Epithelioid Hemangioendothelioma

A tumor of medium-to-large veins, composed of plump-to-spindled endothelial cells that bulge into vascular spaces in a tombstone-like fashion. These tumors are thought to have "borderline" aggression, where one-third develop local recurrences, but only rarely metastasize. It is unclear whether the epithelioid hemangioendothelioma is truly neoplastic or an exuberant tissue reaction, nor is it clear if this is equivalent to Kimura's disease (see ANGIOLYMPHOID HYPERPLASIA WITH EOSINOPHILIA). (Segen, Dictionary of Modern Medicine, 1992)

Also Known As:

Networked: 246 relevant articles (10 outcomes, 12 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hemangiosarcoma (Angiosarcoma)
2.	Neoplasms (Cancer)
3.	Neoplasm Metastasis (Metastasis)
4.	Neuroendocrine Tumors (Neuroendocrine Tumor)
5.	Klatskin Tumor

Experts

1.	Remiszewski, Piotr: 4 articles (10/2020 - 08/2013)
2.	Zieniewicz, Krzysztof: 3 articles (10/2020 - 08/2013)
3.	He, Yong: 2 articles (10/2022 - 01/2022)
4.	Carvalho, Lina: 2 articles (07/2022 - 04/2016)
5.	Rubin, Brian P: 2 articles (01/2022 - 08/2011)
6.	Ahn, Chul-Soo: 2 articles (03/2021 - 12/2016)
7.	Ha, Tae-Yong: 2 articles (03/2021 - 12/2016)
8.	Hong, Seung-Mo: 2 articles (03/2021 - 12/2016)
9.	Hwang, Shin: 2 articles (03/2021 - 12/2016)
10.	Jung, Dong-Hwan: 2 articles (03/2021 - 12/2016)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Epithelioid Hemangioendothelioma:

1.	Sirolimus (Rapamycin)FDA Link 01/01/2022 - "Sirolimus was reported to be an effective drug for epithelioid hemangioendothelioma but only about 10% achieved partial response. " 01/01/2021 - "Sirolimus for patients with progressive epithelioid hemangioendothelioma." 11/01/2020 - "A Retrospective Review of the Use of Sirolimus for Pediatric Patients With Epithelioid Hemangioendothelioma." 01/01/2016 - "We retrospectively reviewed patients with advanced and progressing epithelioid hemangioendothelioma (EHE) treated with sirolimus at the INT and/or within the RTR. " 01/01/2022 - "As a single agent, sirolimus led to impressive improvement in a patient with another vascular tumor-advanced epithelioid hemangioendothelioma. "
2.	Bevacizumab (Avastin)FDA Link 07/01/2010 - "Treatment of pulmonary epithelioid hemangioendothelioma with bevacizumab." 06/01/2012 - "This case report is the first one that describes the activity of the combination of chemotherapy and bevacizumab in epithelioid hemangioendothelioma. " 01/01/2013 - "Bevacizumab is an effective and well-tolerated treatment for metastatic or locally advanced angiosarcoma and epithelioid hemangioendotheliomas. " 01/01/2013 - "An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas." 03/01/2011 - "Bevacizumab chemotherapy for pulmonary epithelioid hemangioendothelioma with severe dyspnea."
3.	Sunitinib (Sutent)FDA Link 01/01/2012 - "To the best of our knowledge, this is the first reported case of metastatic renal epithelioid hemangioendothelioma treated successfully with sunitinib." 01/01/2012 - "Epithelioid hemangioendothelioma of the kidney treated with sunitinib." 01/01/2013 - "Targeting VEGF-VEGFR Pathway by Sunitinib in Peripheral Primitive Neuroectodermal Tumor, Paraganglioma and Epithelioid Hemangioendothelioma: Three Case Reports."
4.	Interferon alpha-2 (Roferon-A)FDA Link 01/01/1994 - "[Multifocal epithelioid hemangioendothelioma with partial remission after interferon alfa-2a treatment]."
5.	Sorafenib (BAY 43-9006)FDA Link 01/01/2016 - "Sorafenib Monotherapy in a Patient with Unresectable Hepatic Epithelioid Hemangioendothelioma." 04/12/2012 - "Malignant epithelioid hemangioendothelioma of the liver successfully treated with Sorafenib." 09/01/2014 - "We have carried out a stratified phase II study of sorafenib (So) in patients with advanced angiosarcoma (n = 32) and epithelioid hemangioendothelioma (n = 13). " 07/15/2013 - "Sorafenib in patients with progressive epithelioid hemangioendothelioma: a phase 2 study by the French Sarcoma Group (GSF/GETO)." 03/01/2015 - "Five patients-a woman with liver epithelioid hemangioendothelioma, three men with metastatic renal cell carcinoma, and a man with hepatocellular carcinoma-have developed sorafenib-associated facial acneiform eruption. "
6.	Fluorodeoxyglucose F18 (Fludeoxyglucose F 18)FDA Link 01/01/2021 - "The purpose of this study was to evaluate the imaging characteristics of epithelioid hemangioendothelioma (EHE) on staging 18F-FDG PET/CT. " 08/01/2023 - "18F-FDG PET/CT findings of hepatic and lung epithelioid hemangioendothelioma." 01/01/2022 - "Case Report: 18F-FDG PET/CT Demonstrating Malignant Spread of a Pulmonary Epithelioid Hemangioendothelioma." 01/01/2021 - "Advancing the diagnosis of epithelioid hemangioendothelioma by 18F-FDG PET/CT." 01/01/2021 - "Epithelioid hemangioendothelioma: evaluation by 18F-FDG PET/CT."
7.	pazopanibFDA Link 05/13/2015 - "The current case presents a patient with metastatic pulmonary epithelioid hemangioendothelioma to the cervical and mediastinal lymph nodes, lungs and liver that has been treated with pazopanib for more than two years with PET avid complete metabolic response in the mediastinum and lungs, and long-lasting stable disease. " 05/13/2015 - "Pazopanib for metastatic pulmonary epithelioid hemangioendothelioma-a suitable treatment option: case report and review of anti-angiogenic treatment options." 07/01/2023 - "Our case highlights the 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) scan findings in a rare case of biopsy-proven epithelioid hemangioendothelioma (EHE) in a 66-year-old woman with multi-organ involvement (lung, liver, and bone) who was subsequently treated with palliative radiation therapy and oral pazopanib. " 01/01/2015 - "Eight years tumor control with pazopanib for a metastatic resistant epithelioid hemangioendothelioma." 01/01/2021 - "Epithelioid Hemangioendothelioma and Epithelioid Hemangioma: Pazopanib as a Potential Salvage Therapy."
8.	DNA (Deoxyribonucleic Acid)IBA 01/01/2018 - "Cytologic features, immunocytochemical findings, and DNA ploidy in four rare cases of epithelioid hemangioendothelioma involving effusions." 06/01/1998 - "In this study, we evaluate the presence of loss of heterozygosity (LOH) of chromosome 22q in tumor DNA from 13 epithelioid sarcomas, four epithelioid angiosarcomas, and two epithelioid hemangioendotheliomas, and investigate its possible role in diagnosis. " 04/01/2005 - "A total of 13 cases of non-Kaposi sarcoma vascular tumors (12 hemangiomas and one epithelioid hemangioendothelioma) and 24 cases of Kaposi sarcoma, all from known HIV-positive patients, were immunostained for LANA and evaluated for the presence of HHV-8 DNA by real-time PCR. "
9.	VimentinIBA 01/01/2023 - "Atypical cells were positive for CD31, CD34, vimentin, and TFE3, and some of them had intracellular vacuoles, leading to the diagnosis of epithelioid hemangioendothelioma (EHE) of the liver. " 07/01/2010 - "Anatomopathological study revealed an epithelioid vascular neoplasm with low mitotic index, and tumor cells reactive to vimentin, CD31 and CD34, leading to the diagnosis of Grade I Epithelioid Hemangioendothelioma. "
10.	Contrast MediaIBA 04/01/2009 - "Hepatic epithelioid hemangioendothelioma (HEH) at MRI using reticuloendothelial system (RES)-specific contrast agent: the "bright-bright sign" rather than the "bright-dark sign"." 08/19/2013 - "The aim of this study was to determine if the appearance of hepatic epithelioid hemangioendothelioma (HEHE) on state-of-the-art MRI including hepatocyte phase after administration of hepatobiliary contrast agent can facilitate preoperative diagnosis and identification of potential candidates for liver transplantation. "

Therapies and Procedures

1.	Liver Transplantation 08/01/2008 - "Liver transplantation at an early stage has greatly improved the prognosis of epithelioid hemangioendothelioma. " 07/01/2023 - "Our patient was a 57-year-old woman who had undergone liver transplantation due to hepatic epithelioid hemangioendothelioma. " 10/15/2021 - "Liver transplantation for hepatic epithelioid hemangioendothelioma." 03/31/2021 - "Prognosis of hepatic epithelioid hemangioendothelioma after living donor liver transplantation." 10/01/2020 - "Liver transplantation provides favorable outcomes for selected patients with a hepatic form of epithelioid hemangioendothelioma."
2.	Transplantation 12/29/2016 - "Clinicopathological Features and Prognosis of Hepatic Epithelioid Hemangioendothelioma After Liver Resection and Transplantation." 01/01/2006 - "Patient with liver epithelioid hemangioendothelioma treated by transplantation: 3 years' observation." 12/01/2005 - "While good survival results have been achieved with transplantation in patients with epithelioid hemangioendothelioma of the liver, the long-term survival of some patients without any radical treatment leaves the benefit of transplantation unclear. " 11/01/1999 - "Hepatic epithelioid hemangioendothelioma: resection or transplantation, which and when?" 03/24/2019 - "Liver transplantation (LT) has improved substantially the prognosis of hepatic epithelioid hemangioendothelioma (HEHE), with 5-year patient survival rates of up to 81%, based on the European Liver Intestine Transplantation Association-European Liver Transplant Registry study. "
3.	Breast Implants (Breast Implant) 07/01/2005 - "Breast implants as a possible etiology of epithelioid hemangioendothelioma and successful therapy with interferon-alpha2."
4.	Orthoptics (Orthoptic) 02/01/2006 - "We report an unusual case of primary cardiac epithelioid hemangioendothelioma (EHE) with atypical features, which was treated by orthoptic transplantation with a good outcome for 10 years despite recurrent pulmonary and nodal metastases. "
5.	Curettage 04/01/2014 - "EH is a benign lesion and it should be differentiated from its histologically similar malignant counterparts such as epithelioid hemangioendothelioma and epithelioid angiosarcoma as the lesion can be successfully treated with curettage or resection."