Hepatic Epithelioid Hemangioendothelioma – a Rare Tumor and Diagnostic Dilemma

Třeška, Vladislav; Daum, Ondřej; Svajdler, M.; Liška, Václav; Ferda, Jiří; Baxa, Jan

doi:10.21873/invivo.11128

Cited by 22 publications

(15 citation statements)

References 12 publications

(11 reference statements)

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“…The typical HEHE sign is concentric enhancement of contrast medium in the vascularized components and almost no enhancement in the corresponding center consisting of fibrotic tissue. Some tumors may also show fine calcification[ 19 ].…”

Section: Auxiliary Examinationmentioning

confidence: 99%

“…VEGF inhibitors, such as sorafinib[ 58 , 59 ], pazopanib[ 60 ], bevacizumab, and others, are known to play a role in the treatment of EHE. It was reported that the combination of the anti-VEGF drug bevacizumab and cell cycle inhibitor capecitabine achieved a good curative effect[ 19 , 61 ]. Other drugs such as mTOR inhibitors[ 56 ], thalidomide[ 62 - 64 ], pegylated liposomal doxorubicin[ 65 ], metronomic cyclophosphaide[ 66 ] and others have been introduced for the treatment of EHE and have acquired good results.…”

Section: Therapymentioning

confidence: 99%

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Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

Kou

Chen

Zhou

et al. 2020

WJCC

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With an estimated incidence of only 1-2 cases in every 1 million people, hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. HEHE is characterized as a low-to-moderate grade malignant tumor and is classified into three types: solitary, multiple, and diffuse. Both the etiology and characteristic clinical manifestations of HEHE are unclear. However, HEHE has a characteristic appearance on imaging including ultrasound, magnetic resonance imaging, and positron emission tomography/computerized tomography. Still, its diagnosis depends mainly on pathological findings, with immunohistochemical detection of endothelial markers cluster of differentiation 31 (CD31), CD34, CD10, vimentin, and factor VIII antigen as the basis of diagnosis. Hepatectomy and/or liver transplantation are the first choice for treatment, but various chemotherapeutic drugs are reportedly effective, providing a promising treatment option. In this review, we summarize the literature related to the diagnosis and treatment of HEHE, which provides future perspectives for the clinical management of HEHE.

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Section: Auxiliary Examinationmentioning

confidence: 99%

Section: Therapymentioning

confidence: 99%

Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

Kou

Chen

Zhou

et al. 2020

WJCC

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“…Thus, radical surgical resection or liver transplantation are considered the methods of choice in patients with localized liver lesions. Owing to the multifocal nature of these tumors in most cases, liver transplantation is proposed as the treatment of choice in patients with multicentric tumors although liver resection has been performed more commonly [ 4 , 14 ]. Liver transplantation has been considered the treatment of choice when partial hepatectomy is not feasible owing to intrahepatic dissemination.…”

Section: Discussionmentioning

confidence: 99%

Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

Din¹,

Rahim²,

Asghari³

et al. 2020

Diagn Pathol

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Background Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature. Methods All cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained. Results Seven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon. Conclusion Clinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.

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“…9 Chemotherapy with several regimens such as Doxorubicin with or without ifosfamide, paclitaxel, epirubicin and dacarbazine, 5FU and mitomycin have been reported in small series. [18][19][20][21][22] Tyrosine kinase inhibitors such as pazopanib, sunitinib, apatinib and sorafenib have been studied showing PRor stabilization of the disease. [23][24][25][26][27][28] Targeting angiogenesis with bevacizumab, interferon alpha 2b, thalidomide and lenalidomide revealed contradictory results.…”

Section: Discussionmentioning

confidence: 99%

<p>Metastatic Hepatic Epithelioid Hemangioendothelioma Treated with Olaratumab: A Falling Star Rising?</p>

Kyriazoglou

Koutsoukos

Zagouri

et al. 2020

TCRM

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Epithelioid hemangioendothelioma (EHE) is a rare vascular malignant tumor with indolent course. Liver transplantation for local disease is the treatment of choice. In the metastatic setting there is no consensus regarding the appropriate systemic treatment. We present two cases of metastatic hepatic epithelioid hemangioendothelioma (hEHE) treated with the combination of Doxorubicin and Olaratumab. Both patients showed Stable Disease (SD) as a response, after the completion of six cycles of this combination therapy.

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Hepatic Epithelioid Hemangioendothelioma – a Rare Tumor and Diagnostic Dilemma

Cited by 22 publications

References 12 publications

Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

<p>Metastatic Hepatic Epithelioid Hemangioendothelioma Treated with Olaratumab: A Falling Star Rising?</p>

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