1. Hemangioendothelioma

2. 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

3. 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

4. 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

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

6. 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

7. Triple Phase CT Liver

8. TUMOR CELLS
BACKGROUND MATRIX

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

10. 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