Leiomyosarcomas arising from the renal vein are rare tumors:only 13 cases are found in the literature. We report a case in a sixty-one-year-old woman who presented with back pain and hematuria. She underwent resection of the tumor and an ipsilateral nephrectomy. The tumor recurred locally, and the patient died thirty months after diagnosis. Review of the cases reported in the literature reveals the aggressive nature of these tumors and the need for a combined therapeutic approach.
ASJC Scopus subject areas