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.

Sarcomatoid Variant of Urothelial Carcinoma of the Renal Pelvis with Inferior Vena Cava Tumour Thrombus: A Case Report and Literature Review

“…Sarcomatoid variant of urothelial carcinoma is an aggressive but uncommon type of urothelial carcinoma with around 100 reported cases, an overwhelming majority of which arise in the urinary bladder [15–17]. In the literature, these tumours have also been designated as carcinosarcoma and spindle cell carcinoma [17].…”

“…In the literature, these tumours have also been designated as carcinosarcoma and spindle cell carcinoma [17]. Lopez-Beltran et al [6] stated that microscopically sarcomatoid urothelial carcinoma contains a urothelial, glandular, or small cell component with variable degrees of differentiation and that carcinoma in situ is found in 30% of these cases.…”

Cutaneous invasion from sarcomatoid urothelial carcinoma: clinical and dermatopathologic features

“…WHO acknowledges as main variants of the urothelial carcinoma: squamous, glandular differentiation, nests, microcystic, micropapilliferous, lymphoepithelioma-simile, lymphoma-simile and plasmocytoid, sarcomatoid (with and without heterologous elements), trophoblastic differentiation, clear cells and lipidic cells. 18 , 19 …”

“…The presence of a morphological variant is associated with a more aggressive biological behavior of urothelial carcinomas and may even reflect in the option for therapeutic conducts different from the customarily used in the conventional urothelial carcinoma. 4 , 19 , 20…”

“…A third theory points to the possibility of occurrence of metaplasia of malignant epithelial elements to sarcomatoid elements, based on the presence of staining for keratins (AE1/AE3) both in the epithelial component and in the mesenchymal component. 19 , 20…”