs.
2A and
2B). Extension to the renal
sinuses and pedicles and also to the proximal ureters and lumbar ureters is
possible and may cause upper urinary tract obstruction (Figs.
2D,
2E, and
3). On MRI, the soft-tissue
infiltration of the pararenal and perirenal fat appears isointense to muscle
on T1- and T2-weighted spin-echo sequences, with a slight and homogeneous
enhancement in signal intensity after gadolinium injection
[3].
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Fig. 1A. —45-year-old man with Erdheim-Chester disease with
histologically proved perirenal involvement. Unenhanced axial CT scan reveals
bilateral homogeneous perirenal infiltration with attenuation similar to that
of muscle.
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Fig. 1B. —45-year-old man with Erdheim-Chester disease with
histologically proved perirenal involvement. Axial CT scan, obtained 5 min
after iodinated contrast injection at same level as A, shows
enhancement (arrows) of perirenal infiltration similar to that of
muscle.
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Fig. 2A. —56-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was obtained from perirenal biopsy. Axial (A)
and coronal (B) reformatted enhanced images reveal bilateral and
symmetric perirenal infiltration (arrows). Irregular bands, present
along posterior and inferior parts of left perirenal infiltration, have
"hairy" appearance. Note also circumferential sheathing of aorta
by perivascular infiltration, including proximal parts of renal arteries.
C, Digital angiogram shows stenoses of proximal parts of both right and
left renal arteries (arrows).
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Fig. 2B. —56-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was obtained from perirenal biopsy. Axial (A)
and coronal (B) reformatted enhanced images reveal bilateral and
symmetric perirenal infiltration (arrows). Irregular bands, present
along posterior and inferior parts of left perirenal infiltration, have
"hairy" appearance. Note also circumferential sheathing of aorta
by perivascular infiltration, including proximal parts of renal arteries.
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Fig. 2D. —56-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was obtained from perirenal biopsy. Abdominal MDCT
scans were obtained 4 weeks after treatment of renal hypertension by
angioplasty and stenting of right renal artery stenosis. Right (D) and
left (E) coronal reformatted maximum-intensity-projection images reveal
bilateral pelvocaliectasis due to obstruction of upper portion of ureters
(arrows). Note that bilateral perirenal infiltration is still
present.
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Fig. 2E. —56-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was obtained from perirenal biopsy. Abdominal MDCT
scans were obtained 4 weeks after treatment of renal hypertension by
angioplasty and stenting of right renal artery stenosis. Right (D) and
left (E) coronal reformatted maximum-intensity-projection images reveal
bilateral pelvocaliectasis due to obstruction of upper portion of ureters
(arrows). Note that bilateral perirenal infiltration is still
present.
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Fig. 3. —60-year-old man with exophthalmia and left leg pain.
Diagnosis of Erdheim-Chester disease was confirmed histologically by bone and
nasal polyp biopsies. Enhanced axial CT scan reveals bilateral asymptomatic
perirenal infiltration extending into and expanding sinus (thin
arrow) of left kidney. Note also homogeneous circumferential periaortic
infiltration (thick arrow).
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Adrenal Fossae
Perirenal infiltration may extend upward to the adrenal fossae
(Fig. 4). This adrenal gland
infiltration may be associated with adrenal hypertrophy, reflecting gland
infiltration by the fibrohistiocytic tissue
(Fig. 5).
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Fig. 4. —46-year-old man with Erdheim-Chester disease. Enhanced
abdominal CT scan shows extension of perirenal infiltration into adrenal
fossae and surrounding adrenal gland (arrows). Note left perirenal
infiltration extending into anterior pararenal space.
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Fig. 5. —54-year-old man with Erdheim-Chester disease proven
histologically by perirenal infiltration biopsy. Enhanced abdominal CT scan
shows bilateral infiltration of adrenal fossae (arrows). Enlarged
right adrenal gland is visible within periadrenal space infiltrate.
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Aorta
Vascular involvement by Erdheim-Chester disease is relatively uncommon,
with 17 cases described in the literature
[1,
4-6].
The frequency of vascular involvement is probably underestimated because it
can be asymptomatic. CT typically shows periaortic tissue infiltration,
extending from the ascending aorta to the iliac junction and creating the
appearance of a "coated aorta"
[4] (Figs.
6A,
6B,
6C,
6D,
6E). In some cases, the aortic
involvement may be limited to the abdominal or thoracic aorta
[4,
5]. Classically, the infiltrate
has the same attenuation as muscle on CT, with homogeneous and weak
enhancement after contrast injection. The infiltrate appears less intense than
fat on T1- and T2-weighted spin-echo images with slight enhancement after
gadolinium administration. The periaortic infiltration is usually
circumferential and regular in contour (Figs.
2A,
2B,
2C and
3). This infiltration seems to
be perivascular or, more precisely, periadventitial
[6], rather than parietal (as
in aortitis). On MRI, the aortic wall is distinguished from the
periadventitial infiltration by the higher signal intensity on the wall on
both T1- and T2-weighted sequences (Fig.
7). Abnormalities seen on CT scans and MR thoracoabdominal images
may suggest the differential diagnosis of retroperitoneal fibrosis. However,
several criteria seem to be characteristic of Erdheim-Chester disease: The
periaortic infiltration is circumferential instead of located at the anterior
and lateral aspects of the aorta, sparing the posterior aspect as seen in
retroperitoneal fibrosis. The inferior vena cava is spared, whereas it is
commonly involved with stenosis or occlusion in retroperitoneal fibrosis.
Bilateral infiltration of the perirenal fat that produces a hairy kidney
appearance is frequently associated with periaortic disease, whereas the
perirenal space is rarely involved in patients with retroperitoneal fibrosis.
The pelvic ureters are always spared in Erdheim-Chester disease and often
involved in retroperitoneal fibrosis.
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Fig. 6A. —54-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was confirmed by perirenal infiltration biopsy and
typical pattern of bone involvement. Contrast-enhanced CT scans of thorax show
presence of periaortic infiltration ("coated aorta") extending
from aortic arch to abdominal aorta. Periaortic sheathing extends upward to
proximal portion of supraaortic trunks (A) and inferiorly involves
proximal portion of intercostal arteries (arrow, C).
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Fig. 6B. —54-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was confirmed by perirenal infiltration biopsy and
typical pattern of bone involvement. Contrast-enhanced CT scans of thorax show
presence of periaortic infiltration ("coated aorta") extending
from aortic arch to abdominal aorta. Periaortic sheathing extends upward to
proximal portion of supraaortic trunks (A) and inferiorly involves
proximal portion of intercostal arteries (arrow, C).
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Fig. 6C. —54-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was confirmed by perirenal infiltration biopsy and
typical pattern of bone involvement. Contrast-enhanced CT scans of thorax show
presence of periaortic infiltration ("coated aorta") extending
from aortic arch to abdominal aorta. Periaortic sheathing extends upward to
proximal portion of supraaortic trunks (A) and inferiorly involves
proximal portion of intercostal arteries (arrow, C).
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Fig. 6D. —54-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was confirmed by perirenal infiltration biopsy and
typical pattern of bone involvement. Coronal reformatted CT images reveal
extent of infiltration around origin of supraaortic trunks (arrows)
and into mediastinal fat, particularly subaortic area (asterisk,
E).
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Fig. 6E. —54-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was confirmed by perirenal infiltration biopsy and
typical pattern of bone involvement. Coronal reformatted CT images reveal
extent of infiltration around origin of supraaortic trunks (arrows)
and into mediastinal fat, particularly subaortic area (asterisk,
E).
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Fig. 2C. —56-year-old man with renovascular hypertension. Diagnosis of
Erdheim-Chester disease was obtained from perirenal biopsy. Digital angiogram
shows stenoses of proximal parts of both right and left renal arteries
(arrows).
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Fig. 7. —56-year-old man with Erdheim-Chester disease associated with
bilateral perirenal involvement and circumferential infiltration of abdominal
aorta. Gadolinium-enhanced T1-weighted spin-echo abdominal image shows aortic
wall as hyperintense (arrows) compared with relatively hypointense
periaortic infiltrative tissue. As a result, higher signal intensity of aortic
inner wall is distinguished from periaortic infiltrate. Perirenal infiltration
and "hairy" appearance are seen as low-intensity perirenal rim and
posterior irregular lines.
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In addition, parietal aortic wall thickening can be encountered in
Takayasu's disease, Horton's disease, and infectious aortitis. These diseases
involve the entire wall, including adventitia, media, and intima, whereas
Erdheim-Chester disease involves the adventitial and periadventitial
periaortic space, sparing the wall itself.
Aortic Branches
Extension to the aortic collaterals, especially their ostial portion, is
frequent and can affect the supraaortic trunks, coronary arteries, and
abdominal branches (Figs. 2A,
2B,
2C,
2D,
2E and
6A,
6B,
6C,
6D,
6E,
7,
8A,
8B,
8C,
8D,
8E,
8F,
8G). Few ischemic
complications have been described, but arterial stenosis can occur
(Fig. 2C). Stenosis of the
renal arteries may cause renovascular hypertension (Figs.
2A,
2B,
2C,
2D,
2E), and stenosis of the
superior mesenteric artery may lead to abdominal angina. The infiltrative
process spreads from the aorta toward its collaterals, including the coronary
(Figs. 8A,
8B,
8C,
8D,
8E,
8F,
8G) and cerebral arteries.
Atypical vascular involvement consisting of an isolated nonostial infiltration
of an aortic collateral, without adjacent periaortic sheathing, occasionally
may occur (Fig. 9).
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Fig. 8A. —56-year-old woman with progressive chronic cardiac failure
who was followed up for 5 years. Erdheim-Chester disease diagnosis was made on
basis of typical bone involvement on femurs and perirenal biopsy.
Contrast-enhanced MDCT scans of chest show huge mediastinal infiltration
sheathing superior vena cava and descending and ascending aorta. Azygos vein
(arrow, A) is dilated and functioning as collateral because of
stenosis of superior vena cava. Mediastinal infiltration (asterisk,
B) involves walls of right atrium and distorts its lumen
(arrow, B). Bilateral pleural thickening and effusion are also
present.
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Fig. 8B. —56-year-old woman with progressive chronic cardiac failure
who was followed up for 5 years. Erdheim-Chester disease diagnosis was made on
basis of typical bone involvement on femurs and perirenal biopsy.
Contrast-enhanced MDCT scans of chest show huge mediastinal infiltration
sheathing superior vena cava and descending and ascending aorta. Azygos vein
(arrow, A) is dilated and functioning as collateral because of
stenosis of superior vena cava. Mediastinal infiltration (asterisk,
B) involves walls of right atrium and distorts its lumen
(arrow, B). Bilateral pleural thickening and effusion are also
present.
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Fig. 8C. —56-year-old woman with progressive chronic cardiac failure
who was followed up for 5 years. Erdheim-Chester disease diagnosis was made on
basis of typical bone involvement on femurs and perirenal biopsy. Coronal
(C) and axial (D) oblique reformations after cardiac-gated MDCT
acquisition show mediastinal infiltration, seen as sheathing ascending aorta
including its orgin and proximal portion of coronary arteries
(arrows).
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Fig. 8D. —56-year-old woman with progressive chronic cardiac failure
who was followed up for 5 years. Erdheim-Chester disease diagnosis was made on
basis of typical bone involvement on femurs and perirenal biopsy. Coronal
(C) and axial (D) oblique reformations after cardiac-gated MDCT
acquisition show mediastinal infiltration, seen as sheathing ascending aorta
including its orgin and proximal portion of coronary arteries
(arrows).
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Fig. 8E. —56-year-old woman with progressive chronic cardiac failure
who was followed up for 5 years. Erdheim-Chester disease diagnosis was made on
basis of typical bone involvement on femurs and perirenal biopsy.
Cardiac-gated T1-weighted spin-echo chest images reveal extent of mediastinal
infiltration sheathing aorta, superior vena cava, and right pulmonary artery.
Infiltration appears as soft-tissue of low intensity (E and F).
Pericardium is not visible because it is probably obscured by mediastinal
tissue infiltration. Right atrium is obscured by wall thickening and lumen
distortion (asterisk, G) associated with presence of
pseudomass appearance in anterior and lateral walls (black arrow,
G). Another pseudomass is also visible on interatrial septum (white
arrow, G). Note dilatation of left ventricle.
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Fig. 8F. —56-year-old woman with progressive chronic cardiac failure
who was followed up for 5 years. Erdheim-Chester disease diagnosis was made on
basis of typical bone involvement on femurs and perirenal biopsy.
Cardiac-gated T1-weighted spin-echo chest images reveal extent of mediastinal
infiltration sheathing aorta, superior vena cava, and right pulmonary artery.
Infiltration appears as soft-tissue of low intensity (E and F).
Pericardium is not visible because it is probably obscured by mediastinal
tissue infiltration. Right atrium is obscured by wall thickening and lumen
distortion (asterisk, G) associated with presence of
pseudomass appearance in anterior and lateral walls (black arrow,
G). Another pseudomass is also visible on interatrial septum (white
arrow, G). Note dilatation of left ventricle.
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Fig. 8G. —56-year-old woman with progressive chronic cardiac failure
who was followed up for 5 years. Erdheim-Chester disease diagnosis was made on
basis of typical bone involvement on femurs and perirenal biopsy.
Cardiac-gated T1-weighted spin-echo chest images reveal extent of mediastinal
infiltration sheathing aorta, superior vena cava, and right pulmonary artery.
Infiltration appears as soft-tissue of low intensity (E and F).
Pericardium is not visible because it is probably obscured by mediastinal
tissue infiltration. Right atrium is obscured by wall thickening and lumen
distortion (asterisk, G) associated with presence of
pseudomass appearance in anterior and lateral walls (black arrow,
G). Another pseudomass is also visible on interatrial septum (white
arrow, G). Note dilatation of left ventricle.
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Fig. 9. —52-year-old man with cardiac insufficiency. Erdheim-Chester
disease was confirmed by perirenal biopsy. Contrast-enhanced axial CT image
reveals distal portion of left renal artery (arrow) and superior
mesenteric artery (arrowhead) infiltrated and sheathed. By contrast,
aortic infiltration is visible only on anterior wall of vessels without
sheathing.
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Pulmonary Arteries and Superior Vena Cava
The abnormal periarterial infiltration of soft tissue seems to respect
venous structures, at least in the early stages of the disease. However, in
case of extensive mediastinal infiltration, the superior vena cava and the
pulmonary trunk and main arteries may be sheathed, with reduction of the
vascular lumen (Figs. 8A,
8B,
8C,
8D,
8E,
8F,
8G).
Heart and Pericardium
Cardiac involvement can be endocardial, myocardial, or pericardial.
Pericardial involvement is most frequent, being reported in 16 patients in the
literature [7]. It is revealed
by pericardial effusion with a risk of tamponade or by pericardial thickening.
Myocardial involvement is rare, appearing as a mass in the right atrium
[8]
(Fig. 8G) or thickening of the
left atrium [7]. Cardiac valve
involvement, either aortic or mitral, can also occur
[5].
Lung Parenchyma
Pulmonary involvement is relatively uncommon and was reported in 14% of
patients in the series by Veyssier-Belot et al.
[1]. It is characterized on
high-resolution CT by smooth symmetric interlobular thickening associated with
fissural thickening, multifocal areas of ground-glass attenuation, and small
centrilobular nodular opacities (Figs.
10A,
10B). Involvement is diffuse
or predominant in the upper or lower lobes. In addition, uni- or bilateral
pleural effusion may also be present (Figs.
8A,
8B,
8C,
8D,
8E,
8F,
8G). Although each of the CT
findings is nonspecific, the constellation is strongly suggestive of
Erdheim-Chester disease [9].
Pathologic correlation has shown that the visceral pleura, interlobular septa,
and bronchovascular bundles are expanded by a combination of inflammation and
fibrosis.
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Fig. 10A. —44-year-old man with Erdheim-Chester disease with bone
involvement and progressive dyspnea. High-resolution CT scan obtained at level
of lung apices shows bilateral smooth thickening of interlobular septa.
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Fig. 10B. —44-year-old man with Erdheim-Chester disease with bone
involvement and progressive dyspnea. High-resolution CT scan obtained at mid
portion of lungs shows bilateral thickening of fissures and patchy areas of
ground-glass attenuation and small centrilobular nodules.
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Conclusion
Symmetric and bilateral peri- and pararenal soft-tissue infiltration (hairy
kidney) extending to the adrenal glands and renal pedicles or circumferential
periaortic infiltration from the aortic arch to the primary iliac artery
origin (coated aorta) or both are suggestive of Erdheim-Chester disease. The
periaortic infiltration may extend to the ascending aorta, coronary arteries,
and aortic branches, with risk of vascular stenosis. Infiltration of the
pulmonary arteries, superior vena cava, pericardium, and myocardium may also
occur. When pulmonary involvement is present, a constellation of CT findings
is commonly suggestive of the diagnosis.
Regardless of the clinical and radiologic presentation, the diagnosis of
Erdheim-Chester disease may easily be reinforced by the presence of bilateral
and symmetric osteocon-densation and thickening of the diaphyseal and
metaphyseal cortex of the long bones shown by scintigraphic radiotracer uptake
and then confirmed by biopsy of osseous or perivisceral infiltration.
Conversely, because retroperitoneal and vascular involvement in
Erdheim-Chester disease is often asymptomatic, thoracoabdominal CT must be
performed in all patients with a clinical suspicion of this disease. CT can
help reveal visceral and vascular sites of involvement that can lead to
life-threatening complications. MRI remains an alternative in patients with
contraindications to iodinated IV contrast media.
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Top
Introduction
Renal and Perirenal Involvement
