Epithelioid hemangioendothelioma of the liver: imaging findings with pathologic correlation

Hepatic epithelioid hemangioendothelioma is a rare malignant neoplasm that has nonspecific clinical signs and symptoms and can be difficult to diagnose on the basis of biopsy results. Radiologists may suggest the diagnosis of this slowly progressive neoplasm by recognizing its characteristic radiologic features. We correlated images from CT (13), sonography (nine), and MR (six) with pathologic findings in resected whole livers (eight) and biopsy specimens (five) from 13 patients 25-58 years old. Gross pathologic examination showed a repetitive pattern of multiple solid tumor nodules, in a predominantly peripheral distribution, with coalescence as individual nodules exceeded 4 cm. Tumor nodules had a hyperemic rim. Lesions adjacent to the capsule often produced capsular retraction. These findings correlated well with imaging findings. On CT, the lesions were of low attenuation, peripherally based, and with capsular retraction or flattening in nine (69%) of 13 patients. Unenhanced CT scans showed superior conspicuity over contrast-enhanced CT scans (9/13, 69%) and showed the extent of lesions more accurately in all cases (13/13, 100%). In nine patients, lesions had a peripheral enhancement pattern of alternating attenuation values correlating with the hyperemic rim at pathologic evaluation. On sonograms, the tumors were solid and predominantly hypoechoic. On MR, tumor signal was low on T1-weighted and high on T2-weighted images, with a low-signal halo present around many of the lesions. CT, sonographic, or MR findings of coalescent peripheral hepatic masses with capsular retraction are highly suggestive of hepatic epithelioid hemangioendothelioma.