Leiomyosarcoma (LMS) is a malignant smooth muscle tumor which is predominantly intra-abdominal in location. LMS arising from vascular smooth muscle is quite uncommon which usually arises from inferior vena cava and is aggressive in nature. Primary venous LMS of splenic vein is exceedingly rare and has unpredictable clinical course with complete surgical resection representing the only potentially curative treatment. We hereby report a case of primary splenic vein LMS in a 59-year-old male patient mimicking an adrenal tumor radiologically.
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