Clinical Case Summary
A 51-year-old gentleman presented with a four week history of weight loss and anaemia. He has no past medical history but previously, he drank alcohol excessively. Clinically, he was only found to have hepatomegaly. His ALT, ALP, GGT were 65, 267 and 726 respectively. His bilirubin, clotting profile, albumin, alpha-fetoprotein (AFP) and non-invasive liver screen were all normal. CT chest abdomen pelvis showed moderate hepatomegaly with homogenous echotexture and no evidence of malignancy. Four months later he represented with abdominal pain and transudative ascites. His liver profile became grossly deranged with ALT, ALP, GGT of 92, 1446 and 3200 respectively. His bilirubin became 21 and albumin 27. A repeat CT scan showed a large liver with marked focal fatty infiltration, new ascites and a suspicion of new poorly defined focal liver lesion in the left liver lobe. MRI liver showed hepatomegaly, moderate ascites, multiple linear subcapsular T1 low signal, T2 high signal non-enhancing foci mainly in segments 2 and 3 which were described as non-specific. His liver biopsy showed cords of epithelioid cells with hyperchromatic nuclei and cytoplasmic vacuoles on a background of myxohyaline stroma in keeping with hepatic haemangioendothelioma (HHE). He was discussed with our local tertiary liver unit and was deemed not for transplantation. He rapidly deteriorated over several days and re-presented with multi-organ failure and died within 6 months of index presentation. HHE is a rare malignant tumour of vascular endothelial lineage, which was first reported by Weiss et al. in 1982 [1]. The disease can be localised or multifocal within the liver but can also be present in other organs. It is a rare cause of hepatic decompensation which can be difficult to diagnose and treat. The tumour has been reported to be misdiagnosed in approximately up to 80% of cases, commonly as less rarer conditions such as angiosarcoma, cholangiocarcinoma, metastatic carcinoma and haepatoma [2,3].
References
1.Weiss, S.W. and Enzinger, F.M. (1982) “Epithelioid hemangioendothelioma a vascular tumor often mistaken for a carcinoma,” Cancer, 50(5), pp. 970–981. Available at: https://doi.org/10.1002/1097-0142(19820901)50:5<970::aid-cncr2820500527>3.0.co;2-z.
2. Torres LR, Timbo LS, Ribeiro CM, Galvao Filho MM, Verrastro CG, et al. Multifocal and metastatic hepatic hemangioendothelioma: case report and literature review. Radiol Bras. 2014;47:194–6.
3. Virarkar, M. et al. (2020) “Hepatic Hemangioendothelioma: An update,” World Journal of Gastrointestinal Oncology, 12(3), pp. 248–266. Available at: https://doi.org/10.4251/wjgo.v12.i3.248.