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Presented by Dr. Argani and prepared by Austin McCuiston.
This is a 63 year old male with multiple liver lesions.
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1. Question
This is a 63 year old male with multiple liver lesions.
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Answer: Epithelioid hemangioendothelioma
Histologic Description: This is a multifocal lesion which has prominent myxochondroid stroma and cords of epithelioid cells with cytoplasmic vacuolization, which raises the differential diagnosis of intrahepatic cholangiocarcinoma and metastatic adenocarcinoma. The vacuoles are focally septate, and there is no overt formation of blood vessels or of true glands. The neoplastic cells label for vascular markers CD34, CD31 and ERG, and demonstrate no immunoreactivity for cytokeratins. The findings are diagnostic of epithelioid hemangioendothelioma.
Differential Diagnosis: Both intrahepatic cholangiocarcinoma and metastatic adenocarcinoma would be expected to show strong immunoreactivity for cytokeratin. Hepatocellular carcinoma is infrequently sclerosing and typically features cells with more prominent granular eosinophilic cytoplasm.
Epithelioid hemangioendothelioma frequently shows focal cytokeratin immunoreactivity, which heightens the mimicry of adenocarcinoma. This lesion demonstrates a characteristic t(1;3) (p36;q25) chromosome translocation resulting in a WWTR1-CAMTA1 gene fusion.
Incorrect
Answer: Epithelioid hemangioendothelioma
Histologic Description: This is a multifocal lesion which has prominent myxochondroid stroma and cords of epithelioid cells with cytoplasmic vacuolization, which raises the differential diagnosis of intrahepatic cholangiocarcinoma and metastatic adenocarcinoma. The vacuoles are focally septate, and there is no overt formation of blood vessels or of true glands. The neoplastic cells label for vascular markers CD34, CD31 and ERG, and demonstrate no immunoreactivity for cytokeratins. The findings are diagnostic of epithelioid hemangioendothelioma.
Differential Diagnosis: Both intrahepatic cholangiocarcinoma and metastatic adenocarcinoma would be expected to show strong immunoreactivity for cytokeratin. Hepatocellular carcinoma is infrequently sclerosing and typically features cells with more prominent granular eosinophilic cytoplasm.
Epithelioid hemangioendothelioma frequently shows focal cytokeratin immunoreactivity, which heightens the mimicry of adenocarcinoma. This lesion demonstrates a characteristic t(1;3) (p36;q25) chromosome translocation resulting in a WWTR1-CAMTA1 gene fusion.
