Primary renal vein leiomyosarcoma (LMS) is a rare tumor with only a few cases reported in the literature. Clinical diagnosis of renal vein LMS can be difficult because of nonspecific symptoms and nonpathognomonic radiological features. The primary treatment modality is radical nephrectomy followed by chemotherapy and/or radiotherapy. There is scarcity of literature regarding prognosis because of rarity of tumor; however, tumor size (>3 cm) determines the risk of local recurrence and distant metastasis. Overall prognosis of renal vein LMS is poor. Here, we describe clinical and histopathological features of a 50-year-old female patient with LMS of right renal vein.
Keywords: Leiomyosarcoma; Renal vein.
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