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Case of the Week #341
A 60 year old woman was found to have multiple, slow growing liver lesions, and underwent a liver biopsy.
What is your diagnosis?
Immunostains were performed:
Left to right: CD34, CD31, HepPar1
The H&E sections show an intrasinusoidal and intravascular infiltrative growth pattern (figure 1) with intraluminal tufting (figure 2) of epithelioid and spindle cells with surrounding compressed hepatocytes with steatosis (figure 3). Figure 4 shows epithelioid cells with intracytoplasmic vacuoles ("signet ring cells"). The immunostains show that the epithelioid cells are strongly positive for CD34 and CD31, and although the hepatocytes are immunoreactive for HepPar1, the intrasinusoidal proliferation is HepPar1 negative.
Grossly, epithelioid hemangioendothelioma is often multifocal, grey-white and firm, with variable calcification. Histologically, it has a characteristic zonal pattern with peripheral tumor cells growing along preexisting sinusoids and terminal hepatic venules, with sinusoidal proliferation and intraluminal polypoid or tuft-like projections. Some epithelioid cells show rudimentary intracytoplasmic lumina. Cytologically, the atypical cells have moderate eosinophilic cytoplasm, relatively large hyperchromatic and irregular nuclei and small inconspicuous nucleoli. The surrounding hepatocytes show compression and focal steatosis. The midzone shows sinusoidal obliteration with marked atrophic hepatocyte plates in a fibrous and myxochondroid stroma, with variable calcification (Clin Mol Hepatol 2013;19:315). Tumor cells are immunoreactive for vascular markers CD31, CD34 and D2-40. They are also positive for CD10.