Leiomyosarcoma of the Inferior Vena Cava is an extremely rare malignant tumor arising from the smooth muscle lining of the vessel wall. They are usually seen in sixth decade with a female preponderance. The symptoms vary greatly from case to case. Exact location and size of the tumor within the vena cava determines the symptoms. Our patient was a 35 year old female who presented with right hypochondriac and lumbar region mass since 2 months. Our study highlights the role of imaging studies and histopathological examination in making an accurate diagnosis of this rare disease.
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