Hepatic Epithelioid Hemangioendothelioma in a Patient With Hemochromatosis

Abstract

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare hepatic vascular tumor that represents a diagnostic challenge. The rarity of this neoplasm precludes establishment of a standard-of-care treatment. Risk factors for HEHE are not well-known. Liver transplant remains the most common therapeutic modality for nonmetastatic disease. This article describes a patient who presented with abdominal pain, fatigue, poor appetite, and weight loss. Genetic testing showed that the patient was homozygous for C282Y consistent with hereditary hemochromatosis, and liver biopsy was consistent with malignant epithelioid hemangioendothelioma. The patient was referred for a liver transplant but was deemed inappropriate for transplant secondary to peritoneal studding, with frozen-section analysis showing metastatic disease at the time of surgery.

The changing epidemiology of the ageing thalassaemia populations: A position statement of the Thalassaemia International Federation

“…Another liver malignancy potentially related to iron overload is hepatic epithelioid hemangioendothelioma, which is a rare hepatic vascular tumour that represents a diagnostic challenge. It was recently described in a patient homozygous for C282Y consistent with hereditary hemochromatosis with liver iron of 6,8 mg/g dry weight at biopsy 22 …”

“…It was recently described in a patient homozygous for C282Y consistent with hereditary hemochromatosis with liver iron of 6,8 mg/g dry weight at biopsy. 22…”

A Rare Hepatic Tumor Mimicking Hepatocellular Carcinoma in a Patient With Hepatitis C Cirrhosis

“…2,3 The diagnosis of HEH can be difficult, with some reports suggesting a 60% to 80% initial misdiagnosis rate. 4 Magnetic resonance imaging findings may be suggestive of other primary liver neoplasms and classically show a heterogeneous lesion with low signal intensity on T1weighted imaging and moderate to high signal intensity on T2-weighted images. 5 Contrast administration typically lacks the characteristic hepatocellular carcinoma arterial phase enhancement and may show only peripheral lesion enhancement or no lesion enhancement.…”

“…5 Histopathologic findings are key to the diagnosis, often showing positive staining for endothelial markers such as CD31, CD34, and Factor VIII-related antigen. 2,4 Treatment options traditionally include hepatic resection or transplantation, but also recently have expanded to involve systemic antiangiogenic therapies. 2 Optimal treatment strategies for this neoplasm have not been fully defined, and our patient elected for hepatic resection without adjuvant chemotherapy.…”