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      Sarcomatoid Variant of Urothelial Carcinoma (Carcinosarcoma, Spindle Cell Carcinoma): A Review of the Literature

      review-article
      1 , * , 1 , 2
      ISRN Urology
      Hindawi Publishing Corporation

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          Abstract

          Background. Sarcomatoid variant of urothelial carcinoma (SVUC) was added to the WHO classification in 2004. Aims. To review the literature. Materials and Method. Various internet databases were used. Result. SVUCs are rare biphasic malignant neoplasms exhibiting morphologic/immunohistochemical evidence of epithelial and mesenchymal differentiation with the presence or absence of heterologous elements. Some cases of SVUC have been associated with radiation therapy and cyclophosphamide treatment. Patients' ages range from 50 to 77 years (mean age 66). Patients tend to be younger and they more commonly presented with high-grade histology and advanced stage disease, in comparison with patients who had conventional urothelial carcinoma (CUC). Results of molecular/genetic studies strongly argue for a common monoclonal cell origin of both the epithelial and mesenchymal components in SUVC. The cancer specific survival of SVUC is poor in comparison with CUC. Radical surgical excision and chemoradiation may be associated with improved prognosis; chemoradiation as an organ preserving alternative to radical excision may be associated with improved outcome. There is no consensus opinion on the best treatment modalities for SUVC. Conclusions. SVUC is rare and is associated with inferior outcome compared with CUC. A multicentre trial of various treatment options is required. Cases of SVUC should be reported.

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          Histological variants of urothelial carcinoma: diagnostic, therapeutic and prognostic implications.

          It is well established that invasive urothelial carcinoma, involving the urinary bladder and renal pelvis, has marked propensity for divergent differentiation. In recent years, several 'variant' morphologies have been described and most have been recognized in the 2004 World Health Organization Classification. These histological variants of urothelial carcinoma have clinical significance at various levels, including diagnostic, that is, awareness of the morphological variant is essential in order to avoid diagnostic misinterpretations; prognostic for patient risk stratification; and therapeutic, where a diagnostic assignment of a particular variant may be associated with the administration of a therapy distinctive from that used in conventional invasive urothelial carcinoma. The diagnoses of micropapillary urothelial carcinoma, small-cell carcinoma, lymphoepithelioma-like carcinoma and sarcomatoid carcinoma are prime examples where treatment protocols may be different than the usual muscle-invasive bladder cancer. This review discusses the variants of urothelial carcinoma, outlining for each the diagnostic features, differential diagnostic considerations and the clinical significance.
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            Differences in survival among patients with sarcomatoid carcinoma, carcinosarcoma and urothelial carcinoma of the bladder.

            Sarcomatoid carcinoma and carcinosarcoma of the bladder are rare tumors that contain epithelial and mesenchymal elements, and may portend a worse prognosis than conventional urothelial carcinoma. We investigated the survival of patients with the 2 tumor subtypes compared to survival in those with urothelial carcinoma. Cases of sarcomatoid carcinoma, carcinosarcoma and high grade urothelial carcinoma of the bladder were identified from the Surveillance, Epidemiology and End Results Program. Demographic and pathological characteristics were compared. Differences in survival based on histological subtype were estimated using Kaplan-Meier analysis and multivariate Cox proportional hazards regression. Overall unadjusted survival rates for 46,515 patients with urothelial carcinoma, 135 with sarcomatoid carcinoma and 166 with carcinosarcoma were 77%, 54% and 48% at 1 year, and 47%, 37% and 17% at 5 years, respectively. Sarcomatoid carcinoma and carcinosarcoma presented at a similar age but at a higher T stage and with more frequent regional and distant metastases compared to urothelial carcinoma. On multivariate analysis patients with sarcomatoid carcinoma (HR 1.18, 95% CI 0.91-1.52) and carcinosarcoma (HR 2.00, 95% CI 1.65-2.41) were at higher risk for death compared to those with urothelial carcinoma. Overall mortality was worse with carcinosarcoma than with sarcomatoid carcinoma (HR 1.70, 95% CI 1.23-2.34). Compared to patients with urothelial carcinoma those with sarcomatoid carcinoma and carcinosarcoma present at a more advanced stage and are at greater risk for death even after adjusting for stage at presentation. The survival rate of sarcomatoid carcinoma is better than that of carcinosarcoma, offering some justification for the continued differentiation of these tumor types for clinical prognostication.
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              Carcinosarcoma and sarcomatoid carcinoma of the bladder: clinicopathological study of 41 cases.

              Carcinosarcoma of the bladder is a rare neoplasm characterized by an intimate admixture of carcinoma and malignant soft tissue neoplasm. The clinical usefulness of separating carcinosarcoma (carcinoma with sarcomatous component) from sarcomatoid carcinoma (carcinoma with spindle cell carcinomatous component) is uncertain, and it comprises the subject of this report. We reviewed the clinical and pathological records of 10 men and 5 women a mean of 66 years old with carcinosarcoma, and 21 men and 5 women a mean of 66.5 years old with sarcomatoid carcinoma of the bladder, as documented in the files of the Mayo Clinic between 1936 and 1995. Of the 15 patients in the carcinosarcoma group 9 had urothelial carcinoma, small cell carcinoma, 3 had squamous cell carcinoma and 2 had more than 1 type. The sarcomatous component included chondrosarcoma in 3 cases, leiomyosarcoma in 3, malignant fibrous histiocytoma in 3, osteosarcoma in 2, fibrosarcoma in 1, rhabdomyosarcoma in 1 and more than 1 type in 2. All disease was high stage at presentation. Treatment included cystectomy in 11 patients with (4) and without (7) radiation therapy, and transurethral resection in 4 with (1) and without (3) radiation therapy. Mean followup available in 14 cases was 34 months (range 1 to 144). A total of 11 patients died of cancer at 1 to 48 months (mean 17.2) and 2 survived for 8 to 131 months. Of the 26 patients in the sarcomatoid carcinoma group 18 had urothelial carcinoma, 1 had squamous carcinoma, 2 had urothelial carcinoma combined with squamous cell carcinoma and 5 had spindle cells only with no recognizable epithelium. All but 1 case was high stage at diagnosis. Treatment included transurethral resection in 17 patients with (7) and without (10) radiation therapy, including 1 who also received chemotherapy, and only cystectomy in 5, including 2 who also underwent radiation therapy and 1 who also received chemotherapy. Mean followup available in 21 cases was 49 months (range 1 to 420). A total of 17 patients died of cancer at 1 to 73 months (mean 9.8), 1 was alive at 140 months and 3 died of unrelated causes. Carcinosarcoma and sarcomatoid carcinoma of the bladder are highly aggressive malignancies with a similar outcome regardless of histological findings and treatment. Pathological stage is the best predictor of survival.
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                Author and article information

                Journal
                ISRN Urol
                ISRN Urol
                ISRN.UROLOGY
                ISRN Urology
                Hindawi Publishing Corporation
                2090-5807
                2090-5815
                2014
                22 January 2014
                : 2014
                : 794563
                Affiliations
                1North Manchester General Hospital, Department of Urology, Delaunays Road, Crumpsall, Manchester, UK
                2Royal Oldham Hospital, Department of Histopathology, Rochdale Road, Oldham, UK
                Author notes
                *Anthony Kodzo-Grey Venyo: akodzogrey@ 123456yahoo.co.uk

                Academic Editors: J. H. Ku and V. Serretta

                Article
                10.1155/2014/794563
                3920806
                24587922
                c959a5bd-ddc0-4b25-9132-334cf6742421
                Copyright © 2014 A. K.-G. Venyo and S. Titi.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 23 September 2013
                : 9 November 2013
                Categories
                Review Article

                Urology
                Urology

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