Hemangioendothelioma

Case Presentation 24 y/o WM with complaint of yellow eyes and skin for the prior few days Increasing abdominal girth Mental slowing Dark color to urine No fever/chills/diarrhea/abdominal pain/N/V No recent travel, unprotected sex, herbals/teas/supplements Vaccinated for HBV

History PMHx: None PSHx: Tonsillectomy Meds: None Allergies: None Soc Hx: No EtOH, tobacco, drugs Heterosexual, single Air Force pilot Fam Hx: No liver disease

Physical Examination General Ill-appearing HEENT Icterus CV RRR Chest Clear Abd +BS, soft, NT Liver enlarged, firm 2cm below costal margin Moderate ascites. Spleen not palpable Ext 2+ pitting edema BLE Neuro + asterixis Skin Jaundice. No spider/palmar erythema

Laboratories PT 19 sec/INR 1.6 Total bilirubin 28.0 mg/dL Creatinine 0.8 mg/dL MELD 24 AST 199 ALT 157 Alkaline phos 841 Platelet 157 Albumin 2.4 mg/dL Ammonia 206 µg/dL

Imaging U/S diffuse echogenic and hypoechogenic scattered areas throughout. CBD normal. No stones seen CT Chest multiple pulmonary nodules Arterial enhancement of multiple nodular areas in liver, largest 15 x 8 cm Portal HTN splenic varices and ascites

Triple Phase CT Liver

TUMOR CELLS BACKGROUND MATRIX

Histology Immunostains positive for endothelial markers CD31, CD34, Factor VIII antigen, as well as cytokeratin 7 Diagnosis: Hepatic hemangioendothelioma

Follow-up OLT performed Native liver with multinodular tumor occupying 75% of the liver Contiguous extension through liver capsule to involve diaphragm, porta hepatis, gall bladder, IVC Now 10 months post successful OLT without complications Maintained on tacrolimus PO 3mg QAM and 2mg QPM with normal hepatic laboratories Pulmonary nodules are of stable size