1. Case of the Month: November 2018
Mirek Mychajlowycz, PGY2
Diagnostic Radiology
McMaster University

2. Case Presentation 65M seen in ER
Chief complaint: RUQ fullness and pain with 4.5kg weight loss
PMHx: non-contributory
Physical exam:
Liver edge palpable 6cm below inferior costal margin and tender
Negative Murphy’s sign, remainder of exam normal
Labs:
WBC, LFTs normal
Imaging: CT abdomen pelvis C+, followed by MR C+ (Gd)

3. CT Findings Portal venous phase:
Large right lobe posterior segment liver mass
Discontinuous, nodular, peripheral enhancement
Enhancement follows aortic attenuation (blood pool)
Central low attenuation in keeping with a fibrous scar

4. CT Findings Delayed phase: Progressive centripetal enhancement
Mass is iso-/hyper-attenuating to liver parenchyma
*Central scar

5. MR Findings T1: Mass hypointense to liver T1 C+ (Gadolinium):
Peripheral nodular discontinuous enhancement that progresses inward
Mass replaced right hepatic lobe
IVC compressed but patent, left portal vein obliterated T1
T1 C+
T1 C+
T1 C+

6. MR Findings T2: Mass hyperintense to liver parenchyma
T2 Coronal
MR Findings
T2:
Mass hyperintense to liver parenchyma
Lower intensity than a hepatic cyst
*Relatively hyperintense central scar
T2 Axial

7. Giant Hepatic Cavernous Hemangiomas (GHCHs)
AKA slow flow venous malformation (ISSVA)
Atypical subset of hepatic hemangioma (most common benign liver tumor)
Epidemiology:
5:1 female-to-male distribution
Age at diagnosis yrs
Thought to be congenital but etiology is unknown
Considered giant when >5 cm
Composed of a tangle of endothelial-lined blood- filled cavities fed by the hepatic artery
H&E stain: varying sized channels with RBCs within surrounded by fibrous connective tissue

8. GHCH Differential Diagnosis
Depending on the imaging modality and patient history, the differential may include:
Hepatic metastases – hypervascular mets (eg. thyroid cancer and renal cell carcinoma) are often multiple, show marked early enhancement and do not retain contrast on delayed images
Hepatocellular carcinoma – enhances vividly in the arterial phase then washes out in the PV phase
Hepatic cyst – generally <1 cm, may be equivocal on CT and can be resolved on targeted ultrasound exam

9. GHCH Differential Diagnosis Continued
Hepatic abscess
Hepatic adenoma
Intrahepatic cholangiocarcinoma
Focal nodular hyperplasia

10. Clinical Presentation of GHCH
Most are asymptomatic
Mass effect, hemorrhage and thrombosis may cause abdominal fullness and pain due to liver capsule stretching
Mass effect on biliary tree or vascular structures may cause variable clinical presentations (ex. jaundice, ischemia)
History of steroid use, pregnancy, ovarian stimulation (clomiphene) and/or hormone replacement therapy may accelerate development

11. GHCH Associations Hepatic hemangiomatosis
Klippel-Trenaunay-Weber disease
Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu disease)
Von Hippel-Lindau disease

12. Further Imaging Preoperative angiography for embolization:
Characteristic “snowy tree” or cotton wool appearance
Almost pathognomonic of a hemangioma

13. Further Imaging * Ultrasound Well-defined hyperechoic lesion
10% hypoechoic on background of hepatic steatosis
*Central anechoic scar
Colour doppler: peripheral feeding vessels
Contrast-enhanced:
Arterial – peripheral nodular discontinuous enhancement
PV/delayed - progressive filling until hyperechoic to liver *

14. Complications of GHCH
Increased risk of complications compared to typical hemangiomas
Inflammatory changes
Intralesional hemorrhage
Intraperitoneal hemorrhage
Volvulus of a pedunculated lesion
Kasabach-Merrit syndrome: consumptive thrombocytopenia resulting in intravascular coagulation, clotting, and fibrinolysis within the hemangioma and may spread systemically; 20-30% mortality

15. GHCH Management Generally conservative unless symptomatic
No known risk of hepatic malignancy
NEVER biopsy due to increased risk of hemorrhage
Non-surgical:
Arterial embolization
+/- radiation and interferon therapy
Surgical:
Enucleation or liver resection

16. References
Bajenaru N, Balaban V, Savulescu F, Campeanu I, & Patrascu T. (2015). Hepatic hemangioma review. J Med Life, 8: 4-11 Bell, D & Weekrakkody, Y. (2018). Hepatic hemangioma. Radiopedia. Retrieved November 1, 2018 from 3#image_list_item_ Knipe, H & Yang, N. (2018). Giant hepatic venous malformations. Radiopedia. Retrieved November 1, 2018 from malformation Machado M et al. (2006). Liver hemangiomas: Ultrasound and clinical features. Radiol Bras, 29(6): Madoff, S. (2009). Case 77: Giant hepatic cavernous hemangioma. UR Medicine Imaging. Retrieved November 1, 2018 from mirc2.urmc.rochester.edu/storage/ss1/docs/ /MIRCdocument.xml Prasanna P, Fredericks S, Winn S, & Christman R. (2010). Giant cavernous hemangioma. RadioGraphics, 30(4):