Multifocal epithelioid hemangioendothelioma of the liver is a rare primary tumor with a variable course of disease. A case is presented of a 27-year-old female patient with multiple hepatic lesions on ultrasonography, suspect of metastatic tumor of the liver. Serum tumor markers were not elevated, while clinical examination of the lungs, gastrointestinal and gynecologic systems did not confirm the presence of a primary tumor process. Metastatic tumor and primary hepatocellular tumor were ruled out by fine needle aspiration cytology. Along with a characteristic immunophenotype of the vascular cell endothelium (positive for CD31 and CD34), high proliferation demonstrated by the analysis of argyrophilic nucleolar organization regions (AgNOR) and DNA aneuploidy, cytomorphological pattern suggested the diagnosis of angiosarcoma. Histopathologic finding corresponded to epithelioid hemangioendothelioma. Ten years after orthotopic liver transplantation, the patient is free from disease relapse, with regular follow up testing. Hemangioendothelioma of the liver is characterized by multifocality, which excludes resection; thus, liver transplantation is the method of choice. Therefore, preoperative diagnostic workup is of utmost importance to differentiate it from other primary and metastatic tumors of the liver.
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Cureus
December 2024
Department of Medicine, Division of Endocrinology and Metabolism, King Abdulaziz Medical City, Riyadh, SAU.
Epithelioid hemangioendothelioma (EHE) is a rare form of vascular neoplasm that can manifest with various symptoms or be discovered incidentally in asymptomatic patients. In this report, we describe a case of a 56-year-old male who presented with progressive lower limb weakness over four years. The evaluation revealed severe hypophosphatemia, an inappropriately normal fibroblast growth factor 23 C-terminal (cFGF-23) level, and a 30 x 20 mm hypermetabolic right pleural mass, which was subsequently proven to be EHE.
View Article and Find Full Text PDFCureus
January 2025
Oral and Maxillofacial Surgery, Queen Elizabeth Hospital Birmingham, Birmingham, GBR.
Epithelioid haemangioendothelioma (EHE) is a rare vascular neoplasm characterised by proliferation of vascular endothelial and pre-endothelial cells. The prevalence is less than one in a million people. It is principally observed in the soft tissues of the extremities but can also occur in the bone, brain, liver, lung and lymph nodes.
View Article and Find Full Text PDFJ Cardiothorac Surg
December 2024
Thoracic and Vascular Surgery Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Background: Epithelioid hemangioendothelioma (EHE) is an uncommon vascular malignancy characterized by an unpredictable clinical course and a high potential for recurrence and metastasis. The lack of standard treatment guidelines, coupled with the tumor's inconsistent response to available treatments, complicates the management of EHE and leads to widely varying patient prognoses.
Case Presentation: We report two cases of EHE with distinct presentations reflecting the site of involvement.
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