RESUMO
Leiomyosarcoma of inferior venacava is a rare tumor. Female are most commonly affected and middle segment of inferior vena cava is the commonest site. The diagnosis can sometimes be challenging as patients present with non-specific symptoms. We present a case of a 65-year-old female who presented with pain in right hypochondrium and epigastric region since 4 months along with weight loss, anorexia and vomiting. Histopathological examination revealed a capsulated, multilobated tumor arising from muscle layer of inferior venacava with extraluminal growth pattern and tumor cells showing cytoplasmic positivity for immuno-histochemical stain smooth muscle actin. With the diagnosis of leiomyosarcoma of inferior venacava, excision of tumor was done with tangential excision of 4 cm length of inferior venacava with primary repair. Keywords: inferior venacava; leiomyosarcoma; smooth muscle actin.