Leiomyosarcoma of vascular origin: a rare presentation

Abstract

Malignant soft tissue neoplasm exhibiting smooth muscle differentiation is called leiomyosarcoma (LMS). LMS arising from the smooth muscles of tunica media of the major blood vessels like inferior vena cava (IVC) is a rare type of neoplasm, accounting for less than 0.5% of adult soft tissue sarcoma, affecting <1/1,00,000 of all adult malignancies. The prognosis of this tumor is poor, as patients often present with intra or extra-luminal growth often with invasion of adjacent structures. Only 400 cases of IVC LMS have been reported. Here we report a case of 40 year old female with large asymptomatic mass per abdomen of 3 months duration occupying the central and right part of abdomen. Investigations revealed a large retroperitoneal mass of 20.4*22.8*22.5 cms arising from the right kidney, likely Renal cell carcinoma. On exploration a huge tumor of size 25*25*20 cms was seen arising from retroperitoneum on right side, right kidney its vessels and ureter could not be seen separately. IVC could not be delineated. Histopathology report revealed it to be LMS of vascular origin. We report one such case because although incidence of such cases is low but should be considered in the differential diagnosis of mass per abdomen which grows unusually fast.