Abstract
Leiomyosarcoma (LMS) of primary vascular origin is a rare entity with only potentially curative option being complete surgical resection; despite which the prognosis remains dismal. Tumour recurrence is very common, and the benefits of adjuvant therapy are undefined. A 39-year-old woman presented with 6 months' history of abdominal pain, abdominal distension and pedal oedema. On evaluation, she was diagnosed to have chronic Budd-Chiari syndrome (BCS) secondary to a tumour arising from the inferior vena cava (IVC) on evaluation. Her liver decompensation included jaundice, gastrointestinal bleed and ascites. Following a detailed multidisciplinary team discussion, she underwent complete excision of the tumour along with a segment of the IVC with living donor liver transplantation. She remains disease-free 24 months following surgery. This is the first reported case of liver transplantation for IVC LMS causing chronic BCS.