Purpose To study the overall survival and prognostic factors of patients with sarcomatoid renal cell carcinoma treated with nephrectomy and systemic therapy in the cytokine and targeted therapy eras. Materials and Methods This is a retrospective study of patients with sarcomatoid renal cell carcinoma who had nephrectomy and received systemic therapy at our center in the cytokine era (1987–2005) or the targeted therapy era (2006–2015). Multivariate regression models were used to determine the association of covariables with survival. Results Among 199 patients with sarcomatoid renal cell carcinoma, 167 (83.9%) have died (median overall survival 16.5 months; 95% CI: 15.2, 20.9). Survival of patients with clear-cell histology was significantly longer vs. those with non-clear cell histology (p = 0.034). Patients with synchronous metastatic disease had a significantly shorter survival compared with patients with metachronous metastatic disease (median 12.1 months vs. 23.3 months; p = 0.0064). Biopsy of the primary tumor or a metastatic site could detect the presence of sarcomatoid features in only 7.5% of cases. Although a significant improvement in survival rate was observed in the first year for patients treated in the targeted therapy era (p = 0.011), this effect was attenuated at year 2, disappeared at years 3–5 after diagnosis, and was not evident in patients with poor risk features. Conclusions Patients with sarcomatoid renal cell carcinoma still have poor prognosis with no clear long-term benefit of targeted therapy, underscoring the need to develop more effective systemic therapies for these patients.

Background Lymph node (LN) metastases are associated with poor outcomes for patients with renal cell carcinoma (RCC). This study compared the survival outcomes of patients with stage III, node‐positive disease (pT123N1M0) and patients with stage III, node‐negative disease (pT3N0M0). Methods A database of 4652 patients with RCC of any histological subtype treated with surgery at The University of Texas MD Anderson Cancer Center from 1993 to 2012 was retrospectively assessed. A total of 115 patients with pT123N1M0 disease, 274 patients with pT3N0M0 disease, and 523 patients with pT123N0/xM1 disease were included. Overall survival (OS) and cancer‐specific survival (CSS) were estimated and compared between each cohort. Results Median OS and CSS times were significantly better for pT3N0M0 patients than pT123N1M0 patients (OS, 10.2 vs 2.4 years, P < .0001; CSS, not reached vs 2.8 years, P < .0001). Similar median OS and CSS times were noted for pT123N1M0 and pT123N0/xM1 patients (OS, 2.4 vs 2.4 years; P = .62; CSS, 2.8 vs 2.4 years; P = .10). In a multivariate analysis, tumor grade (hazard ratio [HR] for OS, 2.47; P < .0001; HR for CSS, 2.99; P < .0001) and pathologic LN involvement (HR for OS, 2.44; P < .0001; HR for CSS, 2.85; P < .0001) were associated with worse OS and CSS. Conclusions Among RCC patients classified with stage III disease, those with pT123N1M0 disease had significantly worse survival than those with pT3N0M0 disease. OS and CSS were similar for patients with pT123N1M0 disease and patients with pT123N0/xM1 disease (stage IV). If validated, these findings suggest that RCC patients with nodal disease should be reclassified as having stage IV disease.

There was a low rate of microscopic PSM in our large cohort of patients undergoing PN despite tumor complexity. Higher nephrometry score, bilateral tumors, and prior treated RCC independently predicted PSM which showed worse survival, recurrence and metastasis compared to patients with NSM.

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Renal cell carcinoma with osseous metaplasia and bone marrow elements is a relatively rare event in these tumors. We discuss pathological differential diagnosis for this tumor with a review of the literature on this unusual case.