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Primary hepatic hemangioendothelioma in a patient with Budd-Chiari syndrome
  1. Preet Mukesh Shah1,
  2. Vijay Waman Dhakre2,
  3. Aabha Nagral1
  1. 1Department of Medical Gastroenterology, Jaslok Hospital and Research Centre, Mumbai, India
  2. 2Department of Surgical Gastroenterology, Jaslok Hospital and Research Centre, Mumbai, India
  1. Correspondence to Dr Vijay Waman Dhakre, vddrvijayd{at}


A 36-year-old woman was diagnosed with compensated cirrhosis of liver secondary to Budd-Chiari syndrome (BCS) and had undergone stenting of a thrombosed left hepatic vein. Eight months later, she presented with jaundice and right upper quadrant pain. CT revealed multiple focal lesions in the liver, which on biopsy proved to be hepatic hemangioendothelioma (HHE). Her liver disease and ascites progressively increased. Four months later, magnetic resonance cholangiopancreatography showed an advanced stage of HHE with infiltration of the common bile duct and vascular invasion with a blocked stent, with metastasis to the spleen and dorsolumbar vertebrae. We believe this is the first reported case of an HHE developing in the background of BCS.

  • liver disease
  • hepatic cancer
  • radiology
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  • Contributors PMS contributed in the writing of the case details, investigations and discussion, and provided histopathological and radiological photographs and figure legends. VWD made necessary corrections in the case report and formulated the discussion and references. AN contributed in the discussion and made significant corrections and finalisation of the complete draft.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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