Primary renal sarcoma in adults. A natural history and management study by the American cancer society, Illinois division

• Published in: Cancer 1993-02, Vol.71 (3), p.804-810
• Main Authors: Vogelzang, Nicholas J., Fremgen, Amy M., Guinan, Patrick D., Chmiel, Joan S., Sylvester, Joanne L., Sener, Stephen F.
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Biological and medical sciences; Female; Humans; Incidence; Kidney Neoplasms - epidemiology; Kidney Neoplasms - pathology; Kidney Neoplasms - therapy; Kidneys; Male; Medical sciences; Middle Aged; Nephrology. Urinary tract diseases; renal sarcoma; Sarcoma - epidemiology; Sarcoma - pathology; Sarcoma - therapy; Tumors of the urinary system; Wilms tumor; Wilms Tumor - epidemiology; Wilms Tumor - pathology; Wilms Tumor - therapy
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/1097-0142(19930201)71:3<804::AID-CNCR2820710324>3.0.CO;2-A

Background. 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. Methods. 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. Results. A primary renal sarcoma occurred in 34 patients (0.8% incidence). Eleven adult patients had Wilms tumor, 21 had primary renal sarcoma (47% leio‐myosarcoma), 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 nephrec‐tomy. Six of the patients with non‐Wilms sarcoma (29%) are long‐term survivors after radical nephrectomy alone. Conclusions. 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.