AJR 2003; 181:139-142
© American Roentgen Ray Society
Carcinosarcoma of the Urinary Bladder: Dynamic Contrast-Enhanced MR Imaging with Clinical and Pathologic Correlation
Aylin Tekes1,
Ihab R. Kamel1,
Gilberto Szarf1,
Theresa Y. Chan2,
Mark P. Schoenberg3 and
David A. Bluemke1
1 Russell H. Morgan Department of Radiology and Radiological Sciences, Johns
Hopkins Hospital, Rm. 100, 600 N. Wolfe St., Baltimore, MD 21287.
2 Department of Pathology, Weinberg Bldg., Johns Hopkins Hospital, Baltimore, MD
21287.
3 James Buchanan Brady Urological Institute, Johns Hopkins University Medical
Institutions, Marburg 150, Baltimore, MD 21287.
Received September 10, 2002;
accepted after revision January 2, 2003.
Address correspondence to I. R. Kamel.
Abstract
OBJECTIVE. This article describes the MR imaging features of
carcinosarcoma of the urinary bladder with clinical presentation and
pathologic correlation in three adults.
CONCLUSION. Carcinosarcoma of the urinary bladder is a rare and
aggressive tumor that has a clinical presentation similar to that of
transitional cell carcinoma of the bladder. Dynamic gadolinium-enhanced MR
imaging features are discussed.
Introduction
Carcinosarcomas are rare neoplasms of the urinary bladder and are more
commonly observed in the female genital tract. Approximately 70 cases have
been reported to date [1].
Carcinosarcoma of the bladder presents in an advanced stage and rapidly
becomes fatal. To our knowledge, two cases of carcinosarcoma depicted on MR
imaging, including T1- and T2-weighted sequences without gadolinium-enhanced
imaging features, have been reported in the radiology literature
[2,
3]. We describe three
pathologically proven cases of urinary bladder carcinosarcoma with dynamic
contrast-enhanced MR imaging, clinicopathologic correlation, and clinical
follow-up.
Materials and Methods
Between 1996 and 2001, we retrospectively reviewed the pathology department
database records of patients with carcinosarcoma of the urinary bladder.
During this time, the records of eight patients were found in the database. Of
these eight patients, three had MR examinations in our institution. This
retrospective case review was exempt from institutional board review at our
institution.
Clinical data including age, sex, presentation, time between MR imaging and
surgery, type of surgery, pathology results, and follow-up after surgery were
noted. The metastatic workup before surgery included CT of the chest,
abdominal MR imaging, and a bone scan. All the patients were followed up for
3–12 months after surgery. In all patients, diagnosis was based on
histopathologic results after surgery. One patient had partial and two
patients had radical cystectomies, and all three patients had bilateral pelvic
lymphadenectomies. For each case, histologic sections were obtained from
cystectomy specimens and correlated with the MR imaging findings.
MR imaging was performed on all three patients using a 1.5-T scanner
(Signa, General Electric Medical Systems, Milwaukee, WI) with a phased array
pelvic coil. Conventional T1-weighted spin-echo images (TR/TE, 550/9; matrix,
512 x 192; field of view, 20 cm; section thickness, 6 mm; intersection
gap, 2 mm; number of excitations, 4) and T2-weighted fast spin-echo images (TR
range/TE range, 3500–5900/80–120; matrix, 256 x 256; field
of view, 24 cm; section thickness, 6 mm; intersection gap, 2 mm; number of
excitations, 4) were obtained in the axial plane with fat suppression.
Subsequently, fast multiplanar spoiled gradient-echo axial images
(220–295/1.7–4.2; flip angle, 70°; matrix, 512 x 192;
slice thickness, 6 mm; intersection gap, 2 mm; number of excitations, 2) with
fat suppression were obtained before and 2 min after injection of
gadopentetate dimeglumine (0.1 mmol/kg). Coronal oblique T2-weighted and
gadolinium-enhanced images were obtained of tumors located in a diverticulum
in one patient.
MR imaging was evaluated for tumor number, size, location, T1- and
T2-signal intensities, and enhancement patterns. We also evaluated tumor
extension into perivesical fat, surrounding organs, and the abdominal wall and
associated pelvic lymph node involvement. All the tumors were staged using the
TNM classification system (updated in 1997)
[4].
Results
Clinical Findings
Two men and one woman, who were from 70 to 79 years old (average age, 74
years), were evaluated. They all presented to an outside institution with
gross hematuria. One patient had exposure to chemical warfare products early
in his occupation, and his medical history was significant for aplastic anemia
of 17 years' duration and surgically treated melanoma of the scalp 10 years
previously. One patient had abdominal pain, a palpable pelvic mass, and
bacteriuria. All three patients had transurethral resection, and biopsy
results revealed carcinosarcoma. One patient had undergone additional
sonography that revealed a bladder diverticulum containing a mass. All
patients were referred to our institution for treatment options. One patient
was treated with two courses of intravesical bacille Calmette-Guérin
therapy before his referral.
MR Imaging Findings
All patients underwent dynamic contrast-enhanced MR imaging. Tumor size
ranged from subcentimeter to 9 cm in each of the three patients. One patient
had four tumor foci within a diverticulum arising from the left lateral
bladder wall. A 2.5-cm tumor was located at the dome of the diverticulum with
three additional subcentimeter tumor foci. These tumors were better visualized
on the oblique coronal plane of the T2-weighted images (Figs.
1A,
1B,
1C). The other tumor locations
included a broad 9-cm mass arising from the left lateral wall (Figs.
2A,
2B,
2C) and a 4-cm sessile mass
arising from the right lateral bladder wall with thickening of the dome and
the anterolateral walls (Figs.
3A,
3B,
3C,
3D).
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Fig. 1A. —70-year-old man with multiple carcinosarcomas (stage T2b) in
bladder diverticulum. Oblique coronal T2-weighted MR image (TR/TE, 5967/96)
shows large diverticulum arising from left lateral bladder wall. Note 2.5-cm
tumor that originates from dome (black arrow) and three additional
papillary tumor foci (white arrows). Large mass is heterogeneous in
signal intensity, which is uncommon for transitional cell carcinoma. Bladder
wall appears irregular due to incomplete distention.
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Fig. 1B. —70-year-old man with multiple carcinosarcomas (stage T2b) in
bladder diverticulum. Oblique coronal fat-suppressed arterial phase
contrast-enhanced fast spoiled gradient-echo MR image (295/4.2) shows
heterogeneous enhancement of large mass (large arrow) and
homogeneously enhancing small tumor foci (small arrows). No evidence
of bladder wall invasion is present.
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Fig. 1C. —70-year-old man with multiple carcinosarcomas (stage T2b) in
bladder diverticulum. Photomicrograph obtained from cystectomy specimen shows
rhabdomyosarcoma component of carcinosarcoma. Tumor shows spindle and plump
cells with eosinophlic cytoplasm and high mitotic activity. (H and E,
x400)
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Fig. 2A. —79-year-old woman with stage T3b carcinosarcoma of urinary
bladder. Axial unenhanced fast spoiled gradient-echo MR image (TR/TE, 237/4.2)
shows large intraluminal bladder mass (arrow). Mass is isointense to
bladder wall muscle.
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Fig. 2B. —79-year-old woman with stage T3b carcinosarcoma of urinary
bladder. Axial arterial phase contrast-enhanced fast spoiled gradient-echo MR
image (220/2.2) shows large heterogeneously enhancing mass arising from left
lateral bladder wall. Peripheral portion (straight arrows) does not
enhance, probably because of adherent thrombus. At site of tumor origin, tumor
invades bladder wall with perivesical fat (curved arrow).
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Fig. 2C. —79-year-old woman with stage T3b carcinosarcoma of urinary
bladder. Photomicrograph obtained from cystectomy specimen shows
adenocarcinoma component (arrows) of carcinosarcoma infiltrating
adipose tissue. (H and E, x400)
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Fig. 3A. —74-year-old man with stage T2b carcinosarcoma of urinary
bladder. Axial T2-weighted MR image (TR/TE, 3500/120) shows sessile mass
(straight arrow) arising from right lateral bladder wall with
associated thickening of dome and anterolateral walls (curved arrow).
Enlarged prostate is present at bladder base.
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Fig. 3B. —74-year-old man with stage T2b carcinosarcoma of urinary
bladder. Axial arterial phase contrast-enhanced fast spoiled gradient-echo MR
image with fat suppression (250/2.5) shows wall thickening (curved
arrow) and no contrast enhancement of mass (straight arrow).
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Fig. 3C. —74-year-old man with stage T2b carcinosarcoma of urinary
bladder. Axial 2-min delayed phase fast spoiled gradient-echo MR image
(250/2.5) shows only submucosal enhancement (arrow) of sessile mass
without any evidence of extravesical tumor invasion. This enhancement pattern
is unusual for transitional cell carcinomas, which usually show bright early
arterial enhancement. Pathologic examination found that entire bladder was
invaded by carcinosarcoma.
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Fig. 3D. —74-year-old man with stage T2b carcinosarcoma of urinary
bladder. Photomicrograph of cystectomy specimen shows squamous component of
carcinosarcoma with keratin formation (arrows) and rhabdomyosarcoma
component with spindle and plump cells. (H and E, x200)
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All tumors were isointense to the bladder wall muscle on T1-weighted
imaging. The T2-weighted imaging signal was variable, with subcentimeter
tumors in the bladder diverticulum isointense, whereas the 2.5-, 4-, and 9-cm
tumors were heterogeneous. After administration of gadolinium, enhancement was
variable. The 2.5- and the 9-cm masses showed heterogeneous enhancement, and
the subcentimeter tumors showed homogeneous enhancement (Figs.
1A,
1B,
1C and
2A,
2B,
2C). The 4-cm sessile mass did
not show any enhancement on early images, and on the second phase, there was
submucosal enhancement only (Figs.
3A,
3B,
3C,
3D).
None o`f the patients had evidence on MR imaging of extravesical disease or
lymph node involvement. Preoperative metastatic workup also did not reveal
distant metastases. Two patients underwent radical and one patient underwent
partial cystectomy with bilateral pelvic lymphadenectomy within 1 week to 2
months (mean, 1 month) after MR imaging. Pathologic staging revealed muscle
invasive tumor (stage T2b) including glandular adenocarcinoma, high-grade
spindle cell carcinoma, small cell carcinoma, and squamous cell carcinoma
(Figs. 1A,
1B,
1C); perivesical fat invasive
tumor (stage T3b) including transitional cell carcinoma, adenocarcinoma, in
situ carcinoma, and rhabdomyosarcoma (Figs.
2A,
2B,
2C); and another muscle
invasive tumor (stage T2b) including rhabdomyosarcoma, high-grade urothelial
carcinoma, squamous cell carcinoma, and small cell undifferentiated carcinoma
(Figs. 3A,
3B,
3C,
3D).
All patients had follow-up CT 3–10 months after surgery (mean, 5
months). One patient had multiple tumors in the bladder, two pelvic masses,
and a right obturator lymph node. Another patient had a large mass abutting
the left pelvic sidewall with lower paraaortic lymph node involvement, and a
third patient had a tumor-free CT at 6 months but presented with two masses in
the left pelvis 4 months later. All recurrences were pathologically proven.
Two patients were offered radiotherapy and chemotherapy but refused treatment,
and the other patient had two cycles of chemotherapy. All patients died within
1–3 months after recurrence.
Discussion
Carcinosarcoma of the urinary bladder is a rare neoplasm, with
approximately 70 cases having been reported in the literature
[1]. The tumor is a high-grade
neoplasm of the bladder with a malignant epithelial component (transitional,
glandular, squamous, or undifferentiated type) and a sarcomatous component
[1,
5–8].
The sarcomatous elements may consist of chondrosarcoma, osteosarcoma, or
rhabdomyosarcoma, in descending order of frequency
[1]. Carcinosarcoma of the
urinary bladder is an aggressive neoplasm with a mortality rate of
approximately 50% [9]. There is
male preponderance, and the median age (sixth to seventh decade) is similar to
that of patients with to transitional cell carcinoma. The most common
presenting symptom is gross hematuria, similar to that in transitional cell
carcinoma. The predominant location of carcinosarcomas is the bladder base,
followed by the trigone and lateral walls
[1]. Most tumors present as
single large polypoid masses, ranging from 1.5 to 12 cm
[1].
In our study, all three patients (two men, one woman) presented with gross
hematuria. Unlike prior reports
[2,
3], none of the tumors in our
series were located in the bladder base. Two of our patients—one who
presented with multiple tumors located in a diverticulum and the other who
presented with a 4-cm sessile mass with associated wall thickening—were
unusual cases. Multiple tumors and a diverticular origin are commonly observed
features for transitional cell carcinoma
[3,
10]. One of the patients
presented with a single 9-cm polypoid mass, which is the most common
presentation of carcinosarcoma. Among our patients, transitional cell
carcinoma was the most common epithelial component, and rhabdosarcoma was the
most common sarcomatous component.
All the tumors were isointense to the muscle on T1-weighted imaging. On
T2-weighted imaging, two patients had tumors with heterogeneous signal
intensity. Heterogeneous T2-signal intensity is not commonly observed in
transitional cell carcinoma of the bladder; most transitional cell carcinomas
are isointense to the bladder wall muscle
[11]. On contrast-enhanced
studies, two patients had heterogeneously enhancing tumors and the third
patient had delayed submucosal enhancement of the tumor. We have previously
observed delayed submucosal enhancement in association with intravesical
chemotherapy for transitional cell carcinoma. None of the tumors showed early
bright arterial enhancement, which is a common feature of transitional cell
carcinoma. Because carcinosarcomas contain both carcinomatous and heterologous
sarcomatous components, pathologic examination shows a variety of different
histologic components. This finding may reflect heterogeneous signal intensity
on T2-weighted imaging and contrast-enhanced MR imaging. T1-weighted MR
imaging is not helpful in differentiating these tumors from transitional cell
carcinoma.
Most patients with carcinosarcoma of the urinary bladder have an advanced
stage of the disease at the time of initial diagnosis. A relationship between
malignant mixed mesodermal tumor occurrence and previous irradiation has been
documented for the female genital tract
[12]. All patients described
in this article had muscle invasive tumors or a higher stage at the time of
initial diagnosis. None of our patients received radiation therapy before
diagnosis of carcinosarcoma.
Radical cystectomy seems to be the treatment of choice for patients with
carcinosarcoma of the urinary bladder, although patients tend to develop local
recurrence after surgery. Adjuvant radiotherapy and various combinations of
chemotherapy have yielded inconsistent results
[1]. Two of our patients had a
radical cystectomy and one had a partial cystectomy, and all three patients
had bilateral lymphadenectomies. Only one patient received chemotherapy after
surgery. All patients developed recurrent tumor 3–10 months after
surgery and died 1–3 months after recurrence.
In conclusion, we describe the MR imaging features of carcinosarcoma of the
urinary bladder with clinicopathologic correlation in three patients. Unlike
transitional cell carcinomas, carcinosarcomas are highly aggressive tumors,
with high recurrence rates and a poor prognosis. These tumors can present as
large single tumors or multiple small to medium-sized tumors. The tumors may
be polypoid or sessile and may arise from anywhere in the bladder. Location,
size, shape, and multiplicity do not help to differentiate carcinosarcomas
from transitional cell carcinomas. Dynamic gadolinium-enhanced imaging may be
helpful to differentiate these tumors from transitional cell carcinomas.
Unlike transitional cell carcinomas, carcinosarcomas do not show early strong
arterial enhancement, and their signal intensity can be heterogeneous on
T2-weighted imaging.
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Abstract
Introduction
