Bilateral leiomyosarcoma of the kidney with family history of kidney cancer.

Sarcomas make up 1%-2% of all malignant renal tumors in adults, and the incidence increases with advancing age. Renal sarcomas are less common, but more lethal than sarcomas of any other genitourinary site. The common clinical presentation of renal sarcomas in adults include a palpable mass, abdominal or flank pain, and hematuria, similar to those seen with large, rapidly growing renal cell carcinomas. Usually, radical nephrectomy remains the treatment of choice for these tumors, which exhibit an aggressive biological behavior and an unfavorable prognosis. We describe an unusual case of bilateral renal leiomyosarcoma in a 61-year-old white male. The patient also had an uncle who had bilateral kidney cancer. In addition, our patient presented with a pulmonary embolism, which is different from the classic presentation of leiomyosarcoma. The patient did not undergo surgery, as the tumor had also invaded the surrounding vasculature and was felt to be unresectable by the consulting surgeons. He was treated with gemcitabine and docetaxel chemotherapy, with stabilization of disease.