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A 60-year-old woman with a 20-years history of hepatitis B presented with 2 weeks of dull pain in the right upper quadrant and intermittent nausea. Abdominal MRI showed a solitary lesion in Segment VIII of the liver. It demonstrated multi-layered target appearance resembling a "maze" on diffusion-weighted image (DWI) and T2 weighted images (T2WI), and appeared as a low signal target with a hypointense core on the hepatobiliary phase. Histopathology confirmed hepatic epithelioid hemangioendothelioma (HEHE). Our case showed a solitary hepatic lesion with as many as six layers and overall appearances are more like a "maze", which was a diagnostic challenge. However, this maze-like manifestation actually makes hepatocellular carcinoma as the main differentials being very unlikely. Other differential diagnosis such as metastasis,cholangiocarcinoma or atypical hemangioma are also unlikely to show more than three layers. Core pattern, regarded as a noval imaging feature of HEHE, is different from general features of hepatocellular carcinoma or cholangiocarcinoma. Our case highlights that HEHE needs to be taken into consideration when a multi-layer appearing hepatic lesion is found incidentally in the cirrhotic liver.

Background Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare borderline tumor of vascular endothelial origin. Laparoscopic resection of HEHE has never been reported. Methods The clinical data of eleven patients with HEHE (4 women and 7 men) who were diagnosed and treated at the Union Hospital (Wuhan, China), and Wuhan Asia General Hospital (Wuhan, China), between March 2012 and July 2020 were analyzed retrospectively. Results The mean age of HEHE patients was 42.4 ± 13.9 years (range 22–67 years). All patients underwent laparoscopic surgery alone or in combination with radiofrequency ablation. Most tumors showed aggressive growth or metastasis. By immunohistochemistry, tumor cells were positive for CD31, CD34, ERG, PCK, FLi-1, TFE-3, and Ki-67 (labeling index range, 5–15%). In one of the patients, the tumor was accompanied by partial necrosis with a local appearance of epithelioid angiosarcoma. Postoperative adjuvant treatment included chemotherapy, sorafenib, and Huaier granule. As of July 2020, the median follow-up duration was 36 months (range, 9–60 months), with 2 (18.2%) patients experiencing tumor recurrence. Conclusions This is the first report of laparoscopic hepatectomy of HEHE. Curative laparoscopic hepatectomy might be an acceptable treatment for appropriate HEHE patients.

Background and aims Hepatic epithelioid hemangioendothelioma is an ultra-rare hepatic vascular tumor, diagnosed more frequently in females. The knowledge about this tumor derives mainly from small case series with sub-optimal treatment outcomes. The aim of this study is to identify the clinical and radiological issues helpful to develop an international prospective registry. Methods We conducted an international multicentric and retrospective study of patients with hepatic hemangioendothelioma. The clinical, pathological and radiological images collected during follow-up were reviewed. Central radiological revision was performed and 3 patterns of contrast were defined. Results Between 1994 and 2016, 27 patients with hepatic hemangioendothelioma were identified in three institutions but the final diagnosis was hepatic angiosarcoma in one. The majority were females, median age was 38.7-years and 17 patients were asymptomatic at diagnosis. No patient had Two out of ten (20%) patients had surgical specimens with positive macro-vascular invasion and 50% had extrahepatic disease, and the most frequent pattern was the progressive-central-contrast-uptake. After a median follow-up of 6.7-years, the 5- and 10-year survival rates are 91.5% and 51.9%, respectively. Conclusions This multicentric study shows the heterogeneous profile of patients with hepatic hemangioendothelioma, reflecting the need to establish a reference network in order to better characterize these patients and ultimately develop a personalized treatment strategy.

To examine the characteristic features of hepatic epithelioid hemangioendothelioma (HEH) on magnetic resonance imaging (MRI) using Gd-EOB-DTPA. Twelve patients (mean age, 50 years; male:female = 6:6) who were pathologically confirmed to have HEH in two tertiary institutions were retrospectively investigated. For qualitative analysis, the MRI features of HEH including core pattern were characterized, and lesions were divided into core and non-core groups. For quantitative analysis, standardized mean signal intensities (SIst) measured at the tumor center, periphery, and liver parenchyma were plotted against the dynamic phases. Differences in SIst between the core and non-core group were calculated for the tumor center and periphery. We also examined the radiologic and pathologic correlation for cases in which surgical resection was performed. Forty-seven nodules in 12 patients were analyzed. The mean size of the lesions was 2.9 ± 1.0 cm. In the per-lesion analysis, ring-like arterial enhancement (74%) on arterial phase was the most frequent feature, followed by core pattern (51%), and hyperintense rim on T1-weighted imaging (43%). In the per-patient analysis, capsular retraction (75%) was the most common sign. The percentage of patients with core pattern was 58%. In the core group, the SIst of the center showed slow enhancement starting from the transitional phase, resulting in divergence between the two graphs throughout the entire dynamic study (p

Background Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor with indolent behavior in terms of malignancy. The treatment of choice is either resection in the case of resectable lesions or liver transplantation (LT) for the disseminated intrahepatic form. The aim of this study was to investigate the outcomes of patients with HEHE treated by LT. Material and Methods There were 18 patients with HEHE who underwent LT between 2002 and 2018 included in this retrospective study. The study group was comprised of young recipients (median age of 39 years) and mainly women (15 of 18; 83.3%). Two recipients had concomitant tumors of epithelioid hemangioendothelioma in the liver and lungs prior to LT. The survival probability was calculated using the Kaplan-Meier estimator. Results According to histopathological data, none of the patients had a macrovascular invasion. In 4 patients (22.2%), the disease had spread to the hilar lymph nodes. The maximum diameter of the tumor in the studied group was 18 cm. The survival probability after 1, 5, and 15 years was 94.0%, 82.6%, and 41.3%, respectively. No disease recurrence was observed during a median follow-up of 65.9 months. Conclusion Liver transplantation provides favorable outcomes for selected patients with a hepatic form of epithelioid hemangioendothelioma.

Rationale: Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of the liver with malignant potential. It can be of solitary type, multifocal type, or diffuse type. Although there are some characteristic features on radiologic imaging, the definitive diagnosis of HEH is based on histopathology. The surgical treatment of HEH includes liver resection and transplant. Patient concerns: A middle-aged woman presented with easy fatiguability and anorexia for 1 month was found to have multifocal lesions on radiological imaging. Diagnosis: HEH was diagnosed by needle biopsy. It can be seen from imaging that this case is a multifocal form. The largest lesion increased from 3 to 3.3 cm within 2 months, with an increase of 9.45%; no other relevant literatures have been reported. Interventions: The possibility of liver transplantation was suggested to the patient. However, the patient refused transplantation and was successfully treated by radical right hepatectomy and resection of the left lobe lesion. Outcomes: She remained disease-free throughout a year follow-up period. Conclusion: HEH is a rare disease with characteristic radiological and pathological features. Although liver transplantation is the preferred treatment for multifocal HEH, surgical excision represents one alternative when the lesions can be guaranteed to be completely excised.

Epithelioid hemangioendothelioma (EHE) is an angiocentric tumor that, when arising in liver, is centered around hepatic/portal veins. However, EHE cells can also track along sinusoids, which is not well recognized or studied. We identified 18 cases of hepatic EHE and 6 nonhepatic EHEs. For all cases, we recorded EHE multifocality and maximum size. When tumor cells were identified apart from the main mass, we recorded their location, maximum distance from the main tumor, density per high-power field, and cytomorphology. Immunohistochemical staining for CAMTA1, ERG, and CAM5.2 was performed on all cases. Lesional cells were present apart from the main mass in 17 of 18 (94%) liver cases, always within sinusoids and occasionally (4/17, 24%) in central veins. They appeared intensely hyperchromatic with vaguely cerebriform nuclei and multinucleation in 6 (35%) of cases. CAMTA1 and ERG positivity was seen in all 17 cases. Two cases (12%) demonstrated focal CAM5.2 positivity. Sinusoidal EHE cells ranged from 0.1 to 0.8 cm away from the main tumor. There were no statistically significant associations between histologic findings and patient outcome. In the 6 nonhepatic cases, tumor cells did not extend beyond the main EHE. Lesional cells in hepatic EHE often extend beyond the main lesion into sinusoids, where they demonstrate an unusual, somewhat distinctive morphology. Care should be taken to identify such cells in limited biopsies; immunohistochemistry for CAMTA1, a specific and sensitive marker for EHE, can be confirmatory.

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.

Rationale: Malignant hepatic epithelioid hemangioendotheliom (HEH) is a rare vascular tumor of endothelial origin, with multiple metastases to the spleen. This report describes a diffuse HEH with splenic metastasis on 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) images and delayed mutifocal bone metastasis after liver transplantation (LTx). Patient concerns: A 30-year-old male was admitted to our hospital with a complaint of abdominal distension, fatigue, and anorexia for 2 months. Diagnoses: Mild to moderate FDG uptake in the whole liver, and multifocal FDG uptake in the spleen were observed on 18F-FDG PET/CT scan. Ultrasound guided liver biopsy was performed, and a diagnosis of HEH was confirmed. Interventions: The patient underwent LTx and splenectomy. Outcomes: The patient developed low back pain due to unknown etiology, 3 months after surgery. A follow-up 18F-FDG PET/CT scan demonstrated multifocal bone destruction. Unfortunately, the patient died 12 months after surgery. Lessons: It is noteworthy that despite liver transplantation for the treatment of HEH, there may be a risk of recurrence. For these patients with extrahepatic lesions, adjuvant chemotherapy may be a useful alternative treatment method for the prevention of recurrence.

International audience; A 36-year-old woman with no relevant medical or surgical history was referred to our attention for abdominal diffuse pain and mild weight loss. No risk factors such as hepatitis, alcohol abuse, oral contraceptive use, or a family history of carcinoma were reported. Liver laboratory studies were unremarkable, except for elevated gamma-glutamyl transferase (138 U/L). Viral serology tests were negative, as were serum carcinoembryonic antigen, CA 19-9, and alpha-fetoprotein.

BACKGROUND AND OBJECTIVES: To study clinical features, imaging findings, and prognosis of hepatic epithelioid hemangioendothelioma (HEHE) in Eastern Hepatobiliary Surgical Hospital and to improve the level of recognition and preoperative diagnosis of HEHE. DESIGN AND SETTINGS: This is a retrospective study. The study was conducted at Eastern Hepatobiliary Surgical Hospital in Shanghai, China from 1999 to 2012. METHODS: Clinical data of 15 pathology-confirmed HEHE patients admitted in our hospital from 1999–2010 were collected and analyzed retrospectively. RESULTS: Besides 7 cases of abdominal discomfort, this cohort of HEHE patients does not show common typical manifestations. Laboratory examinations found 8 low ALB/GLB cases, 3 mild anemia cases, and 1 high alanine aminotransferase case, but no abnormal α-fetoprotein, carcinoembryonic antigen, or CA19-9 cases. Plain computed tomography (CT) scanning found uneven multiple low-density lesions in most cases, dynamic enhanced CT scanning demonstrated peripheral enhancement in the hepatic arterial HA phase and more peripheral enhancement in the portal vein PV phase, and ring enhancement were detected in the delayed phase. Magnetic resonance imaging (MRI) demonstrated low signal intensity and even lower lesion centers in T1-weighted images, but slightly high signal intensity lesions and higher lesion centers in T2-weighted images. Enhanced MRI scanning showed ring enhancements but no obvious enhanced centers. Lesions were further enhanced in delayed MRI scanning. Prognosis of this cohort: 10 of these 15 patients were alive, including 4 cases with a tumor. CONCLUSION: Comprehending the clinical and radiological characteristics of HEHE facilitates the level of recognition and preoperative diagnosis of this disease, and promotes surgeons to take active and appropriate surgeries.

PURPOSE The aim of this study was to evaluate retrospectively magnetic resonance imaging (MRI) and (18)F fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) findings of hepatic epithelioid hemangioendothelioma (HEH). PATIENTS AND METHODS MRI and FDG PET/CT findings were reviewed in 6 patients with HEH confirmed by pathology. All patients underwent magnetic resonance (MR) examination. Early PET/CT scans were performed 1 hour after FDG injection in all 6 patients. After an interval of 1 hour, delayed PET/CT scans were performed in 4 patients. RESULTS A total of 60 lesions were detected in all 6 patients. MRI features of HEH included multifocal hepatic disease, predominantly subcapsular location, coalescence of lesions, and capsular retraction. T2-weighted MR images frequently showed a target-like configuration of the lesions. Contrast-enhanced MR images showed variable degrees of peripheral rim enhancement with delayed central enhancement. Forty lesions (67%) with increased FDG uptake and 20 lesions (33%) with FDG uptake similar to the surrounding liver parenchyma were found in all 6 patients. The mean maximum standardized uptake value (SUV(max)) of all lesions was 3.6 ± 1.1, with a low variability of SUV(max) among lesions ranging from 1.7 to 6.6. There was no relationship between lesion sizes and corresponding SUV(max). Some larger lesions demonstrated a hypermetabolic peripheral rim reflecting hypercellular tumor regions and a relatively hypometabolic central area corresponding to hypocellular stroma. Eleven lesions with increased FDG uptake on the delayed PET/CT images were found in 3 patients, and 19 lesions with decreased FDG uptake were found in all 4 patients with total 32 lesions. CONCLUSIONS MRI demonstrated morphological features of HEH and FDG PET/CT reflected the histopathological composition of the tumors. FDG uptake of HEH may be related to tumor cellularity, but not the tumor size. Dual-time-point imaging may be not useful for differentiating benign lesions from HEH. Familiarity with the morphological and functional imaging findings of HEH is useful for recognition of this rare hepatic tumor.

This multicenter-based retrospective study aimed to investigate the prognostic factors and report our experiences with the diagnosis and treatment of hepatic epithelioid hemangioendothelioma (HEHE), a rare malignant vascular tumor.A total of 33 patients with HEHE from two centers between 2004 and 2011 were retrospectively reviewed with respect to their clinical, radiologic, and pathologic characteristics; treatment modalities and outcomes; and potential prognostic factors.A total of 17 patients underwent liver resections (LRs) alone, 12 patients had transcatheter arterial chemoembolization (TACE) alone, three patients had LR followed by TACE, and one patient underwent liver transplantation (LT). The difference of overall survival (OS) between LR and TACE was not significant (p = 0.499). Older patients [≥47 years, n = 17; p = 0.035, hazard ratio (HR) = 7.0), those with symptoms (n = 17; p = 0.001, HR = 86.5], and those with an elevated serum CA19-9 level (37 U/ml, n = 5; p = 0.018, HR = 5.0) had a poorer OS, according to univariate analysis. The presence of symptoms was validated as a prognostic factor (p = 0.012) by multivariate analysis.Liver resection and TACE have comparable outcomes in HEHE patients. The presence of symptoms indicates a poor prognosis. Older age and elevated serum CA19-9 are potential negative impact factors on outcome.

Epithelioid hemangioendothelioma is a very rare, low-grade vascular tumor known to arise in soft tissues and visceral organs. Clinical diagnosis of hepatic epithelioid hemangioendothelioma remains a challenge, and although it is frequently managed with a liver transplant due to its multifocal nature, recurrence is a common complication. We review recent advances in the diagnosis of hepatic epithelioid hemangioendothelioma, including major genetic breakthroughs, and discuss efforts to reduce post-liver transplant recurrence of hepatic epithelioid hemangioendothelioma.

Hepatic epithelioid hemangioendothelioma (HEH) is an unusual, low-grade malignant vascular tumor of the liver. Here we describe a case of a 40-year-old woman who presented with abdominal pain in the upper right quadrant and giant hepatomegaly, in which imaging studies and a fine-needle liver biopsy confirmed the presence of a large EHE with an isolated lung metastasis. After balancing all possible therapeutic modalities the patient was treated conservatively with thalidomide (300 mg/day). The drug was well tolerated with minimal toxicity and the patient continues on therapy 109 months after treatment was started with no disease progression. Current therapeutic options for HEH are discussed in light of the clinical case with particular emphasis on anti-angiogenic therapies.

We report the MRI findings in three patients with pathologically proven hepatic epithelioid hemangioendothelioma, a rare tumor of the liver that is not well described in the MR imaging literature. The recognition of the imaging features of this rare malignancy may help further early detection and surgical treatment of this potentially curable disease.

Hepatic epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with low- to intermediate-grade malignant potential. We describe cross-sectional imaging findings of pathologically confirmed EHE including preliminary observations on lesion characteristics and apparent diffusion coefficients (ADCs) at diffusion-weighted MR imaging (DWI).CT and MRI examinations in five patients were retrospectively reviewed. Two radiologists evaluated lesion growth patterns, attenuation, signal intensity characteristics, and contrast enhancement patterns. Additionally, DWI features on low and high b-value images as well as ADCs were assessed in three patients.Imaging features of EHE included multifocal hepatic disease (n = 5), predominantly subcapsular location (n = 5), coalescence of nodules (n = 5), capsular retraction (n = 3), and intralesional calcifications (n = 3). Contrast-enhanced CT and MR images showed variable degrees of peripheral rim enhancement. T2-weighted MR images, low b-value DWI and ADC maps frequently depicted a "target-sign" appearance of tumor nodules. A markedly hyperintense central area corresponding to hypocellular stroma was surrounded by a moderately hyperintense outer rim reflecting hypercellular tumor regions. The mean ADC of lesions was 1.86 × 10(-3) mm(2)/s.Cross-sectional imaging displayed typical features of EHE. The mean ADC value of lesions was found to be relatively high in comparison with other hepatic malignancies, which may be helpful in suggesting the diagnosis.

Background/Purpose: Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of the liver typically with a slow but progressive course. We report the clinical and immunohistochemical characteristics of six cases from our institution between 1993 and 2008. Methods: We searched the files of the Department of Pathology in National Taiwan University Hospital from January 1993 to December 2008 and found six cases of primary HEH. The clinical data were reviewed. The microscopic findings of each case were listed and analyzed. Confirmational immunoperoxidase stains were performed with antibodies against two endothelial markers (CD31 and CD34) and one epithelial marker (AE1/AE3 or cytokeratin). Results: There were five female patients and one male patient with HEH, and the mean age was 45.3 years (range, 25-86 years). Most patients were asymptomatic and one third of cases presented as right costal or abdominal pain. Anemia was the most common laboratory abnormality. Liver failure developed at the advanced diffuse stage. Imaging studies revealed three different patterns as single nodular, multiple nodular, or diffuse types, reflecting different stages of disease and clinical symptoms. Microscopic findings included intracytoplasmic vascular lumen formation (100%), sinusoidal spreading (100%), vessel obliteration (66.7%), necrosis (66.7%), and cellular pleomorphism (16.7%). All cases expressed endothelial markers of CD31 and CD34, reflecting their vascular nature. Two patients received surgical treatment including partial liver resection and liver transplantation. Tumor recurrence developed 8 and 17 months later, respectively. Conclusion: HEH showed insidious growth and frequent multicentricity, making early diagnosis and tumor resection difficult. Definite diagnosis totally relies on pathologic study. Tumor progression to hepatic failure is slow. Liver transplantation is currently the most prevalent treatment modality for HEH, but experience in Taiwan is limited due to the rarity of this disease.

We describe the imaging findings in a rare case of multifocal nodular form of hepatic epithelioid hemangioendothelioma on ultrasound (US) and dynamic contrast-enhanced spiral computed tomography (CT) and MRI. The nodules showed multilayered target appearance on MR images with prominent peripheral rim with high signal intensity (SI) on T1-weighted and very low SI on T2-weighted images, corresponding to thrombosed vascular channels. The above combination of imaging findings is to our knowledge distinct and should suggest the diagnosis of hepatic epithelioid hemangioendothelioma.

INTRODUCTION: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare entity. At the present time, there is no standardized effective therapy. Liver transplantation (LT) has emerged as a treatment for this rare tumour.OBJECTIVE: To evaluate the outcome of liver transplantation for HEHE at eight centres across Canada.METHODS: The charts of patients who were transplanted for HEHE at eight centres across Canada were reviewed.RESULTS: A total of 11 individuals (eight women and three men) received a LT for HEHE. All LTs were performed between 1991 and 2005. The mean (± SD) age at LT was 38.7±13 years. One patient had one large liver lesion (17 cm × 14 cm × 13 cm), one had three lesions, one had four lesions and eight had extensive (five or more) liver lesions. One patient had spleen involvement and two had involved lymph nodes at the time of transplantation. The mean duration of follow-up was 78±63 months (median 81 months). Four patients (36.4%) developed recurrence of HEHE with a mean time to recurrence of 25±25 months (median 15.6 months) following LT. The calculated survival rate following LT for HEHE was 82% at five years.CONCLUSIONS: The results of LT for HEHE are encouraging, with a recurrence rate of 36.4% and a five-year survival rate of 82%. Further studies are needed to help identify patients who would benefit most from LT for this rare tumour.

Background This study aims to analyze the computed tomography (CT) and magnetic resonance imaging(MRI) characteristics of hepatic epithelioid hemangioendothelioma (HEHE). Methods Eleven patients with histopathologically confirmed HEHE via surgical excision or biopsy were included. Imaging findings of these 11 patients were retrospectively analyzed (CT images obtained from all patients and MR images from five patients). Patterns of growth, characteristics of distribution, density/signal features, patterns of contrast enhancement, and changes of adjacent tissues were evaluated. Results HEHE is characterized by multiple lesions in the liver. HEHE could be further categorized as three types when considering patterns of growth: nodular type(5 cases), coalescent type(1 case) and mixed type(5 cases). In this study, a total of 312 lesions were detected, 214(74.3 %) of which were subcapsular. All lesions appeared as hypodense while round lower density were found within 10 lesions(

Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of the liver with an unpredictable malignant potential. Its growth can lead to hepatic failure, extrahepatic metastasis and death. Surgical resection or liver transplantation is the treatment of choice if metastasis is not identified. Several antineoplastic agents have been proposed for cases of nonresectable HEH. We report the case of a 52-year-old patient with HEH metastatic to the lungs who was successfully treated with oral thalidomide therapy.