AJR 2003; 180:621-625
© American Roentgen Ray Society
Imaging Characteristics of Cystic Adventitial Disease of the Peripheral Arteries: Presentation as Soft-Tissue Masses
Jeffrey J. Peterson1,
Mark J. Kransdorf1,2,
Laura W. Bancroft1 and
Mark D. Murphey2,3,4
1 Department of Radiology, Mayo Clinic, 4500 San Pablo Rd., Jacksonville, FL
32224-3899.
2 Department of Radiologic Pathology, Armed Forces Institute of Pathology, 6825
16th St., N.W., Bldg. 54, Rm. 127A, Washington, DC 20306-6000.
3 Department of Radiology, University of Maryland Medical Center, 22 S. Greene
St., Baltimore, MD 21201-1595.
4 Departments of Radiology and Nuclear Medicine, Uniformed Services University
of the Health Sciences, 4301 Jones Bridge Rd., Bethesda, MD 20814-4799.
Received June 14, 2002;
accepted after revision August 12, 2002.
Address correspondence to M. J. Kransdorf.
The opinions and assertions contained herein are the private views of the
authors and are not to be construed as official or representing the views of
the Department of the Army or the Department of Defense.
Presented at the annual meeting of the American Roentgen Ray Society,
Atlanta, April-May 2002.
Abstract
OBJECTIVE. Our purpose was to identify the characteristic imaging
features of cystic adventitial disease of the peripheral arteries.
CONCLUSION. Patients with cystic adventitial disease of the
peripheral arteries often present for evaluation of soft-tissue masses
involving the extremities. Noninvasive imaging reveals a characteristic
appearance allowing confident diagnosis.
Introduction
Cystic adventitial disease is an uncommon vascular disorder characterized
by cystic degeneration of a peripheral artery. Any peripheral artery can be
affected; however, the disorder has a striking proclivity for the popliteal
artery and typically affects young to middle-aged men without evidence of
atherosclerosis or other systemic vascular disease
[1]. Affected patients
typically present with sudden onset of rapidly progressive calf claudication
and lower extremity pain. Physical examination often shows a soft-tissue mass,
and, in a younger patient with no evidence of arteriosclerosis, clinicians
often focus on the soft-tissue mass, suspecting a soft-tissue sarcoma or a
popliteal cyst rather than a vascular cause.
We detail our experience with seven patients with cystic adventitial
disease, of whom five were referred for evaluation of a soft-tissue mass and
two, for evaluation of unexplained lower extremity pain. We retrospectively
reviewed imaging characteristics of this lesion to identify those features
that would allow an accurate prospective diagnosis.
Materials and Methods
We retrospectively reviewed the imaging studies of seven patients with
surgically and pathologically confirmed cystic adventitial disease of the
peripheral arteries. The study group consisted of six men and one woman. Ages
ranged from 38 to 79 years (mean, 53 years). MR imaging was performed in all
patients, with IV gadolinium-enhanced MR imaging in four patients. MR imaging
included spin-echo T1-weighted images (n = 6) and either conventional
spin-echo dual-echo or short tau inversion recovery sequences (n =
7). Conventional angiography was performed in six patients, with three of
these patients also having MR angiography. MR angiography was performed with a
two-dimensional time-of-flight technique in two patients and three-dimensional
contrast-enhanced MR angiography in one patient. Sonography was performed in
four patients, including intraarterial sonography in one patient.
Sonographically guided aspiration was performed in one patient. Conventional
radiographs were available in two patients. Five patients presented for
evaluation of soft-tissue masses discovered at physical examination. Two
patients presented for unexplained lower extremity pain.
Radiographs were evaluated for the presence of a mass, calcification, or
bone erosion. The MR images were analyzed for signal intensity, signal
homogeneity, enhancement pattern, lesion size, and location. Skeletal muscle
and fat were used as the reference tissue for all MR imaging sequences.
Sonograms were evaluated for lesion echogenicity and Doppler flow.
Results
All cases showed involvement of a single peripheral artery. Lesions were
located in the popliteal (n = 6) and radial arteries (n =
1). No patients showed evidence of diffuse atherosclerosis or other
generalized arterial disease. Length of arterial involvement varied from 1.7
to 11.2 cm (mean, 4.6 cm). Nonocclusive disease was seen in three patients,
and segmental occlusion in four. The length of occlusion ranged from 1.7 to
11.2 cm (mean, 5.4 cm).
Radiographs failed to show the lesions in both cases. Subtle soft-tissue
prominence was seen in a single case (Fig.
1A,
1B). No arterial vascular
calcification suggesting atherosclerosis was present in any patient.
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Fig. 1A. —38-year-old man with cystic adventitial disease of radial
artery who presented with soft-tissue mass in volar aspect of wrist. Oblique
radiograph of wrist shows subtle, nonspecific, soft-tissue mass
(arrow) in soft tissues adjacent to distal radius in region of radial
artery. No soft-tissue calcifications are seen.
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Fig. 1B. —38-year-old man with cystic adventitial disease of radial
artery who presented with soft-tissue mass in volar aspect of wrist.
Gray-scale sonogram of distal radial artery (arrow) shows multiple
hypoechoic and anechoic masses (asterisk) arising in wall of artery
consistent with cystic adventitial disease.
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MR imaging showed aggregates of multiple small round or oval masses
originating in the wall of the involved peripheral artery, with larger lesions
having a multiloculated appearance. The individual intramural masses showed
homogenous low signal intensity on T1-weighted MR images and high signal
intensity on T2-weighted or fluid-sensitive sequences, consistent with a
cystlike character (Fig. 2A,
2B,
2C,
2D,
2E). After IV gadolinium
administration, no enhancement was seen in three patients (Fig.
3A,
3B,
3C,
3D,
3E). Mild peripheral
enhancement was seen in one patient. There was resultant compromise of the
arterial lumen by the multilobulated cystic masses, which varied in degree
from minimal eccentric indentation of the arterial lumen, to more prominent
crescentic stenosis, to complete occlusion. MR angiography in three patients
showed well the degree of luminal compromise and the extrinsic, intramural
nature of the stenosis (Fig.
3A,
3B,
3C,
3D,
3E). In two patients in whom
MR angiography showed occlusion, prominent collaterals were seen with
reconstitution of the distal arteries. No significant vascular disease was
seen proximal or distal to the lesions. Correlation with MR angiography and
conventional angiography was available in two patients and was excellent.
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Fig. 2A. —61-year-old man with cystic adventitial disease of popliteal
artery who presented with unexplained lower extremity pain and soft-tissue
fullness in popliteal fossa. Sagittal T2-weighted MR image of knee shows
multiple high-signal-intensity adventitial cysts (black asterisk)
arising from wall of popliteal artery with extrinsic compression of arterial
lumen (white asterisk).
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Fig. 2B. —61-year-old man with cystic adventitial disease of popliteal
artery who presented with unexplained lower extremity pain and soft-tissue
fullness in popliteal fossa. Angiogram shows focal extrinsic narrowing of
arterial lumen of popliteal artery (arrow) typical of cystic
adventitial disease.
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Fig. 2C. —61-year-old man with cystic adventitial disease of popliteal
artery who presented with unexplained lower extremity pain and soft-tissue
fullness in popliteal fossa. Intraoperative photograph of popliteal artery
reveals focal bluish-colored mass (arrow) arising from wall of artery
in region of previously noted stenosis on arteriography.
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Fig. 2D. —61-year-old man with cystic adventitial disease of popliteal
artery who presented with unexplained lower extremity pain and soft-tissue
fullness in popliteal fossa. Intraoperative photograph of dissected arterial
wall shows focal nodular masses arising from it (arrow).
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Fig. 2E. —61-year-old man with cystic adventitial disease of popliteal
artery who presented with unexplained lower extremity pain and soft-tissue
fullness in popliteal fossa. Microscopic evaluation reveals areas of cystic
degeneration (asterisks) in adventitia of popliteal artery.
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Fig. 3A. —79-year-old man with popliteal soft-tissue mass. MR angiogram
of popliteal artery shows occlusion of segments of artery. Margins are rounded
and crescentic, consistent with extrinsic nature of occlusion
(arrows). Collateral vessels are seen with distal reconstitution of
artery.
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Fig. 3B. —79-year-old man with popliteal soft-tissue mass. Enhanced
T1-weighted sagittal image of knee with fat saturation shows multiple cystic
masses arising from wall of popliteal artery consistent with cystic
adventitial disease (arrows). Popliteal vein is seen posteriorly
(arrowheads).
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Fig. 3C. —79-year-old man with popliteal soft-tissue mass. Enhanced
T1-weighted axial image of knee depicts adventitial cyst (asterisk)
with complete obliteration of arterial lumen at this level.
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Fig. 3D. —79-year-old man with popliteal soft-tissue mass. Sonogram
shows sonographically guided cyst aspiration. Eighteen-gauge spinal needle
(arrow) is visualized with distal tip in adventitial cyst
(asterisks) arising from popliteal artery.
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Conventional angiography showed smooth, tapered stenoses with curvilinear
or spiral narrowing of the lumina of the involved vessels (Fig.
2A,
2B,
2C,
2D,
2E). The stenoses showed
rounded, spiral, or hourglass morphologies with extrinsic compression.
Nonocclusive segmental narrowing was seen in three patients with no
significant poststenotic dilatation. Arterial occlusion was seen in four
patients with formation of collaterals that reconstituted the distal arteries.
Concomitant atherosclerotic changes were absent.
Sonography showed multilobulated collections of small rounded or oval,
anechoic or hypoechoic masses arising in the walls of the affected arteries
(Fig. 1A,
1B). Crescentic indentation on
the anechoic lumina of the vessels was seen with eccentric narrowing or
occlusion by the cystic masses. Low-level echoes were seen in several masses
indicative of debris in the contents of the cysts. Duplex Doppler sonography
confirmed the absence of flow in the cystlike lesions and extrinsic narrowing
of the affected arteries. Sonographically guided cyst aspiration was performed
in one patient with aspiration of thick gelatinous mucinous material (Fig.
3A,
3B,
3C,
3D,
3E). Intraarterial sonography
showed hypoechoic masses in the wall of the popliteal artery and crescentic
narrowing of the lumen (Fig.
4A,
4B,
4C).
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Fig. 4A. —50-year-old male patient with large adventitial cyst of
popliteal artery. Intraarterial sonogram precisely shows large cystic mass
(asterisk) arising from adventitia of popliteal artery.
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Fig. 4C. —50-year-old male patient with large adventitial cyst of
popliteal artery. Photograph of gross specimen shows adventitial cyst
(white asterisk) after dissection from resected arterial specimen
(black asterisk).
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Discussion
Cystic adventitial disease is a relatively rare vascular condition with the
typical patient a middle-aged man complaining of rapidly progressing
claudicationlike symptoms [2].
Five (71%) of our patients presented for the evaluation of a soft-tissue mass,
a presentation that, to our knowledge, has not been previously emphasized. All
patients had intermittent extremity pain; however, the mass was the dominant
clinical finding and became the focus of the examination. The cause of cystic
adventitial disease is controversial. First reported in 1954 by Ejrup and
Hiertonn [3], cystic
adventitial disease was originally described as a hematoma of the arterial
wall [4]. Further studies
eventually elucidated the true nature of the lesions identifying the cyst
contents as grossly resembling thick mucinous gel containing varying
combinations of mucoproteins, mucopolysaccharides, hyaluronic acid, and
hydroxyproline [5,
6]. The progression of cystic
adventitial disease is slow, occurring over a period of several years
[7], accounting for the large
size and masslike configuration of many lesions, and also explaining the
clinical confusion with a slowly growing soft-tissue tumor.
The MR imaging appearance of cystic adventitial disease is quite
characteristic, showing multiple arterial intramural cystlike masses. The
individual lesions typically show homogenous low signal intensity on
T1-weighted spin-echo MR images and high signal intensity on fluid-sensitive
sequences (Fig. 2A,
2B,
2C,
2D,
2E). The lesions are oriented
along the long axis of the vessel, and this orientation, as well as the
intramural location, is well shown on high-spatial-resolution multiplanar MR
imaging. Large lesions form conglomerate multilobulated masses; however, these
features are still present in the smaller individual components. Individual
lesions showed no enhancement or minimal marginal enhancement (Fig.
3A,
3B,
3C,
3D,
3E). However, we suspect that
because the basic pathophysiology of the disease process entails a myxoid
degeneration in the adventitia of the vessel wall
[8,
9], individual lesions may
enhance as seen in other myxoid processes.
Although angiography has been regarded the gold standard for the diagnosis
of cystic adventitial disease, we found MR angiography to be equally
diagnostic (Figs. 2A,
2B,
2C,
2D,
2E and
3A,
3B,
3C,
3D,
3E). Both techniques showed
with exquisite detail the extrinsic compression of the affected vessel with
absence of significant disease proximal and distal to the lesion. They also
showed the hourglass configuration that is seen with circumferential
involvement and the crescentic margins in cases of complete occlusion. MR
angiography accurately showed the degree and length of stenosis or occlusion
as well as the lack of vascular abnormality elsewhere in the affected
extremity. In our opinion, MR angiography equaled conventional angiography for
both diagnosis and surgical planning, with the obvious advantage of a
noninvasive evaluation.
Sonography is also useful in showing the characteristic appearance of
cystic adventitial disease. Sonography reveals the anechoic or hypoechoic
masses originating in the arterial wall and can show the degree of associated
vascular compromise (Fig. 1A,
1B). Sonography typically
reveals the lesions as multilobulated masses composed of multiple smaller
cysts. Low-level echoes may be seen in the cysts, indicating the gelatinous
nature of the contents of the cyst. Color and duplex Doppler evaluation of the
arterial lumen is useful to depict the extent of the associated arterial
stenosis or complete occlusion of the affected artery. Intraarterial
sonography was performed in one patient in our series, precisely showing the
cystic masses arising from the adventitia of the arterial wall.
In conclusion, cystic adventitial disease is an unusual vascular disorder
that may present clinically as a slowly growing soft-tissue mass. MR imaging
and MR angiography are both characteristic, with the former showing the
multiple small intramural cystlike lesions in the arterial wall and the latter
the eccentric, scalloped stenosis and degree of vascular compromise.
Sonography also shows the lesions well and depicts the degree of arterial
compromise. Although conventional angiography continues to be the gold
standard, noninvasive MR angiography is diagnostic. Radiologists should be
familiar with the entity and be well acquainted with its clinical presentation
and diagnostic radiologic appearance.
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Abstract
Introduction
