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RESUMO
Leiomyosarcoma of the inferior vena cava is a rare condition and is usually fatal. Symptoms vary depending on the site of the lesion and whether the growth is mainly intra-luminal or extra-luminal. Obstruction of the lower part causes oedema of the lower limbs. Higher up it would present with features of Budd-Chiari Syndrome. Lesions where the growth is mainly extra-luminal present with vague symptoms of pain, loss of weight and later a mass. Surgery is the mainstay of treatment