Adult renal sarcoma: clinical features and survival in a series of patients treated at a high-volume institution.
ABSTRACT To examine the clinical characteristics, treatment, and survival of adult patients with renal sarcoma treated at our institution during the past 2 decades.
A retrospective review of the demographic, presentation, treatment, and outcome data for 41 adult patients with renal sarcoma treated at our institution from January 1989 to December 2009 was performed. The clinicopathologic parameters were analyzed to determine their effect on survival.
Of the 41 patients, 18 were women and 23 were men. Their median age was 42 years (range 19-76). The median tumor size was 13 cm (range 4-35); 29 cases (70.7%) were high grade. The predominant histologic subtype was leiomyosarcoma (39.0%). At diagnosis, 6 patients (14.6%) had metastatic disease. Surgical resection was performed in 34 patients (82.9%), with negative margins in 22 (53.7%). After a median follow-up of 24 months (range 3-80), 3 patients (8.1%) had survived disease free, 11 (29.7%) were alive with disease, and 23 (62.2%) had died of disease. The overall 1-, 3-, and 5-year survival rate was 86.3%, 40.7%, and 14.5%, respectively, and the median survival was 28 months. The median survival after recurrence was 10 months (range 4-24) and that after metastasis 8 months (range 0-22). On univariate analyses, nonmetastatic disease (P = .001) and surgical resection (P = .000) were predictive of a favorable outcome. On multivariate analyses, surgical resection was the only independent predictor of survival (hazard ratio 35.629, P = .022).
Adult renal sarcoma accounts for 0.8% of renal cancer cases and has a poor prognosis. Early diagnosis and surgical resection offer patients the best chance of survival.
- [show abstract] [hide abstract]
ABSTRACT: Primary renal sarcomas in adults are rare and unusual neoplasms. This study was performed to better define the natural history and current management of these sarcomas in a typical medical setting in the United States. The hospital records of 4018 adult patients with renal neoplasms treated in the state of Illinois from 1975 to 1985 were examined by American Cancer Society professional volunteers. A primary renal sarcoma occurred in 34 patients (0.8% incidence). Eleven adult patients had Wilms tumor, 21 had primary renal sarcoma (47% leiomyosarcoma), and 2 were not found to have sarcoma on review. The median age of the patients with Wilms tumor was 30 years, whereas that of the patients with non-Wilms sarcoma was 65 years. Four of the patients with Wilms tumor (36%) are long-term survivors and all received adjuvant chemotherapy after radical nephrectomy. Six of the patients with non-Wilms sarcoma (29%) are long-term survivors after radical nephrectomy alone. Primary renal sarcomas, when treated with radical nephrectomy and, in the case of Wilms tumor, adjuvant chemotherapy, appear to be curable in 29-36% of cases. Histologic review of patients younger than 40 years of age with renal neoplasia is recommended.Cancer 03/1993; 71(3):804-10. · 5.20 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: Non-urothelial bladder tumors frequently present a diagnostic and therapeutic challenge. We review the peer-reviewed literature to summarize the available evidence on the etiology, diagnosis and optimal management of malignant non-urothelial bladder tumors. A comprehensive MEDLINE database search was performed. In addition, the proceedings of recent national and international urological and cancer society meetings were reviewed. Primary non-urothelial bladder tumors are rare in Europe and North America representing less than 5% of all bladder lesions combined. A large number of risk factors have been implicated in the etiology of non-schistosomiasis-related squamous cell carcinoma, yet their exact pathomechanism remains poorly defined. Squamous cell carcinoma, adenocarcinoma, small cell carcinoma, sarcoma and carcinosarcoma/sarcomatoid tumors share an unfavorable prognosis despite aggressive surgical management that relates both to an aggressive biological behaviour as well as to an often times advanced stage at the time of diagnosis. Inflammatory pseudotumors are benign tumors of uncertain histogenesis that may mimic sarcomas. Paraganglioma, primary melanoma and lymphoma represent additional, exceedingly rare bladder tumors. The systematic investigation of most non-urothelial bladder tumors is limited by the rarity of these lesions. A concerted effort of multiple institutions linked together in a national or international tumor registry will be necessary to advance our understanding of these tumors, evaluate treatment strategies and optimize patient outcome in the future.European Urology 01/2004; 44(6):672-81. · 10.48 Impact Factor