Hepatic epithelioid hemangioendothelioma: A misdiagnosed rare liver tumor

Elleuch, Nour; Dahmani, W; Ben, Slama Aida; Jaziri, Hanene; Hammami, A; Ksiaa, M.; Jmaa, A.

doi:10.1016/j.lpm.2017.10.026

Cited by 13 publications

(20 citation statements)

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“…It was reported that 25% of patients had no obvious symptoms[ 3 ]. Initial symptoms have included pain in the right upper abdomen, ascites, weight loss, anorexia and jaundice in the late stage[ 3 ] as well as portal hypertension[ 2 ]. Some cases even presented with liver failure caused by a very large tumor[ 14 ], spontaneous rupture of the tumor[ 15 ] or corresponding symptoms caused by metastatic lesions, such as pleural effusion[ 16 ].…”

Section: Clinical Characteristics Of Hehe Casesmentioning

confidence: 99%

“…It can occur in multiple parts of the body such as the lungs, soft tissue, head and neck, pleura, bones and many other organs. EHE in the liver, or hepatic EHE (HEHE) is an even rarer disease, with a reported incidence of 1-2 of every 1 million people[ 2 ]. HEHE most commonly occurs between the ages of 30 and 50 years, with a predominance in females based on a male:female incidence ratio of 2:3[ 3 ].…”

Section: Introductionmentioning

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: Clinical Characteristics Of Hehe Casesmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

Kou

Chen

Zhou

et al. 2020

WJCC

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“…[ 10 – 12 ] It has an estimated incidence of 1 to 2 in 1,000,000 persons. [ 13 , 14 ] CT and magnetic resonance imaging (MRI) characteristics of this tumor are similar to that of cholangiocarcinoma, hepatocellular carcinoma, and colonic adenocarcinoma metastases. [ 1 , 15 – 19 ] The lung, peritoneum, spleen, lymph nodes, and bone are the most common sites of extra-hepatic involvement.…”

Section: Discussionmentioning

confidence: 99%

Complete metabolic response to therapy of hepatic epithelioid hemangioendothelioma evaluated with 18F-fluorodeoxyglucose positron emission tomography/contrast-enhanced computed tomography

Giancipoli¹,

Monti

Baştürk³

et al. 2018

Medicine

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Rationale:Hepatic epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor of endothelial origin with a highly variable clinical presentation and natural history. Given its vascular origin, new therapies with inhibitors of vascular endothelial growth factor (VEGF) have been introduced in the treatment of these patients and have shown promising results. Few reports have described the role of 18F-Fluorodeoxyglucose positron emission tomography/contrast-enhanced computed tomography (18F-FDG PET/CT) in the evaluation of this tumor after treatment with anti-angiogenic agents. Our case reports how 18F-FDG PET-CT scan was critical in the assessment of this tumor after treatment with an anti-angiogenic agent, Pazopanib, demonstrating complete metabolic response.Patient concerns:A 30-year-old man with no previous significant medical history presented with pain in the right upper quadrant for over a year.Diagnoses:Multiple hepatic masses were found on abdominal ultrasound. Liver biopsy confirmed the diagnosis of epithelioid hemangioendothelioma. 18F-FDG PET/CT was performed for staging. Multiple FDG-avid hepatic, splenic, and lymph nodes lesions were detected on 18F-FDG PET/CT. A subsequent spleen biopsy confirmed splenic involvement. Immunohistochemistry was positive for CD31, CD34, and ERG, supporting the diagnosis of epithelioid hemangioendothelioma.Interventions:A 1-year cyclophosphamide treatment was provided followed by Pazopanib for 17 months.Outcomes:Six years after the first 18F-FDG PET/CT, 18F-FDG PET/CT performed for restaging demonstrated complete metabolic response to therapy. Follow-up CT demonstrated no interval changes in size of some of the treated lesions.Lesson:18F-FDG PET/CT is useful for baseline assessment and posttreatment follow-up of this rare cancer.

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“…Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare primary liver sarcoma accounting for approximately 1% of all vascular tumors[ 113 ]. The incidence is reported to be around 1-2/1000000 with a predominance in females[ 114 ]. The natural course of HEHE is unpredictable, ranging from aggressive growth to indolent behavior[ 115 , 116 ].…”

Section: Primary Liver Cancermentioning

confidence: 99%

Liver transplantation in malignant disease

Lang¹,

Bednarsch²,

Czigány³

et al. 2021

WJCO

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Liver transplantation for malignant disease has gained increasing attention as part of transplant oncology. Following the implementation of the Milan criteria, hepatocellular carcinoma (HCC) was the first generally accepted indication for transplantation in patients with cancer. Subsequently, more liberal criteria for HCC have been developed, and research on this topic is still ongoing. The evident success of liver transplantation for HCC has led to the attempt to extend its indication to other malignancies. Regarding perihilar cholangiocarcinoma, more and more evidence supports the use of liver transplantation, especially after neoadjuvant therapy. In addition, some data also show a benefit for selected patients with very early stage intrahepatic cholangiocarcinoma. Hepatic epithelioid hemangioendothelioma is a very rare but nonetheless established indication for liver transplantation in primary liver cancer. In contrast, patients with hepatic angiosarcoma are currently not considered to be optimal candidates. In secondary liver tumors, neuroendocrine cancer liver metastases are an accepted but comparability rare indication for liver transplantation. Recently, some evidence has been published supporting the use of liver transplantation even for colorectal liver metastases. This review summarizes the current evidence for liver transplantation for primary and secondary liver cancer.

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Hepatic epithelioid hemangioendothelioma: A misdiagnosed rare liver tumor

Cited by 13 publications

References 13 publications

Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

Complete metabolic response to therapy of hepatic epithelioid hemangioendothelioma evaluated with 18F-fluorodeoxyglucose positron emission tomography/contrast-enhanced computed tomography

Liver transplantation in malignant disease

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