• Epithelioid hemangioendothelioma
• Interferon
• Liver
• Sirolimus
• Surgery
• Survival
• Transplantation

• Humans
• Male
• Female
• Hemangioendothelioma, Epithelioid/therapy
• Hemangioendothelioma, Epithelioid/mortality
• Hemangioendothelioma, Epithelioid/pathology
• Hemangioendothelioma, Epithelioid/diagnosis
• Liver Neoplasms/therapy
• Liver Neoplasms/mortality
• Liver Neoplasms/pathology
• Liver Neoplasms/diagnosis
• Retrospective Studies
• Adult
• Middle Aged
• Prognosis
• Liver Transplantation
• Young Adult
• Aged
• Treatment Outcome
• Adolescent
• Kaplan-Meier Estimate
• Hepatectomy

• 2024-NHLHCRF-PY I-03 National High Level Hospital Clinical Research Funding
• 2024-NHLHCRF-PY I-03 National High Level Hospital Clinical Research Funding
• 2024-NHLHCRF-PY I-03 National High Level Hospital Clinical Research Funding

• chemotherapy
• epithelioid hemangioendothelioma
• interferon
• liver
• sarcoma
• sirolimus

• National High Level Hospital Clinical Research Funding

• clinicopathologic features
• follow-up
• hepatic epithelioid hemangioendothelioma
• prognosis
• therapeutic strategies

In this article, we describe the case of a 34-year-old woman presenting a multifocal and metastatic epithelioid hemangioendothelioma (HEHE) of the liver. Under classical chemotherapy using cyclophosphamide, there was a fast tumor progression in liver and extra-hepatic metastatic sites (lungs and mediastinal lymph node). Taking into account the patient’s age and the natural history of the HEHE, our goal was to try to bring her to liver transplantation (LT) and lenvatinib was an acceptable candidate for this reason. Shortly after the initiation of lenvatinib before LT and surgery, we observed the enlargement of large devascularized necrotic areas in most of the liver HEHE masses, suggesting a good response. The patient was finally transplanted 6 months after initiation of lenvatinib treatment. Eight months after LT, progression occurred (ascites, peritoneal recurrence, and mediastinal lymph node). After restarting lenvatinib, ascites disappeared and the lymph node decreased in size, suggesting a good response, more than 1 year after her transplantation. This is the first case report to our knowledge that illustrates the benefit of lenvatinib as a neoadjuvant bridge until LT for a multifocal and metastatic HEHE. In addition, this drug has also shown a benefit in term of disease control after a late recurrence of the tumor. We suggest that lenvatinib should be proposed as a bridge to the LT for nonresectable HEHE. Moreover, this drug was also beneficial in the treatment of late recurrence after LT. The absence of pharmacologic interactions between classical immunosuppressive drugs and lenvatinib may allow its use as an early adjuvant approach when the risk of recurrence is high. The strength of our case consists in the long follow-up and the innovative message allowing changing palliative strategies into curative ones in case of advanced HEHE.

• epithelioid hemangioendothelioma
• lenvatinib
• liver transplantation

To investigate the MRI features and clinical significance of hepatic epithelioid hemangioendothelioma (HEHE).

Clinical records and MRI findings were retrospectively evaluated in nine HEHE patients from May 2010 to January 2020.

There were 121 lesions in nine patients with a predominantly peripheral distribution. Five lesions (4.13%) in two patients (22.22%) had evidence of capsular retraction, and three patients had lung metastasis (33.33%). Dynamic contrast-enhanced MRI showed progressive enhancement, mainly in two ways: ring enhancement with hypovascularity in four patients (44.44%) and ring enhancement with hypervascularity in five patients (55.56%). Imaging demonstrated a multilayer ring appearance, which was typically observed on T2-weighted imaging (T2WI). The most common appearance consisted of two layers of varying signal, with some images displaying up to four layers. There were significant differences in the size of lesions between different layers of multilayer ring appearance (p < 0.001). All lesions exhibited a two-layer appearance on diffusion-weighted imaging (DWI), with hyperintensity at the periphery and a slightly high signal at the center (except for those with a single layer on T2WI). The “vascular penetration sign” was observed in most lesions, and the blood vessels of 112 lesions (92.56%) were portal vein branches, and five (4.13%) were hepatic vein branches. Pulmonary metastasis was found in three patients with the “vascular penetration sign” of hepatic vein branches.

The multilayer ring appearance on T2WI, the “vascular penetration sign”, and the two enhancement patterns may be of great significance in the diagnosis and treatment of HEHE. The “vascular penetration sign” of hepatic vein branches may indicate extrahepatic metastasis.

• dynamic contrast-enhanced magnetic resonance imaging (MRI)
• extrahepatic metastasis
• hepatic epithelioid hemangioendothelioma (HEHE)
• hepatic vein branches
• liver tumor

Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor type. No effective medicine or standard treatment for HEH has been established.

From March 2014 to April 2021, 62 patients with pathologically diagnosed HEH were observed regularly, and interferon-alpha 2b (IFN-a 2b) was administered to patients with progressive disease or reoccurrence. Adverse events (AEs) were assessed and recorded, and a tumor assessment scan was performed every 3 months.

A total of 42 patients with HEH received IFN-a 2b treatment in this study. No severe (grade ≥3) AEs were reported in the group overall. The most common treatment-related AEs in patients receiving IFN-a 2b were fever (50.0%) and fatigue (21.4%). Partial response and complete response were achieved in 20 patients (47.6%) and 2 patients (4.8%), respectively, and the objective response rate was 52.4%. Stable disease was observed in 12 patients (28.6%), and the disease control rate was 81.0%. Progressive disease was observed in 8 patients (19.0%). The 1-, 3-, and 5-year progression-free survival rates were 81.0%, 69.2%, and 62.3%, respectively. Only 1 patient died as a result of disease progression during the study. The 1-, 3-, and 5-year overall survival rates were 100%, 97.2%, and 97.2%, respectively.

IFN-a 2b is a safe and effective treatment for patients with HEH. The encouraging results with IFN-a 2b use make it a promising option for patients who have other types of epithelioid hemangioendothelioma; additional clinical trials are needed.

• chemotherapy
• epithelioid hemangioendothelioma
• interferon
• liver
• sarcoma

• Borderline tumor
• Immunohistochemical stain
• Metastasis
• Recurrence
• Vascular tumor

Epithelioid hemangioendothelioma (EHE) is a rare borderline vascular tumor. This retrospective, single-center study evaluated the outcomes of hepatic resection (HR) in patients with hepatic EHE.

Over the 10-year period from 2009 to 2018, 11 patients with hepatic EHE underwent HR, accounting for 0.1% of the 11,979 adults who underwent HR at our center. Diagnosis of hepatic EHE was confirmed by immunohistochemical staining for CD34, CD31, and factor VIII-related antigen.

The 11 patients included 9 females (81.8%) and 2 males (18.2%) with mean age of 43.5 ± 13.6 years. Preoperative imaging resulted in a preliminary diagnosis of suspected liver metastasis or EHE, with 9 patients (81.8%) undergoing liver biopsy. No patient presented with abnormally elevated concentrations of liver tumor markers. The extents of HR were determined by tumor size and location from trisectionectomy to partial hepatectomy. All patients recovered uneventfully from HR. Five patients showed tumor recurrence, with 4 receiving locoregional treatments for recurrent lesions. The 1-, 3- and 5-year disease-free survival rates were 90.9%, 54.5%, and 54.5%, respectively. Currently, all patients remain alive and are doing well. Univariate analysis on tumor recurrence showed that tumor size ≥ 4 cm was significantly associated with tumor recurrence (P = 0.032), but tumor number ≥ 4 was not related to (P = 0.24).

Hepatic EHE is a rare form of primary liver tumor often misdiagnosed as a metastatic tumor. Because of its malignant potential, HR is indicated if possible. HR plus, when necessary, treatment of recurrence yields favorable overall survival rates in patients with hepatic EHE.

• CD31 antigen
• CD34 antigen
• Hemangioendothelioma
• Hemangioma
• Vascular tissue neoplasms

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.

• Hepatic epithelioid hemangioendothelioma
• Diagnosis
• Differential diagnosis
• Therapy
• Prognosis
• Imaging

• Angiosarcoma
• Cholangiocarcinomas
• Epithelioid hemangioendotheliomas
• Halo sign
• Hepatocellular carcinoma
• Lollipop sign

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.

• 1st line treatment
• Olaratumab and Doxorubicin
• metastatic hepatic epithelioid hemangioendothelioma

• Diagnostic
• Hepatic angiosarcoma
• Hepatic epithelioid hemangioendothelioma
• Hepatic hemangiopericytoma
• Hepatic malignant vascular tumors
• Hepatic perivascular epithelioid cell tumor
• Hepatic small vessel neoplasia
• Kaposi sarcoma
• Prognostic
• Treatment

• Liver neoplasms
• haemangiopericytoma
• hepatectomy
• hepatic epithelioid haemangioendothelioma
• hepatic haemangioma
• hepatic haemangiomatosis
• hepatic haemangiosarcoma
• hepatic infantile haemangioendothelioma
• hepatic small vessel neoplasms
• liver transplantation
• nodular regenerative hyperplasia
• vascular tissue

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor. Preoperative diagnosis of HEHE is difficult because it does not manifest specific symptoms or tumor markers. We report a resected case of small and solitary HEHE. The patient, a 74-year-old man, had undergone surgical resection for left renal cell carcinoma 20 years ago. During follow-up, a tumor approximately 1.3 cm in diameter was detected by computed tomography (CT) at liver segment VIII. It showed isodensity in the arterial phase, low density in the portal venous phase, and homogeneous enhancement in the late phase on CT and magnetic resonance imaging (MRI). We performed hepatic resection of the right hepatic vein drainage area. A pathological diagnosis of HEHE was made. Although small and solitary HEHE is rare, an enhancement pattern in each phase on CT and MRI, using contrast media, can yield clues for the diagnosis of HEHE.

• Hepatic epithelioid hemangioendothelioma
• Hepatic nodule
• Rare liver tumor

• cancer intervention
• gastrointestinal surgery
• hepatic cancer

• hepatic cancer
• liver disease
• radiology