Sarcomatoid variant of urothelial carcinoma (carcinosarcoma, spindle cell carcinoma): a review of the literature.

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.