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Case Reports
. 2018 Sep 28:2018:bcr2018225827.
doi: 10.1136/bcr-2018-225827.

Liver transplantation for inferior vena cava leiomyosarcoma: from a Maslow's hammer to the Occam's razor

Vasanthakumar Gunasekaran  1   2 , Ashwin Rammohan  1   2 , Srinivas Mettu Reddy  1   2 , Mohamed Rela  1   2   3
Affiliations
Case Reports

Liver transplantation for inferior vena cava leiomyosarcoma: from a Maslow's hammer to the Occam's razor

Vasanthakumar Gunasekaran et al. BMJ Case Rep. .

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.

Keywords: liver disease; surgical oncology; transplantation.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Contrast-enhanced CT abdomen showing leiomyosarcoma of the inferior vena cava (IVC), occluding the hepatic venous orifices causing Budd-Chiari syndrome (white arrow: tumour in the IVC). (A) Coronal view. (B) Sagittal view. (C) Axial view.
Figure 2
Figure 2
Intraoperative images of en bloc excision of leiomyosarcoma with a segment of the inferior vena cava (IVC) along with total hepatectomy and a right lobe living donor liver transplantation. (A) En bloc tumour excision with total hepatectomy (white arrow). (B): IVC reconstruction with cryopreserved IVC vein graft forming a neocava (yellow arrow: neocava). (C) Right lobe implantation onto the neocava (yellow arrow: neocava).
See this image and copyright information in PMC

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