Leiomyosarcoma of the inferior vena cava is an extremely rare tumor that is characterized by a poor prognosis and nonspecific symptoms, a fact that may delay the diagnosis for several years. The only therapeutic modality proven to prolong the survival of patients is total surgical resection of the tumor. In this study, the authors report the case of a 50-year-old patient with a diagnosis of leiomyosarcoma of the inferior vena cava, affecting the middle and distal thirds, who was submitted to surgical treatment.
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