Carcinosarcoma of the urinary bladder following cyclophosphamide therapy: evidence for monoclonal orgin and chromosome 9p allelic loss
We report a case involving a 45-year-old man with a 12-year history of Wegener granulomatosis, who developed a carcinosarcoma of the urinary bladder after long-term cyclophosphamide therapy. Cyclophosphamide is well recognized as an etiologic agent for urothelial carcinoma of the urinary bladder. However, only 5 cases of carcinosarcoma of the urinary bladder following cyclophosphamide therapy have been reported. We used loss of heterozygosity studies and microsatellite markers to define the molecular basis of this rare neoplasm. These studies revealed evidence supporting a monoclonal origin for the 2 components of this tumor. We also demonstrated allelic loss of chromosome 9p. This loss associated with carcinosarcoma of the urinary bladder is in agreement with previous studies, suggesting a possible role for the tumor suppressor gene p16 in the pathogenesis of this tumor.
Available from: Dogan Atilgan
- "The mesenchymal element of carcinosarcoma lacks epithelial markers [2, 4], and patients with carcinosarcoma present at a more advanced stage and are at greater risk for death compared to patients with high-grade urothelial carcinoma . In cases of bladder carcinosarcoma, evidence supporting a monoclonal origin for the epithelial and mesenchymal components was revealed using loss of heterozygosity studies with microsatellite markers [5, 6], while there exists a hypothesis that multiclonal stem cells of the epithelial and mesenchymal components play a causative role. It is also known that bladder carcinosarcoma develops following cyclophosphamide therapy  as well as radiotherapy to the bladder . "
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ABSTRACT: Carcinosarcoma of the urinary bladder is a rare neoplasm that is composed of malignant epithelial and mesenchymal components. In these tumors, histogenesis and biological behaviour remain controversial. Approximately 70 cases have been reported in the literature, usually as case reports or a small series. A series of 221 cases using the Surveillance, Epidemiology and End Results (SEER) Program database has been reported recently. Optimal treatment is uncertain. Herein, we report a case of sarcomatoid carcinoma of urinary bladder of a farmer aged 84 years old with a year history of hematuria and dysuria. A transurethral resection of the tumor (TUR-T) revealed a carcinosarcoma. The patient underwent radical cystectomy, and there is no tumor recurrence for 15 months after treatments.
Available from: Akiko Tonooka
- "In cases of bladder carcinosarcoma, evidence supporting a monoclonal origin for the epithelial and mesenchymal components was revealed using loss of heterozygosity studies with microsatellite markers  , while there exists a hypothesis that multiclonal stem cells of the epithelial and mesenchymal components play a causative role. It is also known that bladder carcinosarcoma develops following cyclophosphamide therapy   as well as radiotherapy to the bladder . Here we report an interesting case of bladder carcinosarcoma which was more than 10 cm in diameter, nevertheless it was without submucosal invasion. "
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ABSTRACT: Carcinosarcoma is a rare biphasic neoplasia containing both malignant mesenchymal and epithelial elements. Bladder carcinosarcoma commonly presented as high-grade, advanced stage, and aggressive behavior with a poor prognosis. An 83-year-old male presented with painless gross hematuria to our hospital. Cystoscopy revealed massive nonpapillary bladder tumor on the right wall. The 91 g tumor could be completely removed with transurethral resection. Histology of the tumor was diagnosed as carcinosarcoma with no submucosal invasion composed of biphasic malignant epithelial and mesenchymal cells. Epithelial malignancy was urothelial cancer and mesenchymal one was chondrosarcoma and leiomyosarcoma. The specimens taken at the second-look TUR-Bt revealed that carcinoma in situ (urothelial cancer) but not sarcoma existed at the mucosa surrounding the previous tumor site. 80 mg of BCG instillation intravesically every week for six weeks was successfully administered to the patient. There is no tumor recurrence for 6 months after treatments.
Available from: George Hemstreet
- "In most cases, the epithelial component consists of highgrade transitional cell carcinoma with possible epidermoid and/or glandular differentiation, while the heterologous component consists of chondrosarcoma, malignant fibrous histiocytoma, osteosarcoma, leiomyosarcoma, fibrosarcoma, or rhabdomyosarcoma    . Clinically, carcinosarcomas occur more commonly in older males, and present as advanced stage, rapidly growing polypoid neoplasms      . The histogenesis of carcinosarcomas remains a matter of controversy       . "
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ABSTRACT: Background. Urinary bladder sarcomatoid carcinoma (carcinosarcoma) is rare. The objective of this study was to examine the epidemiology, natural history, and prognostic factors of urinary bladder carcinosarcoma using population-based registry.
Methods. The Surveillance, Epidemiology, and End Results (SEER) Program database was used to identify cases by tumor site and histology codes. The association between clinical and demographic characteristics and long-term survival was examined.
A total of 221 histology confirmed cases were identified between 1973 and 2004, this accounted for approximately 0.11% of all primary bladder tumors during the study period. Median age of the patients was 75 years (range 41–96). Of the patients with a known tumor stage (N = 204), 72.5% had a regional or distant stage; 98.4% of patients with known histology grade (N = 127), had poorly or undifferentiated histology. Multiple primary tumors were indentified in about 40% of study subjects. The majority of patients (95.9%) received cancer directed surgery, 35.8% had radical or partial cystectomy, 15.8% of patients received radiation therapy combination with surgery. The median overall survival was 14 months (95% CI 7–21 months). 1-, 5-, and 10-year cancer specific survival rate were 53.9%, 28.4% and 25.8%. In a multivariate analysis, only tumor stage was found to be a significant prognostic factor for disease-specific survival.
Conclusions. Urinary bladder carcinosarcoma commonly presented as high grade, advanced stage and aggressive behavior with a poor prognosis. Emphasis on early detection, including identification of risk factors is needed to improve the outcome for patients with this malignancy.
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