JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
RADIOLOGICAL DIAGNOSIS: Hepatic Adenomatosis
result in intraperitoneal hemorrhage. The risk of malignant transformation into hepatocellular carcinoma is around 10%. 3 Biochemically, HA is characterized by an increase of serum gamma-glutamyl transpeptidase and alkaline phosphatase secondary to local biliary obstruction and inflammation, consistent with our patient’s findings. 3 Hepatic adenomatosis is more frequent in young females. The adenomas have a 63% risk of bleeding, which may result in intraperitoneal hemorrhage. The risk of malignant transformation into hepatocellular carcinoma is around 10%. 3 Biochemically, HA is characterized by an increase of serum gamma-glutamyl transpeptidase and alkaline phosphatase secondary to local biliary obstruction and inflammation, consistent with our patient’s findings. 3 Imaging studies described to aid in the diagnosis of HA include ultrasound, CT and Magnetic Resonance (MR). Adenomas demonstrate variable echogenicity on ultrasound with
IMAGING FINDINGS
Triple phase CT of the abdomen reveals numerous hepatic masses, the majority of which demonstrate arterial phase hyperenhancement and portal venous hypoenhancement (arrows). Given the multiplicity of these lesions and patient's age, hepatic adenomatosis was considered as the most likely diagnosis (Figure 1).
HISTOPATHOLOGY
Well-differentiated hepatocellular neoplasms characterized by hepatocytes with aberrant blood vessels and loss of portal tracts (arrows). Immunostains for Alpha Feto Protein and Glypican – 3 are negative in the hepatic parenchyma. The histopathological findings are considered most likely to be related with hepatic adenoma (Figure 2).
Figure 2: (A) Hematoxylin & Eosin, (B) Alfa Feto Protein, and (C) Glypican – 3 stains of Hepatic Parenchyma.
pertiumoral or intratumoral vessels exhibiting flat continuous or triphasic waveforms on Doppler interrogation. Hepatic adenomas are hypo- to isodense on noncontrast CT images although may be mildly hyperdense in patients with hepatic steatosis. Adenomas are commonly sharply-marginated and nonlobulated and may show varying amounts of intratumoral hemorrhage, fat, calcifications, or cystic changes. 5 Postcontrast arterial phase demonstrates early homogeneous hypervascular enhancement of the lesion that is transient due to extensive intralesional arteriovenous shunting. As adenomas are composed of sheets of normal hepatocytes and sparse Kuppfer cells, lesions typically demonstrate isoenhancement to liver parenchyma on portal venous and delayed phases. 5 On MR, hepatic adenomas are isointense to mildly hyperintense lesions on T1 weighted images and variably hyperintense lesions on T2 weighted images. 3,5 Differential diagnosis for HA include Focal Nodular Hyperplasia, Hemangiomas, Hypervascular Metastases, Hepatocellular Carcinoma and Hepatic Hemangioendothelioma. 1 Of note in our case, several lesions demonstrated early arterial hyperenchancemnet with subsequent portal venous
DISCUSSION
Hepatic adenoma is an uncommon benign tumor composed of a sheet like distribution of hepatocytes without the presence of bile ducts and portal venules. Risk fctors for the development of hepatic adenoma include oral contraceptive use, anabolic steorid abuse, glycogen storage disorders and fanconi anemia, among others. 1 In 1985, Flejou et al. described the term hepatic adenomatosis (HA) as tenormore adenomaswithnormal hepatic parenchyma. 2 In comparison to hepatic adenomas, HA demonstrates no associations with oral contraceptive or steroid use. 2,3 Genotypically, HA is linked to hepatocyte nuclear factor (HNF)- 1a mutation. 1 In 2000, Chiche et al. described two types of this disease. HA type 1 or Massive HA, is characterized by hepatic architectural distortion and hepatomegaly with multiple hepatic nodules. Type 2 HA, or Multifocal HA, contains several smaller adenomas and a preserved hepatic contour, leading to less severe clinical manifestations. 3,4
Hepatic adenomatosis is more frequent in young females. The adenomas have a 63% risk of bleeding, which may
J La State Med Soc VOL 169 NOVEMBER/DECEMBER 2017 179
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