Parker L. Ellison, Jr., MD, Ira Willner, MD
Medical University of South Carolina, Charleston, SC
Introduction: While liver transplantation itself is an invasive and specialized procedure, often with an arduous medical course preceding the surgery; post-transplant life is rarely without complication. Permanent immune suppression introduces its own cohort of increased risks including unique infections, malignancies, and side effects of immunosuppressive or prophylactic therapies. Special consideration should be given to these patients when potential complications arise.
Case Description/Methods: A 51 year old male with a an orthotopic liver transplant 6 years prior due to NASH cirrhosis presents with rapid significant weight loss and worsening abdominal pain. MRI revealed development of T2 hyper-intense peripherally enhancing lesions throughout the liver, as well as massive splenomegaly with possible areas of splenic infarct. Transjugular liver biopsy was expedited and ultimately revealed epitheliod hemangioendothelioma (EHE). Initial infectious work up was unrevealing outside of EBV viral load returning weakly positive; however, given possible association with EHE with EBV, treatment was initiated with valgancyclovir. Immunosuppression was also adjusted from tacrolimus to everolimus given anti-neoplastic properties of mTOR inhibitors over calcineurin inhibitors. He was started on pazopanib as an outpatient given anecdotal evidence of VEGF inhibitors in treating EHE, however, treatment was complicated shortly thereafter by syncope and refractory TTP, and palliative care was ultimately pursued.
Discussion: Epithelioid hemangioendothelioma is a rare vascular tumor originating from mesenchymal cells. While risk of malignancy is increased post transplant due to immunomodulators, post transplant Hepatic EHE remains an exceedingly rare diagnosis. EHE is considered to have intermediate behavior between hemangioma’s and hemangiosarcoma’s however HEHE is metastatic at diagnosis in 50% of diagnoses. Common presenting symptoms include vague abdominal pain, weight loss, and weakness/fatigue. Histopathological examination via biopsy is the gold standard of diagnosis and positivity of factor VIII-dependent antigen, CD-34, and CD-31 are important. Course is variable however prognosis is poor and there is no standardized management plan. VEGF inhibitors including Bevacizumab and Pazopanib have been a target of therapy given histologic positivity for target receptors, however results have been mixed to date.
Citation: Parker L. Ellison, Jr., MD, Ira Willner, MD. P0734 - MALIGNANT HEPATIC EPITHELIOD HEMANGIOENDOTHELIOMA IN A PATIENT WITH A PRIOR LIVER TRANSPLANT. Program No. P0734. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.