The patient is a 48-year-old woman with an incidental liver lesion on imaging. The CT scan with contrast highlights a 3.7 cm well-defined hepatic mass, hyper-enhanced in the arterial phase with early washout. A hepatic enzymes assay indicates a mildly elevated ALT but is otherwise normal. AFP and CEA levels are within the normal range. A liver biopsy is obtained, and photomicrographs are provided for your review.

The lesion is positive for a combination of myoid and melanocytic markers and has patchy staining with CD68 and TFE3, but it is negative for SOX10, DOG1, keratin AE1/AE3, ERG and arginase, amongst others. Based on the provided information, choose the best answer.

A. The lesion is a hepatic angiomyolipoma (AML) which, in contrast to its renal counterpart, has no association with tuberous sclerosis complex.

B. As a hepatic perivascular epithelioid cell tumor (PEComa), this lesion is exclusively hereditary in the context of tuberous sclerosis complex.

C. Staining pattern and imaging features of this tumor are in keeping with hepatic angiosarcoma.

D. This is a steatohepatitic type of hepatocellular carcinoma (HCC) arising from a non-cirrhotic background.

E. This tumor is a hepatic AML, reportedly associated with inactivating somatic mutations of TSC2.

F. As a hepatic AML, this tumor is known to have uniformly characteristic radiographic features.

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Images

2 photo(s) Updated on: 11/01/2023

Submitting pathologist

Maryam Zenali, MD and Dharamainder Choudhary PhD

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