title: 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.
authors.affiliation: From the Department of Internal Medicine, University of Cincinnati College of Medicine; Division of Hematology Oncology, UC Department of Pediatrics, Cincinnati Children's Hospital; and the Divisions of Pathology and Hematology and Oncology, University of Cincinnati College of Medicine, Cincinnati, Ohio.
authors.forename: Nisar
authors.initials: N
authors.lastname: Ahmad
authors.affiliation: From the Department of Internal Medicine, University of Cincinnati College of Medicine; Division of Hematology Oncology, UC Department of Pediatrics, Cincinnati Children's Hospital; and the Divisions of Pathology and Hematology and Oncology, University of Cincinnati College of Medicine, Cincinnati, Ohio.
authors.forename: Denise M
authors.initials: DM
authors.lastname: Adams
authors.affiliation: From the Department of Internal Medicine, University of Cincinnati College of Medicine; Division of Hematology Oncology, UC Department of Pediatrics, Cincinnati Children's Hospital; and the Divisions of Pathology and Hematology and Oncology, University of Cincinnati College of Medicine, Cincinnati, Ohio.
authors.forename: Jiang
authors.initials: J
authors.lastname: Wang
authors.affiliation: From the Department of Internal Medicine, University of Cincinnati College of Medicine; Division of Hematology Oncology, UC Department of Pediatrics, Cincinnati Children's Hospital; and the Divisions of Pathology and Hematology and Oncology, University of Cincinnati College of Medicine, Cincinnati, Ohio.
authors.forename: Rajan
authors.initials: R
authors.lastname: Prakash
authors.affiliation: From the Department of Internal Medicine, University of Cincinnati College of Medicine; Division of Hematology Oncology, UC Department of Pediatrics, Cincinnati Children's Hospital; and the Divisions of Pathology and Hematology and Oncology, University of Cincinnati College of Medicine, Cincinnati, Ohio.
authors.forename: Nagla Abdel
authors.initials: NA
authors.lastname: Karim
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D006432:Hemochromatosis / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008113:Liver Neoplasms / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D008279:Magnetic Resonance Imaging; D049268:Positron-Emission Tomography; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult