Sarcomatoid carcinoma of the urinary bladder: a clinicopathological study of 4 cases and a review of the literature.
ABSTRACT Sarcomatoid carcinoma (SC) of urinary bladder is a rare tumor exhibiting aggressive behavior. Here we are reviewing the pathologic and clinical characteristics of 4 consecutive cases of this rare tumor. Three out of 4 patients were males and one female. The median age was 72.8 years (range, 60-79 years). Patients underwent transurethral resection of bladder tumor and the diagnosis of bladder SC was established on the pathologic examination of the resected bladder tissue. Despite treatment all patients died within 22 months of the diagnosis of SC. SC of the bladder are true biphasic malignant neoplasm exhibiting morphologic and immunohistochemical evidence of epithelial and mesenchymal differentiation with the presence or absence of heterologous elements. The aggressive of the tumor precludes radical therapy whenever possible, since adjuvant therapy seems to have little effect.
- SourceAvailable from: Mustafa Yıldırım[Show abstract] [Hide abstract]
ABSTRACT: Background and Objectives: This study aimed to present the clinicopathological characteristics and treatment of patients with bladder carcinoma with sarcomatoid differentiation at our institution. Methods: Between 1995- 2009, 950 patients were followed-up for bladder carcinoma. Among them, 14 patients with sarcomatoid carcinoma were retrospectively reviewed, and their clinical, pathological features and treatment were recorded. Results: Median age of the patients was 65 years (range: 41-86 years), 12 (86%) being male and 2 (14%) female. All the patients presented with hematuria and 11 (88%) had a history of smoking. The tumor growth pattern was solid in 10 patients, papillary in 2, and mixed in 2. In all, 5 of the patients had urothelial carcinoma with sarcomatoid differentiation and 9 were diagnosed with sarcomatoid carcinoma. Five patients underwent radical cystectomy with ileal conduit surgery, 2 patients refused cystectomy, and 8 patients underwent re-TUR. Following diagnosis ,12 of the patients died in mean 10.7 months (range: 1-48 months). Conclusion: Urothelial carcinomas with sarcomatoid features are aggressive and are usually at advanced stage at the time of diagnosis. The outcomes of multimodal treatment are not satisfactory. Significant findings of the present study are that early diagnosis positively affect survival and that gemcitabine and cisplatin in combination can positively affect survival.Asian Pacific journal of cancer prevention: APJCP 11/2012; 13(11):5729-33. · 1.50 Impact Factor
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ABSTRACT: Adult rhabdomyosarcoma (RMS) in the urinary bladder is rare, and is the subject of case reports and small series. It consists of sheets of small round blue cells with high nuclear cytoplasmic ratio, brisk mitosis and apoptosis. In this study, we reported one case of pure rhabdomyosarcoma and two cases of urothelial carcinomas with extensive rhabdomyosarcomatous differentiation. In addition, their immunohistochemical profile was compared to that of small cell carcinoma of the bladder. Our study showed that sufficient sampling was critical for the diagnosis of urothelial carcinoma with extensive rhabdomyosarcomatous differentiation. As adult RMS in the bladder and urothelial carcinoma with rhabdomyosarcomatous differentiation shared morphological features with small cell carcinoma of the bladder, appropriate immunohistochemical stains were necessary in the differential diagnosis. We showed both rhabdomyosarcoma and rhabdomyosarcomatous areas of the urothelial carcinoma were positive for myogenin, negative for cytokeratin and chromogranin stains. In contrast, small cell carcinoma was positive for cytokeratin, and 7 out of 9 cases were also positive for chromogranin. Both rhabdomyosarcoma and small cell carcinoma could be positive for synaptophysin, a potential pitfall to avoid. In addition, all of the tumors with rhabdomyosarcomatous differentiation were negative for FKHR rearrangement.Diagnostic Pathology 01/2011; 6:66. · 2.41 Impact Factor
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ABSTRACT: A 57-year-old man who presented with urinary retention was found to have a sarcomatoid carcinoma of the urethra. Evaluation with CT scan of the abdomen and pelvis revealed multiple pulmonary nodules and osteolytic lesions of left posterior ribs. After external beam radiation therapy and six cycles of systemic chemotherapy, patient underwent a surgical resection of the urethral cancer. After his surgery, patient was also found to have multiple brain metastases and underwent whole brain radiation therapy, nine months after his initial diagnosis. Sarcomatoid carcinomas of the genitourinary tract are extremely rare tumors that require a very aggressive, multimodal treatment approach.Case reports in urology. 01/2013; 2013:931893.
Korean Journal of Urology
Ⓒ The Korean Urological Association, 2010
Korean J Urol 2010;51:724-728
Sarcomatoid Carcinoma of the Urinary Bladder: A
Clinicopathological Study of 4 Cases and a Review of the Literature
Konstantinos Stamatiou, Nikolaos Galariotis, Ioannis Michailidis, Nerantzoula Petrakopoulou1,
Helen Moustou1, Adamantia Zizi-Sermpetzoglou1
Departments of Urology and 1Pathology, Tzaneio General Hospital of Pireas, Pireas, Greece
Sarcomatoid carcinoma (SC) of urinary bladder is a rare tumor exhibiting aggressive
behavior. Here we are reviewing the pathologic and clinical characteristics of 4 consec-
utive cases of this rare tumor. Three out of 4 patients were males and one female. The
median age was 72.8 years (range, 60-79 years). Patients underwent transurethral re-
section of bladder tumor and the diagnosis of bladder SC was established on the patho-
logic examination of the resected bladder tissue. Despite treatment all patients died
within 22 months of the diagnosis of SC. SC of the bladder are true biphasic malignant
neoplasm exhibiting morphologic and immunohistochemical evidence of epithelial and
mesenchymal differentiation with the presence or absence of heterologous elements.
The aggressive of the tumor precludes radical therapy whenever possible, since ad-
juvant therapy seems to have little effect.
Key Words: Transitional cell carcinoma; Urinary bladder neoplasms
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial
License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use,
distribution, and reproduction in any medium, provided the original work is properly cited.
received 24 May, 2010
accepted 13 July, 2010
Department of Urology, Tzaneio
General Hospital of Pireas, 0002
Salepoula Street, Pireas 18536,
Sarcomatoid carcinoma (SC) of the urinary bladder is a
very rare variant of transitional cell carcinoma (TCC) that
accounts for 0.3% of all histologic subtypes. Less than 100
cases have been reported in the literature until now .
Most cases have been reported as single case reports and
small series; only one study examining 41 consecutive cas-
es from a single institute exists in the literature .
This type of tumor is often termed “carcinosarcoma” (CS)
because of its carcinomatous and sarcomatous component.
Although the distinction between SC and CS has been ex-
tensively discussed in the past, the most recent World
Health Organization classification of urinary tract tumors
does not distinguish between SC and CS and uses the term
“sarcomatoid carcinoma” to denote all of these lesions .
The epithelial component may be TCC, squamous cell car-
cinoma, carcinoma in situ, small cell carcinoma, and ad-
enocarcinoma, whereas the sarcomatous component could
consist of leiomyosarcoma, chondrosarcoma, rhabdomyo-
sarcoma, and rarely liposarcoma. More than one type of
heterologous differentiation may be present .
In the present study, we present 4 cases of SC of the uri-
nary bladder that were diagnosed and treated in our in-
stitute and discuss the morphogenesis and histologic fea-
tures of this rare entity.
Four specimens of SC of the bladder were obtained between
1995 and 2009 and all were received via consultation with
one of the authors. The sections were fixed in 10% buffered
formalin, processed in the standard fashion, embedded in
paraffin, cut at 4 μm intervals, and stained with H&E.
Immunohistochemical staining was performed by the
standard immunoperoxidase technique. The antibodies
used included cytokeratin, AE1/AE3, 34BE12, vimentin,
muscle-specific actin (HHF-35), smooth-muscle-specific
actin, prostate-specific antigen (PSA), prostate alkaline
phosphatase (PSAP), monoclonal carcinoembryonic anti-
gen (mCEA), chromogranin, P63, CD31, and factor VIII.
Cases were scored for the percentage of sarcomatoid com-
ponent, necrosis, and heterologous elements.
1. Presentation of cases
1) Patient demographics and prior history: Three of the
Korean J Urol 2010;51:724-728
Sarcomatoid Carcinoma of the Urinary Bladder
FIG. 2. (A) A chondrosarcomatous area with atypical and pleomorphic chondrocytes (H&E, x200). (B) Transitional cell grade 3
carcinoma: epithelial component admixed with chondromatous areas (H&E, x100)
FIG. 1. (A) Transitional cell grade 3 carcinoma. Beneath that can be seen a spindle cell neoplasm with a sarcomatous appearance
(H&E, x100). (B) Immunoperoxidase stain with vimentin showing immunoreactivity in the spindle cell component (Vim, x100).
4 patients were males and one was a female. Their median
age was 72.8 years (range, 60-79 years), and most of the pa-
tients were older than 70 years. Two patients presented
with de novo bladder cancer. In one of them, the tumor was
found accidentally by abdominal ultrasound, whereas in
the remaining patient, the tumor was diagnosed by cysto-
scopy upon investigation of gross hematuria.
Two patients had a prior history of TCC of the urinary
bladder. In one of these two patients, the tumor was diag-
nosed by cystoscopy during regular follow-up, whereas in
the remaining patient, the tumor was diagnosed before the
scheduled follow-up cystoscopy.
All patients underwent transurethral resection of the
bladder tumor (TUR-BT) for diagnostic and staging pur-
poses (involvement of bladder muscolaris). The diagnosis
of bladder SC was established by the pathologic examina-
tion of the resected bladder tissue in all patients.
2) Detailed clinical features
(1) Case 1: A 76-year-old man with no prior history of
bladder cancer presented with gross hematuria. Upon ul-
trasound investigation, he was found to have a large blad-
der mass arising from the right lateral wall. He reported
a history of recurrent hematuria in the last 6 months.
Cystoscopy showed that the tumor protruded in the blad-
der lumen, covering the orifice of the right ureter. It had
a partly typical papillary appearance and was partly solid,
invading most of the right lateral wall. A computed tomog-
raphy (CT) scan of the abdomen revealed a mild dilatation
of the right renal pelvis and regional lymphadenopathy. No
metastatic disease was found.
(2) Case 2: A 77-year-old male smoker with a history of
recurrent non-muscle-invasive grade 3 urothelial carcino-
Korean J Urol 2010;51:724-728
Stamatiou et al
FIG. 4. (A) Sarcomatoid component of the tumor characterized by spindle cells with elongated and blunt nuclei (H&E, x100). (B)
Immunoperoxidase stain with cytokeratin showing immunoreactivity in the spindle cell component (Cyt, x100).
FIG. 3. (A) Spindle cell-like appearance of the sarcomatous component with numerous mitoses (H&E, x200). (B) Immunoperoxidase
stain with desmin showing immunoreactivity in some spindle cell components (Desm, x200).
ma and multiple TUR-BTs presented with gross hema-
turia and anemia. Cystoscopy showed the tumor to appear
as a solid mass located mainly in the bladder base. A CT
scan of the abdomen was suspicious for locally advanced
disease (invasion of the vescical fat). CT scans of the abdo-
men, chest, and brain as well as a bone scan were negative
for metastatic disease.
(3) Case 3: A 79-year-old man who was a heavy smoker,
suffering from diseases that seriously influenced his qual-
ity of life, and with a history of recurrent bladder cancer and
subsequent TUR-BTs was found to have a recurrent tumor
during follow-up cystoscopy. The radiologic evaluation was
not considered suspicious for metastatic disease.
(4) Case 4: A 60-year-old woman who presented with fre-
quency, urgency, and persistent dysuria was found to have
a suspicious lesion on abdominal ultrasound. Cystoscopy
showed a tumor arising from the bladder base. No regional
lymphadenopathy or metastatic disease was shown on a
3) Histologic features
(1) Case 1: The biopsy specimen had an overall volume
of 70 cc. It consisted partly of papillary and partly of solid,
transitional cell grade 3 carcinoma. Beneath that was a
spindle cell neoplasm with a sarcomatous appearance (Fig.
1A). The spindle cell component showed mild nuclear pleo-
morphism and severe mitotic activity. In immunohisto-
chemistry, the epithelial component was stained for P63.
The spindle cell component was positively stained for vi-
mentin (Fig. 1B) with a small subset of these cells being at
least focally positive for cytokeratin. The stains for HHF-
35, SMA, and desmin were negative.
(2) Case 2: The biopsy specimen had an overall volume
Korean J Urol 2010;51:724-728
Sarcomatoid Carcinoma of the Urinary Bladder
of 35 cc. It mainly consisted of sarcomatous elements that
had morphologic characteristics of chondrosarcoma (Fig.
2A). The epithelial component was a solid, grade 3 TCC
with large areas of necrosis (Fig. 2B). In immunohisto-
chemistry, the epithelial component was focally positively
stained for P63. The cartilaginous element was positively
stained for S100p.
(3) Case 3: The biopsy specimen had an overall volume
of 0.5 cc. The tumor was characterized by a biphasic pro-
liferation of malignant epithelial and sarcomatoid elements.
The epithelial component was a solid, invasive, grade 3
TCC. The sarcomatous component had a spindle cell-like
appearance and was characterized by severe nuclear pleo-
morphism. Mitoses were numerous (Fig. 3A). In immuno-
histochemistry, spindle cells were positively stained for vi-
mentin, whereas a small subset of these cells were pos-
itively stained for desmin (Fig. 3B). Focally, spindle cells
were positively stained for cytokeratin.
(4) Case 4: The biopsy specimen had an overall volume
of 3 cc. The tumor mainly consisted (up to 80%) of sarcoma-
tous elements with a spindle cell-like appearance (Fig. 4)
and a large areas of necrosis. Immunohistochemically, the
epithelial component was positively stained for cytoker-
atin and P63. Spindle cells were positively stained for vi-
mentin and negatively for actin and Melan A. A small sub-
set of these cells was positively stained for cytokeratin (Fig.
4) Patient treatment and outcomes: Patients unfit for
radical surgical treatment (cases 1, 2 and 3) were advised
to receive radiotherapy and adjuvant chemotherapy.
Case 1 patient received radiotherapy and adjuvant che-
motherapy consisted of 4 cycles of cisplatin, methotrexate,
vinblastine (CMV) regiment. Although he was alive with
CS at the point of final follow-up (18 months after diag-
nosis), he died 3 months later.
Case 2 patient refused any intervention and discharged
home. He died within 1 year of the diagnosis of SC.
Case 3 patient received radiotherapy only. Despite treat-
ment he developed metastatic disease to the sacrum and
liver 6 and 9 months after the diagnosis of SC respectively.
He was started chemotherapy however he died 1 month af-
ter the diagnosis of liver metastases.
The remaining patient (case 4) underwent radical
cystectomy. Although surgical margins were disease free
in pathology report she developed local recurrence involv-
ing rectum and liver metastases 8 months after surgery
and she died 6 months later.
Malignant tumors that display a biphasic pattern are rela-
tively unusual. They are more commonly observed in the
female genital tract but may also be encountered in other
locations, including the male genital system, lower and up-
per respiratory tract, and urinary tract . Although their
existence was recognised as early as the mid 19th century,
the exact histogenesis of these neoplasms remains a con-
troversial issue in tumor pathology. According to the most
widely accepted theories, these tumors may develop from
a pluripotent neoplastic cell or may be true collision tu-
mors, in which both malignant epithelial and mesen-
chymal components arise independently of each other
[4,5]. Histologically, the first theory is supported by the
presence of ultrastructural features (desmosomes or tono-
filaments) of epithelial differentiation in sarcomatoid
elements. Genetic and molecular studies argue for a mono-
clonal origin of both the epithelial and mesenchymal com-
ponents in SC of the urinary bladder . Interestingly,
Sung et al found that a subset of tumors displayed dis-
cordant allelic losses associated with advanced urothelial
carcinoma, which may reflect genetic divergence during
the clonal evolution of SC .
SCs of the bladder are true biphasic malignant neo-
plasms exhibiting morphologic and immunohistochemical
evidence of epithelial and mesenchymal differentiation
with the presence or absence of heterologous elements .
Microscopically, the sarcomatoid component is composed
of a urothelial glandular or small cell component showing
a variable degree of differentiation. The most common is
urothelial carcinoma (80%), followed by squamous cell car-
cinoma (32%), adenocarcinoma (26%), and small cell carci-
noma (5%). The mesenchymal component most frequently
observed is osteosarcoma (97%), followed by chondrosarcoma
(30%), rhabdomyosarcoma (20%), undifferentiated high-
grade spindle cell neoplasm (17%), leiomyosarcoma (7%),
liposarcoma, angiosarcoma, and other mixed types of mes-
enchymal differentiation . By immunohistochemistry,
the epithelial component reacts with cytokeratins. Mesen-
chymal cells react with vimentin or specific markers corre-
sponding to mesenchymal differentiation. SC does not pose
diagnostic difficulties to pathologists. In most cases, the di-
agnosis can be established by conventional H&E exami-
nation. Differential diagnosis should include pure sarco-
ma, particularly in cases composed exclusively of spindle
cells; leiomyosarcoma; carcinoma with pseudosarcoma-
tous stroma; and sarcomas with pseudoepitheliomatous
hyperplasia . Pseudosarcomatous stroma and other mor-
phologic forms of pseudosarcomatous proliferations (such
as inflammatory pseudotumors and postoperative spindle
cell nodules) are usually highly vascularized with numer-
ous small slit-like vessels. The cells show minimal re-
active-type atypia and atypical mitotic Figures are absent.
Pseudoepitheliomatous hyperplasia associated with sar-
coma of the urinary bladder can resemble the architecture
of malignant epithelial proliferation on low-power exami-
nation. More detailed examination shows a less intimate
association between the two components with a more
abrupt interface between true malignant sarcomatous ele-
ments and epithelial elements of purely hyperplastic
nature. According to the literature, these tumors occur pre-
dominantly in male smokers, with a mean age of 72 years.
Usual signs and symptoms include hematuria, dysuria,
and urinary tract infection [2,8]. Less often, SC arises in
patients with a history of radiotherapy, chemotherapy, or
Korean J Urol 2010;51:724-728
Stamatiou et al
conditions that cause cell replication abnormalities . In
our cases, the patients had no such history; however, a pro-
gression of the preexisting grade 3 urothelial carcinoma to
a sarcomatoid tumor at the time of diagnosis cannot be ex-
cluded (Case 2, 3).
The appropriate treatment for such rare tumors has not
yet been defined; however, the aggressive behavior sug-
gests radical therapy whenever possible . Total cys-
tectomy often followed by radiation therapy and/or chemo-
therapy seems to be the preferred treatment . The effec-
tiveness of these treatments is not known because of the
varying results of each case. The only factors predictive of
long-term survival are negative surgical margins and the
absence of metastatic disease at the time of presentation
. Unfortunately, cases with metastasis, such as our case
3, have a very poor prognosis. Further study with more cas-
es and experience would be of great value both pathologi-
cally and clinically.
Conflicts of Interest
The authors have nothing to disclose.
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