Hepatocellular carcinoma in a patient with alpha-1 antitrypsin deficiency

Case contributed by Bruno Di Muzio
Diagnosis certain


Known history of alpha 1 antitrypsin deficiency.

Patient Data

Age: 60 years
Gender: Female

The liver is not clearly cirrhotic. There is a segment V/VI hyperenhancing tumor that shows partial washout, and also becoming partially indistinct in the portal venous phase. Lung bases demonstrate features of predominantly panlobular emphysema in keeping with the history of alpha-1 antitrypsin deficiency. 

Segment V/VI hypervascular lesion with washout. 

Case Discussion

The imaging features are those of a hypervascular tumor in a patient referred with a clinical history of both alpha-1 antitrypsin deficiency. The findings are highly concerning for a hepatocellular carcinoma. The patient went to surgical resection: 

Macroscopic Description: Labeled "Liver resection, Segment 5 & 6".  A wedge resection of liver 70.5 g and 75 x 47 x 44 mm (length x thickness capsular edge to capsular edge x depth free edge to apex) with an intact, brown and slightly granular capsule.  Sectioning reveals a circumscribed, lobulated green-brown mass 23 x 21 x 21 mm, 5 mm from the resection margin and within 1 mm from the capsule.  Part processed.

Microscopic Description: Sections show liver tissue containing a circumscribed and rounded but nonencapsulated hepatocellular tumor. The tumor is composed of atypical hepatocytes forming small nests and fairly frequent acinar formations. There is moderate intra tumoral macrosteatosis. The tumor cells have pleomorphic nuclei with prominent nucleoli. Conspicuous bile plugs are seen in tumor cell acini. No lymphovascular or perineural invasion is identified. The tumor does not breach the hepatic capsule and is clear of the parenchymal resection margin.

The surrounding tissue has a normal architecture with patent central veins and portal tracts. There is macrovesicular steatosis affecting less than 5% of hepatocytes. Occasional large PASD positive globules are present within the cytoplasm of periportal hepatocytes. The Perls stain for iron is negative. There is fibrous expansion of portal tracts with periportal septa formation, but no bridging fibrosis.

Diagnosis: Segment 5 and 6 liver resection: Moderately differentiated hepatocellular carcinoma, 23 mm in size, clear of margins. No lymphovascular invasion identified. AJCC (8th edition): pT1b. Surrounding liver shows features consistent with alpha-1 AT deficiency with mild fibrosis. No iron overload.

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