Sarcomatoid Carcinoma in the Urinary Bladder

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					전남의대학술지 제46권 제2호
Chonnam Medical Journal
Vol. 46, No. 2, pp. 121∼124
DOI: 10.4068/cmj.2010.46.2.121

                             Sarcomatoid Carcinoma in the Urinary Bladder

              Dong Hoon Yoo, Woo Huck Jang, Joo Young Yim, Mu Yeal Seo and Seong Woon Park*

                                   Department of Urology, Kwangju Christian Hospital, Gwangju, Korea

          Sarcomatoid carcinoma of the urinary bladder is a rare malignant neoplasm in which the tumor consists
       of both carcinomatous and sarcomatous elements. In Korea, only 1 case of bladder and renal pelvis
       primary sarcomatoid carcinoma and 4 cases of carcinosarcoma involving the bladder have been reported.
       Recently, we experienced a case of sarcomatoid carcinoma of the bladder in a 78-year-old male patient
       experiencing severe gross hematuria. An ultrasound demonstrated a broad-based tumor 4.5×3.6 cm in size
       on the lateral side of the right ureteral orifice in the bladder dome. Under the diagnosis of a bladder tumor,
       transurethral resection of the bladder tumor (TURBT) was performed. A subsequent histological
       examination of the biopsy specimens showed both malignant mesenchymal and epithelial elements. As a
       result, the tumor was pathologically confirmed as a primary sarcomatoid carcinoma of the urinary bladder.

       Key Words: Urinary bladder; Carcinoma; Carcinosarcoma

                         Introduction                                           toid carcinomas are typically malignant with a very fast
                                                                                growth velocity, such that once diagnosed, most cases
  In the urinary tract, sarcomatoid carcinoma is a very                         have a poor prognosis because the patients have already
rare malignant neoplasm. The tumor consists of                                  advanced to a stage at which the carcinoma has already
carcinomatous tissues originating from epithelium cells                         invaded the surrounding tissues. The purpose of this
and multiform cells originating from the mesenchymal                            report is to present a case of sarcomatoid carcinoma in
components of either poorly differentiated or spindle                           the bladder, in which a 78-year-old male patient was
cells. The tumor cells have specific characteristics that                       diagnosed with a painless gross hematuria that developed
indicate that they are mixed within their own border.                           in the right lateral wall of the bladder. We also review
Sarcomatoid carcinoma is known to occur in only 0.3%                            the related literature.
of total bladder cancers; according to domestic reports
(Korea), one case was reported as a sarcomatoid carcin-
oma confined in the bladder, and other cases included                                                Case Report
sarcomatoid carcinomas confined in the pelvis of the
kidney or in the diverticulum in the bladder.2 Sarcoma-                           A 78-year-old male patient had a history that
                                                                                included painless, severe gross hematuria that began 2
Received: March 22, 2010, Accepted for Publication: June 8, 2010                weeks before his diagnosis. He was thus referred to our
*Corresponding author: Seong Woon Park, 503-715, Department of Urology,
 Kwangju Christian Hospital, Phone: +82-62-650-5171, FAX: +82-62-650-           hospital on the basis of a kidney and bladder ultrasound
 5174, E-mail:                                                 that was performed at a local clinic. During the initial

122 전남의대학술지 제46권 제2호 2010

evaluation at the clinic, a 2.5×1.6 cm irregular hypere-                  examinations all fell within the normal range. The gross
choic mass was found on the right lateral bladder wall,                   hematuria continued, and as a result, the patient's
and the patient was therefore referred to our institution.                blood pressure decreased to 80/50 mmHg. Emergency
  No specific symptoms or signs were noted in the                         TURBT was then performed under the impression that
initial review of systems or in the physical examination,                 the    patient     had   bladder        cancer.   A   cystoscopic
with the exception of the severe gross hematuria, and                     examination revealed a broad-based tumor 4 cm in
the patient did not have a specific past surgical or                      diameter on the lateral side of the right ureteral orifice.
medical history. His serum creatinine, Hb, and platelet                   The mass was revealed to be a non-papillary, large,
values were 1.4 mg/dl, 9.2 g/dl, and 94,000/ml, respec-                   bulky tumor having a necrotic tumor surface. Despite
tively, with 10∼19 RBC/HPF and 20∼29 WBC/HPF                              several attempts, we could not perform a complete
obtained from a urine analysis. Other laboratory                          resection because of the danger of bladder perforation.
                                                                          As such, we subsequently controlled the bleeding and
                                                                          finished the surgery. Microscopically, the sizes of the
                                                                          cancer cells were relatively uniform and dense. The
                                                                          shape of these cells generally looked like whirlpools in a
                                                                          pyramid-shaped bundle, although some cells were
                                                                          composed      of    epithelioid    cells    or    irregular-shaped
                                                                          multiform cells. Under high magnification, a minimum
                                                                          of 3 mitosis periods were observed and the cancer was
                                                                          considered       malignant      (Fig.      1).    A   subsequent
                                                                          immunohistochemical examination showed that the
                                                                          area of the epithelium component was positive for
                                                                          cytokeratin, one of the characteristics of carcinoma;
                                                                          also, the nonepithelial area tested positive for vimentin,
Fig. 1. Microscopic findings showing a mixture of carcinomatous
                                                                          an indicator of sarcoma. Therefore, we determined that
and sarcomatous elements. The tumor contains spindle-shaped
cells (H&E, ×400).                                                        the cancer was a sarcomatoid carcinoma (Fig. 2).

Fig. 2. Immunohistochemical staining for (A) cytokeratin and (B) vimentin (×200). The images reveal the diffuse cytoplasmic positivity in the
tumor cells of the sarcomatoid area.
                                                       Dong Hoon Yoo, et al: Sarcomatoid Carcinoma in the Urinary Bladder 123

                                                                       ently found in transitional cell carcinoma, adenocar-
                                                                       cinoma, or in an admixture of these. As such, the
                                                                       tumor has been previously described under a variety of
                                                                       names, such as sarcomatoid carcinoma, pseudosarcoma,
                                                                       malignant mixed mesodermal/Mullerian tumor, metapla-
                                                                       stic carcinoma, and spindle cell carcinoma.2 Sarcomatoid
                                                                       carcinoma is a cancer that has the appearance of the
                                                                       prominent spindle cells and in most cases occurs in the
                                                                       uterus, although it also occurs in the breasts, ovaries,
                                                                       urinary system, oral cavity, and esophagus.         The carci-
Fig. 3. After transurethral resection of the bladder tumor (TURBT),    nosarcoma is classified as a mosaic condition of totally
the CT scan shows the irregular thickening of the right lateral wall   different epithelial cell elements and sarcomatoid cell
and perivesical haziness.
                                                                       elements; if the carcinosarcoma has characteristics of
                                                                       dysmorphic differentiation into the cartilage and skeletal
During the stage work-up after the operation, an                       muscle, it can be considered a carcinosarcoma. However,
abdomen-pelvic computed tomography scan showed a                       carcinomatous sarcoma and sarcomatoid carcinoma
remnant 4.0×1.5 cm-sized hypervascular mass on the                     have similar clinical courses, and no histological or
right lateral wall of the bladder and multiple lymph                   clinical differences have been shown between these two
node metastases (Fig. 3). In addition, the whole-body                  classifications of cancer. Hence, the clinical usefulness
bone scan showed multiple bone metastases. The                         of separating carcinosarcoma from sarcomatoid sarcoma
patient had gross hematuria, making voiding difficult,                 (carcinoma with spindle cell carcinoma component) is
and displayed general weakness only 2 months after                     uncertain. This tumor predominantly occurs in male
surgery. Based on these symptoms, he visited the                       smokers; the male:female ratio is approximately 3:1
hospital as an outpatient, and with his family made                    with a mean onset age of 72 years (49 to 88),8 and the
arrangements for home hospice care. He expired 1                       most common presenting symptom is gross hematuria,
month after initiating home care.                                      similar to that in transitional cell carcinomas.9 The
                                                                       predominant location of carcinosarcomas is the bladder
                                                                       base, followed by the trigone and lateral walls. Most
                         Discussion                                    tumors present as a single, large polypoid mass, ranging
                                                                       in diameter from 1.5 cm to 12 cm.1 Grossly, these
  Sarcomatoid carcinoma of the urinary bladder is a                    tumors tend to be polypoid and ulcerated, and most
very rare tumor. Although more than 70 cases of                        patients with carcinosarcoma of the urinary bladder
primary sarcomatoid carcinoma of the urinary bladder                   have an advanced stage of the disease, with muscularis
have been reported in the literature, only 1 case has                  mucosa involvement typically present at the time of
been previously reported in Korea.
                                                       This tumor      initial diagnosis.1 As a result, the diagnosis of sarcomatoid
contains both the sarcoma element from mesenchymal                     carcinoma using light microscopy alone can be difficult,
tumors and the carcinoma element from epithelial cells.                especially with the small fragments of biopsied specimens
The mesenchymal component may be expressed as a                        or undifferentiated spindle cell tumors without obvious
variety of histologic types, including chondrosarcoma,                 glandular differentiation. In other words, it is difficult to
leiomyosarcoma, osteogenic sarcoma, and rhabdomyo-                     distinguish sarcomatoid carcinoma from sarcomas that
sarcoma. The epithelial component may be most frequ-                   have more frequent spindle cell areas, such as carcino-
124 전남의대학술지 제46권 제2호 2010

sarcoma, leiomyosarcoma, malignant fibrous histiocytoma,           included in the differential diagnosis. The experience in
and malignant melanoma. To establish a more effective              this case emphasizes that sarcomatoid carcinoma in the
sarcomatoid        carcinoma    diagnosis,    the   sarcomatous    urinary bladder should be managed as a highly aggressive
component should show obvious epithelial differentiation           tumor with poor outcome requiring more aggressive
with    no     heterogeneous      mesenchymal       components.    therapy.
Carcinosarcoma of the urinary bladder is an aggressive
neoplasm that has a mortality rate of approximately
50%. Lopez-Beltran et al. reported that in 26 cases of                                         References
sarcomatoid carcinoma originating in the bladder, 81%
of the patients died within 9.8 months. Most patients               1. Arenas LF, Fontes DA, Pereira EM, Hering FL. Sarcomatoid carcinoma
died either from a local disease or of lymph node, lung,               with osseous differentiation in the bladder. Int Braz J Urol 2006;32:
pleura, brain, liver, or bone metastases. Because of the
                                                                    2. Jeong YB, Park JK, Kim HJ, Moon WS. A case of sarcomatoid
aggressive nature of this neoplasm and its tendency for                carcinoma of the bladder. Korean J Urol 1999;40:252-5.
local recurrence, radical cystectomy seems to be the                3. Fromowitz FB, Bard RH, Koss LG. The epithelial origin of a
                                                                       malignant mesodermal mixed tumor of the bladder: report of cases
treatment of choice for patients with sarcomatoid                      with long-term survival. J Urol 1984;132:978-81.
carcinoma of the urinary bladder, although patients                 4. Iezzoni JC, Mills SE. Sarcomatoid carcinomas (carcinosarcomas) of the
often      tend     to   experience   local   recurrence   after       gastrointestinal tract: a review. Semin Diagn Pathol 1993;10:176-87.
        3,10                                                        5. Guarino M, Tricomi P, Giordano F, Cristofori E. Sarcomatoid carcin-
surgery.          Alternative treatments such as adjuvant              omas: pathological and histopathogenetic considerations. Pathology
radiotherapy and various combinations of chemotherapy                  1996;28:298-305.
(cisplatin and gemcitabine) have yielded inconsistent               6. Kang SG, Yoon CY, Cho JH, Yoon DK, Kim IS. Sarcomatoid
                                                                       carcinoma of the renal pelvis. Korean J Urol 2005;46:651-4.
results. Therefore, no specific guidelines regarding                7. Norchomoviz LE, Metwalli NE, Gupta P. The renal pelvis, ureter,
adjuvant therapy have been described. In the present                   urinary bladder, and urethra. In: Silverberg SG, DeLellis RA, Frable
                                                                       WS, eds. Principles and practice of surgical and cytopathology. 3rd ed.
case, the patient had a severe gross hematuria just 1
                                                                       New York: Churchill Livingston, 1997;235-85.
month after his operation, and died 1 month later. On               8. Lopez-Beltran A, Pacelli A, Rothenberg HJ, Wollan PC, Zincke H,
the basis of this experience, we confirm that sarcomatoid              Blute ML, et al. Carcinosarcoma and sarcomatoid carcinoma of the
                                                                       bladder: clinicopathological study of 41 cases. J Urol 1998;159:1497-503.
carcinoma indeed has an early metastasis with a high
                                                                    9. Perret L, Chaubert P, Hessler D, Guillou L. Primary heterologous
invasion of neighboring tissues. As a result, if a rapidly             carcinosarcoma (metaplastic carcinoma) of the urinary bladder: clinico-
growing tumor is detected that seems to originate from                 pathologic, immunohistochemical, and ultrastructural analysis of eight
                                                                       cases and a review of the literature. Cancer 1998;82:1535-49.
the kidney and extends to the ureter and bladder, even             10. Nuwahid F, German K, Campbell F, Stephenson T. Carcinosarcoma in
though it is rare, sarcomatoid carcinoma should be                     a bladder diverticulum. Urology 1994;44:775-8.

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