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CT Differentiation of Large
Exophytic Renal Angiomyolipomas
and Perirenal Liposarcomas
Gary M. Israel 1 OBJECTIVE. The purpose of our study was to describe the imaging findings and CT
Morton A. Bosniak characteristics that lead to accurate distinction of large exophytic renal angiomyolipomas
Chrystia M. Slywotzky from retroperitoneal perirenal liposarcomas, which at times can be confused on imaging stud-
Robert J. Rosen ies and even at pathologic examination.
MATERIALS AND METHODS. We retrospectively analyzed CT images of 15 large
exophytic renal angiomyolipomas and 12 well-differentiated perirenal liposarcomas. Patho-
logic correlation was available for six of 15 angiomyolipomas and all of the liposarcomas. All
examinations were evaluated for lesion size, renal parenchymal defect, enlarged vessels, kid-
ney displacement, lesion encapsulation or margination, associated hemorrhage, and additional
angiomyolipomas. The records of patients with tuberous sclerosis or the forme fruste of that
condition were excluded from the study.
RESULTS. The average size of the angiomyolipomas was 14 10 cm. They showed a re-
nal parenchymal defect (n = 15), enlarged vessels (n = 12), renal displacement (n = 14), good
margination without a distinct capsule (n = 14), hemorrhage (n = 1), and additional (one or
two) angiomyolipomas (n = 4). The average size of the liposarcomas was 18 11.6 cm. They
showed enlarged vessels (n = 3), renal displacement (n = 11), and encapsulation (n = 4); none
showed a renal parenchymal defect, hemorrhage, or associated angiomyolipomas.
CONCLUSION. Although large exophytic angiomyolipomas and well-differentiated ret-
roperitoneal liposarcomas may have similar appearances on imaging, careful evaluation for a
defect in the renal parenchyma combined with the presence of enlarged vessels in angiomyo-
lipomas should enable accurate differentiation in almost all cases. Achieving an accurate di-
agnosis can have a significant impact on patient treatment.

L iposarcoma and exophytic renal an-

Received January 8, 2002; accepted after revision
March 5, 2002.
1
All authors: Department of Radiology, Division of
Abdominal Imaging, HW 202, New York University Medical
Center, 560 First Ave., New York, NY 10016. Address
correspondence to G. M. Israel.
AJR 2002;179:769773
0361803X/02/1793769
American Roentgen Ray Society
giomyolipoma represent two retro-
peritoneal masses that contain fatty
elements. On occasion, their appearances may
be so similar that they can be confused on imag-
ing and even sometimes at histologic examina-
tion. However, their differentiation is important
because the prognosis and treatment are differ-
ent. For liposarcomas, surgical resectionusu-
ally with the adjacent kidneyis necessary,
although complete surgical removal is difficult,
and recurrence is common [1, 2]. Angiomyoli-
pomas, although benign, may hemorrhage and
require emergent treatment (embolization or
surgery) if life-threatening bleeding occurs, but
they do not necessarily require surgery [35].
Liposarcomas, which occur slightly more fre-
quently in men than in women, are among the
most common primary retroperitoneal malig-
nancies, with the perinephric region a frequent
location [2, 6]. Angiomyolipoma (renal hamar-
toma), a benign tumor, is more common in
women. These tumors may grow to be large and
bulky, extending into the perinephric space. The
objective of our article is to describe the imag-
ing findings and CT characteristics that lead to
accurate diagnosis of these two entities, which
can be difficult to differentiate when large.
Materials and Methods
We searched a computerized radiology database
(1988 to present) and a computerized pathology data-
base (1996 to present) in our institution for all large (>10
cm) exophytic angiomyolipomas and for large well-dif-
ferentiated retroperitoneal liposarcomas. Those cases
were supplemented with material from our teaching
files and cases sent to us for consultation from outside
institutions. This search yielded 27 cases. We identified
15 patients (14 women and one man) with exophytic an-
giomyolipomas, whose ages ranged from 24 to 76 years
(mean age, 50.5 years). Twelve patients (five women
and seven men) had retroperitoneal liposarcomas; their
age range was 4879 years (mean age, 62.3 years).

AJR:179, September 2002 769


Six of 15 angiomyolipomas were surgically
proven. Five patients underwent angiography and
embolization and had follow-up examinations
with CT from 4 months to 7 years 3 months later
(mean follow-up time, 2 years 2 months). In one
of the five patients who underwent embolization,
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symptoms persisted, and the tumor and kidney
were removed. Lesions in the five remaining pa-
tients showed characteristic CT findings of angi-
omyolipoma (fat density mass, renal parenchymal
defect, enlarged vessels) and were being fol-
lowed clinically at the conclusion of this study.
All 12 liposarcoma cases were surgically proven.
Because our study was retrospective, the examina-
tions were performed on a variety of helical and con-
ventional CT scanners using various slice
collimations (range, 310 mm: 3 mm [n = 2]; 5 mm
[n = 16]; 7 mm [n = 4]; 10 mm [n = 5]). All examina-
tions were performed with IV contrast material, but
the type and amount of contrast agent varied.
All cases were analyzed by two of the authors
working in consensus. Each mass was evaluated for
its size in the two greatest dimensions, presence or
absence of a defect in the renal parenchyma associ-
ated with the lesion, presence and measurement of
enlarged vessels in the mass (the two largest when
present), extent of displacement of the kidney from
the renal fossa, encapsulation or margination of the
mass, and any associated hemorrhage.
A renal parenchymal defect (not an extrinsic pres-
sure defect or deformity) was graded as definitely
present, probably present, or not present. Displace-
ment of the kidney from the renal fossa was graded
as mild displacement (score of 1), moderate displace-
ment (score of 2), or severe displacement (score of
3). In addition, both kidneys were evaluated for the
presence of additional angiomyolipomas.
Results
Angiomyolipomas
The average angiomyolipoma size was 14
10 cm. A defect in the renal parenchyma was
770
Israel et al.
definitely identified in 13 of the 15 lesions. A de-
fect was probably present in the remaining two
lesions. Twelve of the 15 angiomyolipomas con-
tained enlarged vessels (diameter range, 0.51.8
cm; mean diameter of the two largest vessels,
1.2 and 0.8 cm). Enlarged vessels were present
in the two lesions in which a parenchymal defect
was not definitely identified but probably
present. In 14 of 15 cases, the kidney was dis-
placed from the renal fossa (mean, 1.5 on a 13
scale). In the remaining case, the kidney was not
displaced by the lesion. Fourteen cases were
well marginated, and none showed a distinct
capsule. Associated hemorrhage was present in
one patient. In four patients, one or two addi-
tional angiomyolipomas (diameter range, 0.52
cm; mean, 1.1 cm) were present in either the ip-
silateral or contralateral kidney.
In one of the six cases of pathologically
proven angiomyolipoma, the kidney and tu-
mor had been sent to pathology labeled as a
liposarcoma. At pathology, the tumor was
initially thought to represent a liposarcoma.
However, when a defect in the renal paren-
chyma and the presence of enlarged vessels
were revealed on CT, the findings at pathol-
ogy were reviewed. The tumor was then con-
firmed to be an angiomyolipoma.
Liposarcomas
The average liposarcoma size was 18 11.6
cm. A sharp defect in the renal parenchyma was
not identified in any case. In a single case, in
which only 10-mm-thick images were available
for review, renal parenchymal invasion was
questioned on imaging, but the renal capsule
was found at pathology to be intact and without
parenchymal infiltration. Prominent enlarged
vessels were present in three cases (diameter
A B
range, 0.81.0 cm; mean diameter of the two
largest vessels, 0.9 and 0.9 cm). In 11 of the 12
cases, the kidney was displaced from the renal
fossa (mean, 2.5 on a 13 scale). In four cases,
the liposarcoma showed a distinct capsule; in
the other eight cases, the margin of the mass
was not clearly defined. In none of the liposar-
coma cases was there associated hemorrhage or
renal angiomyolipomas.
Discussion
Angiomyolipoma of the kidney, or renal
hamartoma (sometimes referred to as choris-
toma), is easily diagnosed preoperatively when
intratumoral fat can be shown [1, 5, 7]. However,
a large predominately exophytic angiomyoli-
poma may be difficult to distinguish from a
well-differentiated perirenal liposarcoma be-
cause both are large fat-containing lesions and
superficially appear similar [8]. Because the
prognosis and often the treatment differ for these
two conditions, it is important that they be accu-
rately differentiated before treatment or manage-
ment is instituted. Although these lesions may be
confused on occasion as discussed in the imag-
ing and pathologic literature [810], they can al-
most always be diagnosed correctly by close
attention to three major imaging findings.
Defect in the Renal Parenchyma
Because renal angiomyolipomas may grow
exophytically, most of the neoplasm may ex-
tend into the perirenal space. However, because
these angiomyolipomas arise from the kidney,
a defect will be present in the renal parenchyma
at their origin (Figs. 1 and 2). This finding was
present in all of our cases of angiomyolipoma.
In comparison, liposarcomas arise in the retro-
peritoneal fat, including the perirenal fat within
Fig. 1.53-year-old woman with 20
15 cm angiomyolipoma.
A, Contrast-enhanced CT scan
shows large predominately fat-con-
taining mass arising from left kidney
with sharp defect in renal paren-
chyma (black arrow) and enlarged
vessels measuring up to 1.5 cm
(white arrow). Findings are charac-
teristic of angiomyolipoma.
B, Contrast-enhanced CT scan ob-
tained inferior to A shows additional
enlarged vessels (arrows) and fur-
ther extent of lesion.
AJR:179, September 2002
 CT of Renal Angiomyolipomas and Perirenal Liposarcomas
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Fig. 2.39-year-old man with 10 10 cm angiomyoli-
poma. Contrast-enhanced CT scan shows well-de-
marcated defect in renal parenchyma (black arrows).
Enlarged vessels (white arrows) are also visible
within fatty tumor.
Gerotas fascia. They are often closely associ-
ated with the renal capsule and are sometimes
called capsular liposarcomas. When these le-
sions grow, they displace, compress, and distort
the kidney but usually do not invade the adja-
cent renal parenchyma [11]. Therefore, liposar-
comas do not cause a defect in the renal
parenchyma, and the interface of the lesion
with the kidney is smooth (Figs. 35).
In our series, none of the perirenal liposar-
comas invaded the renal parenchyma. In one
case in which CT was performed with 10-mm-
thick sections, parenchymal invasion was
questioned on the CT scan. However, the renal
capsule was found at pathology to be intact
and without parenchymal infiltration. More
aggressive liposarcomas could invade the kid-
Fig. 3.68-year-old man with 25 12
cm pathologically proven liposarcoma.
A, Contrast-enhanced CT scan
shows large fatty tumor compress-
ing kidney parenchyma with smooth
interface (arrows) and without evi-
dence of parenchymal defect. These
findings are indicative of well-differ-
entiated liposarcoma. Note mild hy-
dronephrosis.
B, Contrast-enhanced CT scan ob-
tained inferior to A shows extent of
neoplasm. No evidence of enlarged
vessels is seen.
AJR:179, September 2002
ney, but these are not the well-differentiated,
noninvasive fatty liposarcomas being dis-
cussed in this article.
A liposarcoma originating within the renal
sinus fat might be difficult to differentiate from
a central angiomyolipoma. In this case, the re-
nal parenchyma would be splayed around the
neoplasm and any possible defect of the renal
parenchyma might not be noted [9].
Vessels in the Lesion
Angiomyolipomas commonly contain en-
larged vessels that can be seen on contrast-
enhanced CT. In comparison, well-differentiated
liposarcomas are relatively avascular, and those
vessels that are present are not usually enlarged.
In our series, 12 of the 15 angiomyolipomas con-
tained enlarged vessels as compared with only
three of the 12 liposarcomas. Only one of the 12
liposarcomas had vessels as large as those present
in the angiomyolipoma group. Although the pres-
ence of these vessels is not as important in diag-
nosis as the presence of a defect in the renal
parenchyma, it is a significant ancillary finding. If
present, this finding should lead to a more thor-
ough search for a defect in the renal parenchyma
if one was not originally identified. The finding of
enlarged vessels can be used as an additional sign
that the lesion is an angiomyolipoma.
For the two cases of angiomyolipoma in
which a renal parenchymal defect was identi-
fied as not definitely but probably present,
enlarged and apparently aneurysmal vessels
were present within the tumor (Fig. 6). This
additional finding was a strong indicator that
the lesion was an angiomyolipoma and not a
liposarcoma. At pathology, both lesions were
proven to be angiomyolipomas.
A B
Presence of Additional Angiomyolipomas
Cases of tuberous sclerosis and the forme
fruste of tuberous sclerosis (bilateral multi-
ple angiomyolipomas without the full spec-
trum of the disease) were not included in this
study. The occurrence of large angiomyoli-
pomas in these settings is common and will
rarely create difficulty in making the diagno-
sis. However, angiomyolipomas may be mul-
tiple, even without associated tuberous
sclerosis. This finding occurred in 27% (4/
15) of patients with angiomyolipomas in our
study. The presence of other fatty lesions in
the ipsilateral or contralateral kidney, inde-
pendent of the dominate lesion, is a strong
indicator that the tumor in question is an an-
giomyolipoma. This can be a particularly im-
portant finding. As might be suspected, no
associated angiomyolipomas were seen in
our liposarcoma cases.
Additional Considerations
The lesions in our study were large: the li-
posarcomas measured on average 18 11.6
cm, and the angiomyolipomas, 14 10 cm.
Although the liposarcomas were generally
larger, overlap was found between the two
groups, and thus the size of the lesion was of
no diagnostic help.
Liposarcomas vary in their aggressiveness
and their histologic patterns and have been sep-
arated into five subtypes: well-differentiated li-
pomatous (the type under discussion), myxoid,
round cell, pleomorphic, and mixed [2]. It is the
well-differentiated form of liposarcoma that on
occasion can be difficult to differentiate from
the large exophytic angiomyolipoma both at
gross inspection and microscopically. Many of
771
 Israel et al.

Fig. 4.67-year-old woman with 16 13 cm patholog-

ically proven liposarcoma.
A, Contrast-enhanced CT scan shows displacement
of left kidney from renal fossa by large encapsulated
retroperitoneal fatty mass. No evidence is seen of re-
nal parenchymal defect or enlarged vessels, and in-
terface of tumor with kidney is smooth.
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B, Contrast-enhanced CT scan obtained inferior to A

confirms absence of enlarged vessels in extensive
fatty mass. Well-defined capsule (arrows) can be
seen.

A B

the renal and perirenal liposarcomas previously
reported in the literature were actually renal an-
giomyolipomas [10].
In many angiomyolipomas, the smooth
muscle cells can show variation in nuclear
size, which causes concern about the biologic
potential of the tumor. Occasional mitosis in
the smooth muscle portion compounds this
misleading suggestion of malignancy [10].
This finding can be a problem in particular
when a frozen section obtained at surgery is
evaluated. A tissue sample containing fatty tis-
sue and spindle cells could be called a liposar-
coma or an angiomyolipoma. The pathologist
must be alerted to the possibilities in the case
so that enough tissue is assessed to make a cor-
rect diagnosis. This difficulty occurred in one
case in this series in which the kidney and
mass were removed and labeled as a liposar-
coma. However, after review by the patholo-
gist, it was realized that the lesion was a large
exophytic angiomyolipoma.
It can even be difficult to determine patho-
logically whether the liposarcomatous mass
represents the rather common retroperitoneal
liposarcoma or the rare intrarenal tumor. The rar-
ity of a primary renal liposarcoma is illustrated
by a comprehensive review of the English-lan-
guage literature in 1990, which found only eight
cases of renal liposarcoma with unequivocal in-
volvement of the renal parenchyma [12]. Angi-
omyolipomas can be found in perirenal lymph
nodes [1], but usually they are small foci of this
hamartomamore often found in patients with
tuberous sclerosis and rarely leading to the large
and bulky lesions under discussion.
The prognosis for patients with angiomyoli-
poma is different than that for patients with
liposarcoma. Well-differentiated liposarcomas
grow slowly and do not tend to metastasize.
However, when they are large, they are diffi-
cult to entirely remove, and recurrent tumor is
a common result. Angiomyolipomas, on the
other hand, are benign lesions that are consid-
ered to be hamartomas or choristomas, not
neoplasms. Once removed, they will not recur.
The major complication associated with angi-
omyolipomas is hemorrhage (Fig. 6). This

A B

Fig. 5.67-year-old woman with 14 13 cm pathologi-
cally proven liposarcoma. Contrast-enhanced CT scan
shows that neoplasm displaces, compresses, and dis-
torts right kidney, but interface of kidney with tumor
(arrow) is smooth. Most of neoplasm is seen on other
sections (not shown) that reveal sparsity of vessels.
Fig. 6.76-year-old woman with 16 15 cm pathologically proven angiomyolipoma.
A, Contrast-enhanced CT scan obtained at level of upper pole of left kidney shows large fatty tumor in retroperi-
toneum surrounding kidney (K) and displacing spleen (S) anteriorly. Defect in upper pole of renal parenchyma is
probably present (long straight arrow). Enlarged vessels (short straight arrow) and surrounding hemorrhage
(curved arrow) can be seen. Note inferior vena cava filter in place.
B, Contrast-enhanced CT scan obtained inferior to A further reveals fatty tumor surrounding kidney (K) with as-
sociated enlarged vessels (straight arrow) and surrounding hemorrhage (curved arrow). S = spleen.

772 AJR:179, September 2002

 CT of Renal Angiomyolipomas and Perirenal Liposarcomas
problem may be controlled prophylactically or
with embolization techniques at the time of
such an event [13]. Alternatively, a watchful
waiting approach may be used, in which the
patient is informed of the potential complica-
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tion and alerted to obtain treatment should a
possible episode of hemorrhage occur [3].
Because angiomyolipomas and liposarco-
mas require different treatment, accurate diag-
nosis is important. Obviously, liposarcomas
should be surgically removed, and almost al-
ways the adjacent kidney is removed as well.
On the other hand, most angiomyolipomas can
be treated with embolization techniques that
will devascularize the lesion and possibly
shrink it. This treatment will prophylactically
address the major complication of angiomyoli-
poma, spontaneous hemorrhage [4].
In our series, five patients with angiomyo-
lipomas underwent embolization. At short-
term follow-up, four patients showed mini-
mal shrinkage of the tumor and were asymp-
tomatic. The fifth patient had a 20-cm lesion.
In this patient, initial shrinkage occurred af-
ter embolization, but the lesion reenlarged
after a 7-year interval and was surgically re-
moved because it caused discomfort. Six of
the patients with angiomyolipomas under-
went surgical removal of the tumor and the
kidney. In these cases, either the surgeon or
the patient decided on this course of treat-
ment. Five patients had not undergone sur-
gery or embolization at the conclusion of our
study and were being followed up clinically.
These cases were generally the smaller le-
sions in the series, and all of them were
clearly seen to be angiomyolipomas accord-
ing to imaging criteria. Three of these pa-
tients had one or two other small
angiomyolipomas in the ipsilateral or con-
tralateral kidney. In those few instances in
AJR:179, September 2002
which differentiation is ambiguous, surgery
for removal of the lesion is indicated.
The CT protocol was not standard for all
patients, and this represents a limitation in the
study. Although most imaging was performed
using 3- or 5-mm collimation, some patients
were studied using 10-mm sections, and these
were the cases that caused difficulty in deter-
mining the presence or absence of a parenchy-
mal defect. In the two patients with
angiomyolipomas in which a parenchymal de-
fect was not definitely identified but consid-
ered probably present, having thinner sections
might have increased the confidence level. In
addition, for the single patient having liposar-
coma in which renal parenchymal invasion
was questioned, thinner sections might have
helped clarify this finding. Having thinner sec-
tions is particularly helpful when a lesion
arises from a pole of the kidney (Fig. 6). Fur-
thermore, sagittal reconstructed images or the
multiplanar capability of MR imaging may
have been useful. Also, although pathologic
proof is not available for all of the angiomyoli-
poma cases, these lesions clearly represent an-
giomyolipomas arising from the kidney when
evaluated using established imaging criteria.
Finally, the sample size is relatively small, and
larger series of cases need to be studied to cor-
roborate our findings.
Conclusion
Large exophytic angiomyolipomas and
well-differentiated retroperitoneal liposarco-
mas can have similar appearances on imaging
studies. A careful evaluation for a sharp defect
in the renal parenchyma and the presence of en-
larged vessels and associated angiomyolipomas
should enable accurate differentiation of these
lesions in almost all cases and should lead to
proper clinical management.
Acknowledgments
We thank Yale Shulman (Newark, NJ), Pe-
ter Nardi (Brooklyn, NY), and Daniel Alter-
man (Bronx, NY) for their contributions of
case material.
References
1. Murphy WM, Beckwith JB, Farrow GM. Tumors of
the kidney, bladder, and related urinary structures.
In: Rosai J, ed. Atlas of tumor pathology, 3rd series,
fasc. 11. Washington, DC: Armed Forces Institute of
Pathology, 1994:161174
2. Patel SK. Retroperitoneal tumors and cysts. In: Pol-
lack HM, McClennan BL, eds. Clinical urography,
2nd ed. Philadelphia: Saunders, 2000:28232865
3. Kennelly MJ, Grossman HB, Cho KJ. Outcome
analysis of 42 cases of renal angiomyolipoma. J Urol
1994;152:19881991
4. Wagner BJ, Wong-You-Cheong JJ, Davis CJ Jr.
From the archives of the AFIP: adult renal hamar-
tomas. RadioGraphics 1997;17:155169
5. Bosniak MA. Angiomyolipoma (hamartoma) of
the kidney: a preoperative diagnosis is possible in
virtually every case. Urol Radiol 1981;3:135142
6. Lane RH, Stephens DH, Reiman HM. Primary retro-
peritoneal neoplasms: CT findings in 90 cases with clin-
ical and pathologic correlation. AJR 1989;152:8389
7. Bosniak MA, Megibow AJ, Hulnick DH, Horii S,
Raghavendra BN. CT diagnosis of renal angi-
omyolipoma: The importance of detecting small
amounts of fat. AJR 1988;151:497501
8. Medina Perez M, Garcia Ferris G, Martin San-
juan A, Valero Puerta J. Exophytic renal angioli-
poma versus retroperitoneal liposarcoma [in
Spanish]. Arch Esp Urol 1998;51:10341036
9. Zagoria RJ, Dyer RB, McCullough DL. Angiomy-
olipoma arising in the renal sinus: a difficult radio-
logic diagnosis. Urol Radiol 1989;11:139141
10. Peterson RO. Urologic pathology, 2nd ed. Phila-
delphia: Lippincott, 1992:115120
11. Farrow GM. Disease of the kidney. In: Murphy
WM, ed. Urologic pathology, 2nd ed. Philadel-
phia: Sanders, 1997:430502
12. Mayes DC, Fechner RE, Gillenwater JY. Renal li-
posarcoma. Am J Surg Path 1990;14:268273
13. Rosen RJ, Schlossberg P, Roven SJ, Rothberg M.
Management of symptomatic renal angiomyolipo-
mas by embolization. Urol Radiol 1984;6:196200
773