Hepatic epithelioid haemangioendothelioma (HEH) is a rare vascular neoplasm accounting for less than 1% of all hepatic malignancies.¹ x 1. Weitz, J., Klimstra, D.S., and Cymes, K. Management of primary liver sarcomas. Cancer. 2007; 109: 1391–1396 Crossref | PubMed | Scopus (99) | Google Scholar See all References Since initially described in 1982 by Weiss and Enzinger,² x 2. Weiss, S.W. and Enzinger, F.M. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982; 50: 970–981 Crossref | PubMed | Scopus (868) | Google Scholar See all References less than 500 cases have been reported.³ x 3. Mehrabi, A., Kashfi, A., and Fonouni, H. Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer. 2006; 107: 2108–2121 Crossref | PubMed | Scopus (207) | Google Scholar See all References HEH primarily occurs in women in the mid fifth decade of life without underlying chronic liver disease. Aetiologic risk factors are unknown, although HEH has occurred in association with oral contraceptive pills (OCPs), exposure to vinyl chloride or major hepatic trauma.^3. x 3. Mehrabi, A., Kashfi, A., and Fonouni, H. Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer. 2006; 107: 2108–2121 Crossref | PubMed | Scopus (207) | Google Scholar See all References ^{, 4.} x 4. Furuta, K., Sodeyama, T., and Usuda, S. Epithelioid hemangioendothelioma of the liver diagnosed by liver biopsy under laparoscopy. Am J Gastroenterol. 1992; 87: 797–800 PubMed | Google Scholar See all References ^{, 5.} x 5. Gelin, M., Van de Stadt, J., and Rickaert, F. Epithelioid hemangioendothelioma of the liver following contact with vinyl chloride. Recurrence after orthotopic liver transplantation. J Hepatol. 1989; 8: 99–106 Abstract | Full Text PDF | PubMed | Scopus (39) | Google Scholar See all References ^{, 6.} x 6. Ishak, K.G., Sesterhenn, I.A., and Goodman, Z.D. Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases. Hum Pathol. 1984; 15: 839–852 Abstract | Full Text PDF | PubMed | Scopus (308) | Google Scholar See all References ^{, 7.} x 7. Makhlouf, H.R., Ishak, K.G., and Goodman, Z.D. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer. 1999; 85: 562–582 Crossref | PubMed | Scopus (306) | Google Scholar See all References Clinical presentation is variable and non-specific and ranges from an indolent course^6. x 6. Ishak, K.G., Sesterhenn, I.A., and Goodman, Z.D. Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases. Hum Pathol. 1984; 15: 839–852 Abstract | Full Text PDF | PubMed | Scopus (308) | Google Scholar See all References ^{, 7.} x 7. Makhlouf, H.R., Ishak, K.G., and Goodman, Z.D. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer. 1999; 85: 562–582 Crossref | PubMed | Scopus (306) | Google Scholar See all References to a rapidly progressive disease.^8. x 8. Clements, D., Hubscher, S., and West, R. Epithelioid haemangioendothelioma. A case report. J Hepatol. 1986; 2: 441–449 Abstract | Full Text PDF | PubMed | Scopus (43) | Google Scholar See all References ^{, 9.} x 9. Dietze, O., Davies, S.E., and Williams, R. Malignant epithelioid haemangioendothelioma of the liver: a clinicopathological and histochemical study of 12 cases. Histopathology. 1989; 15: 225–237 Crossref | PubMed | Scopus (50) | Google Scholar See all References ^{, 10.} x 10. Eckstein, R.P. and Ravich, R.B. Epithelioid hemangioendothelioma of the liver. Report of two cases histologically mimicking veno-occlusive disease. Pathology. 1986; 18: 459–462 Crossref | PubMed | Scopus (22) | Google Scholar See all References ^{, 11.} x 11. Fukayama, M., Nihei, Z., and Takizawa, T. Malignant epithelioid hemangioendothelioma of the liver, spreading through the hepatic veins. Virchows Arch A Pathol Anat Histopathol. 1984; 404: 275–287 Crossref | PubMed | Scopus (56) | Google Scholar See all References ^{, 12.} x 12. Hayashi, Y., Inagaki, K., and Hirota, S. Epithelioid hemangioendothelioma with marked liver deformity and secondary Budd-Chiari syndrome: pathological and radiological correlation. Pathol Int. 1999; 49: 547–552 Crossref | PubMed | Scopus (21) | Google Scholar See all References In nearly 25% of patients, HEH is diagnosed incidentally. The diagnosis of HEH is confirmed by immunostaining for factor VIII-related antigens and other endothelial markers on biopsy.² x 2. Weiss, S.W. and Enzinger, F.M. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982; 50: 970–981 Crossref | PubMed | Scopus (868) | Google Scholar See all References No imaging features of HEH are pathognomonic.