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Background: Hepatic epithelioid hemangioendothelioma (HEHE) is a malignant vascular tumor known for its rarity. The different types of this hepatic tumor (single, multiple-nodular or diffused) indicate different prognosis and treatment plans. However, the heterogenic clinical manifestation creates a dilemma and a wide range of challenges when attending to HEHE patients. This review addresses the unique profile and clinical challenges that complicate the diagnosis and treatment of HEHE while focusing on current therapeutic strategies and their limitations. Main text: The unclear etiology is a challenging feature of HEHE. The exact involvement of potential risk factors and mechanism of development are still undefined. Relevant genetic alterations like WWTR1–CAMTA1 fusion have been investigated; however, they are only applicable as diagnostic markers and their influence on therapeutic efficacy is largely unknown. Other characteristics include asymptomatic manifestation, lack of unique hepatic functional alterations, high rates of misdiagnosis and late-stage identification when metastases already exist. Currently, tissue biopsy is the main tool to establish a definite diagnosis but is challenged with the limited awareness to suspect HEHE at early stages and the lack of relevant guidelines due to the rarity and the insufficiency of relevant research. The absence of treatment guidelines is the greatest challenge of HEHE. Generally, Surgical approaches are recommended due to the benefits of prolonged survival and enhanced prognosis. Nonetheless, only a minority of patients are eligible for resections while liver transplants are faced with severe insufficiency of donor organs and long wait-lists. On the other hand, a variety of non-surgical treatments (like anti-angiogenic agents, interferon alpha-2B and sirolimus) are presented with a promising potential. However, relevant studies are challenged with limited sample-sizes and lack of prospective designs. Conclusion: Regardless to decades passing since its discovery, HEHE still creates a dilemma due to its challenging clinical profile and lack of treatment guidelines. Raising awareness of HEHE in clinical practices improves the ability to diagnose this rare tumor at early stages and develop stronger research strategies and treatment guidelines to regulate the medical care provided to HEHE patients. [ABSTRACT FROM AUTHOR]

Background: Epithelioid hemangioendothelioma (EHE), is an uncommon, intermediate-grade malignant vascular tumor that can manifest in diverse organs, including the liver, lungs, and bones. Given its unique malignancy profile and rarity, there lacks a consensus on a standardized treatment protocol for EHE, particularly for hepatic epithelioid hemangioendothelioma (HEHE). This study aims to elucidate factors influencing the clinical prognosis of EHE by analyzing data from the SEER database, complemented with insights from a departmental cohort of 9 HEHE cases. Through this, we hope to shed light on potential clinical outcomes and therapeutic strategies for HEHE. Methods: Using SEER data from 22 registries, we analyzed 313 liver cancer patients with ICD-O-3 9130 and 9133 histology. Twelve variables were examined using Cox regression and mlr3 machine learning. Significant variables were identified and compared. Clinical data, imaging characteristics, and treatment methods of nine patients from our cohort were also presented. Result: In univariate and multivariate Cox regression analyses, Age, Sex, Year of diagnosis, Surgery of primary site, Chemotherapy, and Median household income were closely related to survival outcomes. Among the ten survival-related machine learning models, CoxPH, Flexible, Mboost, and Gamboost stood out based on Area Under the Curve(AUC), Decision Curve Analysis(DCA), and Calibration Curve Metrics. In the feature importance analysis of these four selected models, Age and Surgery of primary site were consistently identified as the most critical factors influencing prognosis. Additionally, the clinical data of nine patients from our cohort not only demonstrated unique imaging characteristics of HEHE but also underscored the importance of surgical intervention. Conclusion: For patients with resectable HEHE, surgical treatment is currently a highly important therapeutic approach. [ABSTRACT FROM AUTHOR]

Backgrounds: Primary hepatic epithelioid hemangioendothelioma (HEHE) is a rare neoplasm of vascular origin with varying biologic behavior, making it challenging to diagnose. Case presentation: We present a case of synchronous hepatocellular carcinoma (HCC) and HEHE in a 43-year-old Chinese male patient. Multiple hypoechoic liver lesions were depicted, but no specific imaging findings were detected on enhanced computed tomography (CT) or contrast-enhanced magnetic resonance imaging (MRI). The patient then underwent [18F]-FDG PET/CT, [11C]-acetate PET/CT, and [68Ga]Ga-FAPI-04 PET/CT. The HEHE lesions demonstrated no uptake on both 18F-FDG and 11C-acetate PET/CT imaging, but presented a clear visualization in [68Ga]Ga-FAPI-04 PET/CT. The largest lesion located in segment VII was finally diagnosed as HCC, while the other smaller ones were diagnosed as HEHE, which was confirmed by immunohistochemical staining for CD31. To the best of our knowledge, only 2 cases have been reported in the worldwide literature, and the first case undertook both 11C-acetate and [68Ga]Ga-FAPI-04 PET/CT instead of 18F-FDG PET/CT. Conclusion: In this report, we show that HCC and HEHE may occur synchronously, and HEHE should be considered when liver lesions are detected. [68Ga]Ga-FAPI-04 PET/CT has great potential in the detection, staging and therapy selection of HEHE. [ABSTRACT FROM AUTHOR]

Abstract Background Hepatic epithelioid hemangioendothelioma (HEHE) is a malignant vascular tumor known for its rarity. The different types of this hepatic tumor (single, multiple-nodular or diffused) indicate different prognosis and treatment plans. However, the heterogenic clinical manifestation creates a dilemma and a wide range of challenges when attending to HEHE patients. This review addresses the unique profile and clinical challenges that complicate the diagnosis and treatment of HEHE while focusing on current therapeutic strategies and their limitations. Main text The unclear etiology is a challenging feature of HEHE. The exact involvement of potential risk factors and mechanism of development are still undefined. Relevant genetic alterations like WWTR1–CAMTA1 fusion have been investigated; however, they are only applicable as diagnostic markers and their influence on therapeutic efficacy is largely unknown. Other characteristics include asymptomatic manifestation, lack of unique hepatic functional alterations, high rates of misdiagnosis and late-stage identification when metastases already exist. Currently, tissue biopsy is the main tool to establish a definite diagnosis but is challenged with the limited awareness to suspect HEHE at early stages and the lack of relevant guidelines due to the rarity and the insufficiency of relevant research. The absence of treatment guidelines is the greatest challenge of HEHE. Generally, Surgical approaches are recommended due to the benefits of prolonged survival and enhanced prognosis. Nonetheless, only a minority of patients are eligible for resections while liver transplants are faced with severe insufficiency of donor organs and long wait-lists. On the other hand, a variety of non-surgical treatments (like anti-angiogenic agents, interferon alpha-2B and sirolimus) are presented with a promising potential. However, relevant studies are challenged with limited sample-sizes and lack of prospective designs. Conclusion Regardless to decades passing since its discovery, HEHE still creates a dilemma due to its challenging clinical profile and lack of treatment guidelines. Raising awareness of HEHE in clinical practices improves the ability to diagnose this rare tumor at early stages and develop stronger research strategies and treatment guidelines to regulate the medical care provided to HEHE patients.

BackgroundEpithelioid hemangioendothelioma (EHE), is an uncommon, intermediate-grade malignant vascular tumor that can manifest in diverse organs, including the liver, lungs, and bones. Given its unique malignancy profile and rarity, there lacks a consensus on a standardized treatment protocol for EHE, particularly for hepatic epithelioid hemangioendothelioma (HEHE). This study aims to elucidate factors influencing the clinical prognosis of EHE by analyzing data from the SEER database, complemented with insights from a departmental cohort of 9 HEHE cases. Through this, we hope to shed light on potential clinical outcomes and therapeutic strategies for HEHE.MethodsUsing SEER data from 22 registries, we analyzed 313 liver cancer patients with ICD-O-3 9130 and 9133 histology. Twelve variables were examined using Cox regression and mlr3 machine learning. Significant variables were identified and compared. Clinical data, imaging characteristics, and treatment methods of nine patients from our cohort were also presented.ResultIn univariate and multivariate Cox regression analyses, Age, Sex, Year of diagnosis, Surgery of primary site, Chemotherapy, and Median household income were closely related to survival outcomes. Among the ten survival-related machine learning models, CoxPH, Flexible, Mboost, and Gamboost stood out based on Area Under the Curve(AUC), Decision Curve Analysis(DCA), and Calibration Curve Metrics. In the feature importance analysis of these four selected models, Age and Surgery of primary site were consistently identified as the most critical factors influencing prognosis. Additionally, the clinical data of nine patients from our cohort not only demonstrated unique imaging characteristics of HEHE but also underscored the importance of surgical intervention.ConclusionFor patients with resectable HEHE, surgical treatment is currently a highly important therapeutic approach.

Purpose: To investigate and compare the ultrasonic features of hepatic epithelioid hemangioendothelioma (HEHE) and other common hepatic malignancies, such as hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (ICC) and hepatic metastatic tumor (HMT). Methods: A total of 37 patients with pathologically proven HEHE, 37 HCC cases, 37 ICC cases, and 37 HMT cases were enrolled from single hospital. The clinical characteristics and ultrasonic features of all cases were summarized and statistically analyzed. Results: There were significant differences in sex and age between the HEHE group and other three groups (P < 0.001). The probability of HEHE infection with hepatitis B virus was lower than that of HCC and ICC groups (P < 0.05). The probability of elevated serum tumor markers in HEHE was significantly lower than that in the other three groups (P < 0.05). On conventional ultrasound (CUS), the probability of multiple lesions in HEHE was significantly higher than that in the other three groups (P < 0.05). On contrast-enhanced ultrasound (CEUS), the time to wash out in HEHE was significantly shorter than that of the other three groups (P < 0.001). The proportion of synchronous or slow enhancement in HEHE was significantly higher than that of the other three groups (P < 0.001). The proportion of HEHE with iso- or hypo-enhancement was significantly higher than in HCC and HMT groups (P < 0.05). Conclusion: HEHE mainly performed multiple hypoechoic lesions on CUS and displayed greater odds of synchronous enhancement in arterial phase, iso- or hypo-enhancement in peak time and wash out more quickly on CEUS, which allowed for differentiation from other common malignant tumors. [ABSTRACT FROM AUTHOR]

Background: Primary hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare tumour of vascular origin with an incidence of

Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely uncommon tumor of the liver. It typically lacks recognizable clinical signs and is diagnosed with the aid of imaging and histopathology combined with immunohistochemical analysis. We discuss the case of a 40-year-old woman with HEHE. The aim of this case report and literature review is to increase doctors’ knowledge of HEHE and reduce the incidence of missed clinical diagnoses.

Objectives: The aim of the present studywas to describe the experience at a single institution in the management of hepatic epithelioid hemangioendothelioma (HEHE). Methods: We included 51 patients with histologically confirmed HEHE. We performed log-rank (Cox--Mantel) survival analyses using Kaplan--Meier methods to test differences in survival between patients in different groups. Univariate Cox regression analyses and multivariate proportional hazards regression model were carried out to identify independent prognostic factors. Results: Different imaging modalities were used to diagnose HEHE with various presentations. Liver resection (LR), liver transplantation (LT), systemic treatment (ST), and surveillance had been used in our study. A significant difference was noted between the LR group and the surveillance group with respect to mean survival (p = 0.006), as was in the LR group and the ST group (p = 0.036), and in surgical approach (LR and LT) and nonsurgical approach (ST and surveillance) (p = 0.008). The mean survival between the ST group and the surveillance group was not significantly different (p = 0.851). LR (p = 0.010) and surgical approach (p = 0.014) were favorable predictors of outcome, while macrovascular invasion (MaVI) (p = 0.037), lung metastasis (p = 0.040), and surveillance (p = 0.033) were poor prognostic factors in univariate analysis. Multivariate analysis showed that LR (p = 0.010) and surgical approach (p = 0.014) were independently associated with good OS, while surveillance (p = 0.033) was independently associated with poor OS. After adjusting for confounding factors, patients in the LR group have much better OS than those in the surveillance group (p = 0.013). However, there was no significant difference in OS between the LR group and ST group (p = 0.254), as was in the ST group and the surveillance group (p = 0.857). Conclusions: The definitive diagnosis of HEHE was dependent on histopathology, and it was not possible to make a specific diagnosis without biopsy because the radiological findings were similar to those in some hepatic malignancies. ST was not recommended for patients who were not candidates for surgical approaches, and surgical approaches should be warranted regardless of disease stage. The retrospective nature and the small size of the data limited the generalizability of the study, designing a worldwide database that contains all data about patients with HEHE independent of their therapy, which was highly recommended. [ABSTRACT FROM AUTHOR]

Key points 1. CT appearances of HEH differed significantly among lesions of different sizes. 2. HEH lesions were categorized into three types: nodular, locally and diffusely coalescent. 3. CT characteristics of HEH variated greatly among the three lesion types.

Background: Hepatic epithelioid hemangioendothelioma (HEH) is extremely rare, and CT features have never been analyzed in a large group of patients. Methods: A retrospective study was designed to review the contrast-enhanced CT images of HEH patients. Intrahepatic lesions were categorized into three types: nodular, locally coalescent (coalescent lesion contained in one segment) or diffusely coalescent (coalescent lesion occupied more than one segment). CT features were compared among lesions of different sizes and patients with different lesion types. Results: A total of 93 HEH patients were included in this study, and 740 lesions were analyzed. The results of per-lesion analysis showed that medium lesions (2–5 cm) had the highest rate of lollipop sign (16.8%) and target-like enhancement (43.1%), while lesions in large group (> 5 cm) had the highest rate of capsular retraction (38.8%) and vascular invasion (38.8%). The differences on enhancement pattern and the rates of lollipop sign and capsular retraction were significant among lesions of different sizes (p < 0.001, respectively). The results of per-patient analysis showed that patients in locally coalescent group had the highest rates of lollipop sign (74.3%) and target sign (94.3%). All patients in diffusely coalescent group had capsular retraction and vascular invasion. CT appearances of capsular retraction, lollipop sign, target sign and vascular invasion differed significantly among patients with different lesion types (p < 0.001, p = 0.005, p = 0.006 and p < 0.001, respectively). Conclusion: CT features variated among HEH patients with different lesion types, and radiological appearances of HEH should be classified into nodular type, locally coalescent type and diffusely coalescent type. Key points: CT appearances of HEH differed significantly among lesions of different sizes. HEH lesions were categorized into three types: nodular, locally and diffusely coalescent. CT characteristics of HEH variated greatly among the three lesion types. [ABSTRACT FROM AUTHOR]

Abstract Background Hepatic epithelioid hemangioendothelioma (HEH) is extremely rare and the MRI features have never been investigated in a large group of patients. Methods A retrospective study was designed to review the MRI images of HEH patients. Two radiologists separately evaluated signal intensity (SI) on unenhanced imaging, morphological features, contrast-enhancement pattern at dynamic study. The MRI features were compared between patients with HEH and hepatic metastatic tumor (HMT). Results Fifty-seven HEH patients were included in this study and a total of 412 lesions were evaluated. On per-lesion analysis, the rate of coalescent lesion and subcapsular lesion were 18.2% and 39.8%, respectively. Capsular retraction and lollipop sign were observed in 47 lesions (11.4%) and 60 lesions (14.6%), respectively. Large lesions (> 5 cm) had the highest rate of coalescent lesion, subcapsular lesion, capsular retraction and lollipop sign. Target sign appeared in 196 lesions (47.6%) on T2 weighted (T2W) and 146 lesions (35.4%) on portal phase. Medium lesions (2–5 cm) had the highest rate of target sign on both T2W (72.9%) and portal phase (55.2%). On per-patient analysis, compare with HEH patients, HMT patients seldom had the appearance of lollipop sign (66.7% versus 6.4%, p

Epithelioid hemangioendothelioma is a low-grade malignant tumor of vascular origin. The rarity of hepatic epithelioid hemangioendothelioma (HEHE) makes the diagnosis and treatment of this entity challenging. We report a case of a 69-year-old female patient who suffered from HEHE and complained of abdominal distension pain with dizziness and appetite loss for more than half a month. Enhanced computed tomography of the upper abdomen indicated multiple space-occupying lesions in the liver. The pathological results of color ultrasound puncture suggested HEHE. We performed transcatheter arterial chemoembolization and relevant examinations according to the patient's condition and their choice. We followed the patient for 5 years and found that she developed recurrent intrahepatic metastasis of the tumor. Computed tomography was performed again after 3 months of treatment with anlotinib and the tumor did not show any progression. HEHE is a relatively rare hepatic malignant tumor derived from vascular endothelial cells, with a low incidence, atypical clinical manifestations, and a difficult diagnosis that can only be confirmed with pathological results. Currently, appropriate treatment methods should be selected according to the specific conditions of the patient., Competing Interests: Declaration of conflicting interestThe authors declare that there is no conflict of interest.

Background: Hepatic epithelioid hemangioendothelioma (HEH) is extremely rare and the MRI features have never been investigated in a large group of patients. Methods: A retrospective study was designed to review the MRI images of HEH patients. Two radiologists separately evaluated signal intensity (SI) on unenhanced imaging, morphological features, contrast-enhancement pattern at dynamic study. The MRI features were compared between patients with HEH and hepatic metastatic tumor (HMT). Results: Fifty-seven HEH patients were included in this study and a total of 412 lesions were evaluated. On per-lesion analysis, the rate of coalescent lesion and subcapsular lesion were 18.2% and 39.8%, respectively. Capsular retraction and lollipop sign were observed in 47 lesions (11.4%) and 60 lesions (14.6%), respectively. Large lesions (> 5 cm) had the highest rate of coalescent lesion, subcapsular lesion, capsular retraction and lollipop sign. Target sign appeared in 196 lesions (47.6%) on T2 weighted (T2W) and 146 lesions (35.4%) on portal phase. Medium lesions (2–5 cm) had the highest rate of target sign on both T2W (72.9%) and portal phase (55.2%). On per-patient analysis, compare with HEH patients, HMT patients seldom had the appearance of lollipop sign (66.7% versus 6.4%, p < 0.01), capsular retraction (59.6% versus 3.2%, p < 0.01) and target appearance on both T2Wand portal phase (64.9% versus 12.7%, p < 0.01). Conclusion: MRI features of HEH correlated with the lesion size. Capsular retraction, lollipop sign and coexistence of target sign on both T2W and portal phase were relatively specific MRI features of HEH, which could be helpful in suggesting the diagnosis. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to analyze the patterns of conventional ultrasound (CUS) and contrast-enhanced ultrasound (CEUS) in 20 patients with diagnosis of hepatic epithelioid hemangioendothelioma (HEHE). Methods: Twenty patients (12 females and 8 males) with mean age of 43.6 ± 13.6 years were included in this study from January 2012 to May 2020. CUS, CEUS, computed tomography (CT) and magnetic resonance imaging (MRI) features of the twenty patients with histologically proven HEHE were retrospectively reviewed by two radiologists. The clinical manifestations and the pathological findings of all patients with HEHE are described. Results: There were 3 types of HEHE in imaging, including single nodular (8/20, 40%), multifocal nodular (10/20, 50%), and diffuse type (2/20, 10%). The mean size of lesions was 4.2 ± 2.6 cm. B-mode ultrasound of HEHE showed hypoechoic (15/20, 75%), heterogeneous echogenicity (4/20, 20%), or hyperechoic (1/20, 5%) lesions with regular shape (18/20, 90%) near the liver capsule (17/20, 85%), and occasionally with a halo (4/20, 20%) and calcifications (3/20, 15%). Eight out of the 20 patients also had CEUS. On CEUS, HEHE demonstrated peripheral rim-like (5/8, 62.5%), heterogeneous (2/8, 25%), or homogeneous (1/8, 12.5%) hyperenhancement in the arterial phase. All patients (8/8, 100%) showed hypoenhancement in the portal and late phase. CEUS detected more lesions than CUS in 3 patients (3/8, 37.5%). In addition, central irregular unenhanced zones were observed in 6 patients (6/8, 75%). On contrast-enhanced CT or MRI, most cases presented with capsule retraction sign and lollipop sign. Conclusions: HEHE demonstrated specific findings on ultrasound, which includes multifocal hypoechoic lesions in a subcapsular distribution with typical enhancement characteristics of malignant hepatic tumors. [ABSTRACT FROM AUTHOR]

Abstract Background Optimal treatment for patients with hepatic epithelioid hemangioendothelioma (HEHE) remains unclear. Laparoscopic repeat liver resection (LR) is a minimally invasive and potentially effective surgical option for multiple HEHEs. Case presentation A 42-year-old woman with no relevant history was admitted for multiple liver tumors. Six tumors were observed on T2-weighted magnetic resonance imaging (MRI) including one in S2, two in S3, two in S7, and one in S8. Pathological evaluation of percutaneous tumor biopsy tissue suggested a diagnosis of HEHE and laparoscopic LR was planned. The procedure began with partial resection of S7 and partial resection of S8 and left lateral sectionectomy were performed. Another tumor was found intraoperatively on the surface of S6, necessitating removal by partial resection. Pathological evaluation of the resected tumor tissue from all seven tumors concurred with that of the preoperative biopsy. The patient was discharged on postoperative day 6 without any complications. A follow-up MRI 15 months after the primary surgery revealed one tumor each in S4, S6, and S8. Laparoscopic repeat LR was performed. The patient was discharged on postoperative day 5 without any complications. All three recurrent tumors were pathologically confirmed as HEHEs. Conclusions We successfully treated primary and recurrent HEHEs with laparoscopic LR, which is a reasonable minimally invasive procedure considering the possibility of multiple courses of liver surgery in patients with HEHE.

Abstract 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.

ObjectivesThis study aimed to analyze the patterns of conventional ultrasound (CUS) and contrast-enhanced ultrasound (CEUS) in 20 patients with diagnosis of hepatic epithelioid hemangioendothelioma (HEHE).MethodsTwenty patients (12 females and 8 males) with mean age of 43.6 ± 13.6 years were included in this study from January 2012 to May 2020. CUS, CEUS, computed tomography (CT) and magnetic resonance imaging (MRI) features of the twenty patients with histologically proven HEHE were retrospectively reviewed by two radiologists. The clinical manifestations and the pathological findings of all patients with HEHE are described.ResultsThere were 3 types of HEHE in imaging, including single nodular (8/20, 40%), multifocal nodular (10/20, 50%), and diffuse type (2/20, 10%). The mean size of lesions was 4.2 ± 2.6 cm. B-mode ultrasound of HEHE showed hypoechoic (15/20, 75%), heterogeneous echogenicity (4/20, 20%), or hyperechoic (1/20, 5%) lesions with regular shape (18/20, 90%) near the liver capsule (17/20, 85%), and occasionally with a halo (4/20, 20%) and calcifications (3/20, 15%). Eight out of the 20 patients also had CEUS. On CEUS, HEHE demonstrated peripheral rim-like (5/8, 62.5%), heterogeneous (2/8, 25%), or homogeneous (1/8, 12.5%) hyperenhancement in the arterial phase. All patients (8/8, 100%) showed hypoenhancement in the portal and late phase. CEUS detected more lesions than CUS in 3 patients (3/8, 37.5%). In addition, central irregular unenhanced zones were observed in 6 patients (6/8, 75%). On contrast-enhanced CT or MRI, most cases presented with capsule retraction sign and lollipop sign.ConclusionsHEHE demonstrated specific findings on ultrasound, which includes multifocal hypoechoic lesions in a subcapsular distribution with typical enhancement characteristics of malignant hepatic tumors.

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Hepatic angiosarcoma (AS) and hepatic epithelioid hemangioendothelioma (HEHE) are rare primary hepatic vascular malignancies (PHVM) that remain poorly understood. To guide management, we sought to identify factors and trends predicting survival after surgical intervention using a national database. In a retrospective analysis of the National Cancer Database patients with a diagnosis of PHVM were identified. Clinicopathologic factors were extracted and compared. Overall survival (OS) was estimated and predictors of survival were identified. Three hundred ninty patients with AS and 216 with HEHE were identified. Only 16% of AS and 36% of HEHE patients underwent surgery. The median OS for patients who underwent surgical intervention was 97 months, with 5-year OS of 30% for AS versus 69% for HEHE patients (P < 0.001). Tumor biology strongly impacted OS, with AS histology (Hazard Ratio [HR] of 3.61 [1.55-8.42]), moderate/poor tumor differentiation (HR = 3.86 [1.03-14.46]) and tumor size (HR = 1.01 [1.00-1.01]) conferring worse prognosis. The presence of metastatic disease in the surgically managed cohort (HR = 5.22 [2.01-13.57]) and involved surgical margins (HR = 3.87 [1.59-9.42]), were independently associated with worse survival. In this national cohort of PHVM, tumor biology, in the form of angiosarcoma histology, tumor differentiation and tumor size, was strongly associated with worse survival after surgery. Additionally, residual tumor burden after resection, in the form of positive surgical margins or the presence of metastasis, was also negatively associated with survival. Long-term clinical outcomes remain poor for patients with the above high-risk features, emphasizing the need to develop effective forms of adjuvant systemic therapies for this group of malignancies. [ABSTRACT FROM AUTHOR]

Abstract Hepatic epithelioid hemangioendothelioma is a rare neoplasm with a variable malignant potential and a high risk of recurrence. No general treatment guidelines have been established. Fortunately, we were able to minimize immunosuppressant after liver transplantation because of a full HLA‐matched case. There was no recurrence 1 year after treatment.

The paper gives a clinical example of diagnosing hepatic epithelioid hemangioendothelioma. With allowance made for that the tumor occurs rarely (less than 1% of cases of all vascular tumors), the authors provide the clinical example to demonstrate the capabilities of imaging techniques, by applying an integrated approach.

Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor that is usually found incidentally during computed tomography. It is often misdiagnosed as other hepatic tumors due to its rarity and the lack of typical imaging features, and the final diagnosis requires histopathological examination. We hereby report a 48-year-old woman with hepatic epithelioid hemangioendothelioma. [ABSTRACT FROM AUTHOR]

Abstract Background Hepatic epithelioid hemangioendothelioma (HEHE) is a rare angiogenic tumor with no recognized effective treatment. Treatment options used worldwide include liver transplantation (LT), liver resection (LR), radiofrequency ablation (RFA), chemotherapy, and observation. The aim of this study was to describe the efficacy of different treatment options used for HEHE at our center. Methods The medical charts of 12 patients with HEHE (9 women and 3 men) who were diagnosed and treated at the First Affiliated Hospital of Zhejiang University, China, between January 2011 and December 2017 were retrospectively reviewed. Results The patients were diagnosed by postoperative histopathology or fine needle aspiration biopsy. Two patients with diffuse lesions received LT and were alive without recurrence at the last follow-up. Three patients received LR as the initial treatment, and all of them developed recurrence during the follow-up period. One patient received RFA and remained free of disease, while the remaining six patients opted for simple observation rather than treatment. One of the patients who received LR passed away because of tumor recurrence within 32 months after surgery; the other patients showed no significant disease activity after treatments for their recurrent lesions. As of April 2018, the mean follow-up duration was 39.6 ± 20.1 months (15–82 months). Conclusions There are multiple strategies for HEHE. Considering its indolent course, initial observation for assessment of the lesion behavior may aid in the selection of appropriate treatment. Surgery or LT is suitable for patients with disease progression during the observation period. However, our sample size was small, and further studies are required to gather more information that can aid in optimal treatment selection.

Hepatic EHE (epithelioid hemangioendothelioma) is an uncommon entity of vascular origin and a low-grade malignant tumor. Primary hepatic EHE is rare. These tumors can be multifocal at presentation like in the soft tissues, bones, brain, liver, and small intestine. First described by Weiss and Enzinger in 1982 as a malignant vascular neoplasm with indolent behavior. We report the case of a 23-year-old male, known case of chronic liver disease, who presented with incisional hernia following exploratory laparotomy 8 months back, performed for intestinal obstruction. Contrast-enhanced computed tomography abdomen revealed an incisional hernia with a large defect along with multiple lesions in the liver (suspected metastases) and peritoneal deposits and a few discrete lung nodules. Some areas of interloop collections were also noted. Biopsies were taken from the liver which revealed benign cirrhotic lesion. Relevant to the findings, multiple biopsies were performed and fluid was sent for evaluation. The liver biopsy came out positive for borderline vascular malignancy (epithelioid hemangioendothelioma). This was confirmed with the immunohistochemistry report. Epithelioid hemangioendothelioma occurs mostly in soft tissues of extremity and lungs. The involvement of the liver may be seen as metastasis or rarely as a primary tumor. The incidence of primary malignant hepatic hemangioendothelioma is about 0.1/100,000; the mean age at the time of diagnosis is 41.7 years, and male:female ratio is 2:3. Liver transplantation, hepatectomy, chemoembolization, radiotherapy, and chemotherapeutic agents are reported treatment regimens. Malignant EHE of liver presents as multiple hepatic nodules. Being locally aggressive, it can invade the peritoneum, gut, and lungs. Orthoptic liver transplantation appears to be the only remedy because of the multifocal nature of the disease. Partial hepatectomy is possible for localized tumors. [ABSTRACT FROM AUTHOR]

Background: Primary hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare tumour of vascular origin with an incidence of <0.1 cases per 100,000 people worldwide., Case Description: A 29-year-old female with the history of epigastric pain and unintentional weight loss (3 kg over six months) was referred for upper endoscopy. The examination was without visual pathological findings, but a rapid urease test was positive. First-line treatment with clarithromycin-containing triple therapy for Helicobacter pylori infection was given. After completion of eradication therapy, diffuse abdominal pain developed. An abdominal computed tomography (CT) showed multiple liver nodules. Three consecutive core liver biopsies were performed and were inconclusive. A subsequent surgical liver nodule resection was performed. Histopathology of the specimen revealed grade 2 hepatocellular carcinoma; bone scintigraphy was negative for metastasis. A multidisciplinary team (MDT) recommended giving the patient sorafenib, which was poorly tolerated. The histology was reviewed using immunohistochemistry staining at the request of the oncologist, which showed expression of CD31 and CD34. Based on clinical, morphological and immunohistochemistry findings, a diagnosis of hepatic epithelioid hemangioendothelioma was made. Based on the multidisciplinary team's findings, liver transplantation was indicated as the only curative treatment., Conclusion: Because of the rarity of this disease, combining clinical, radiological and histopathological methods as well as an MDT approach can help to reach the correct final diagnosis. As demonstrated in this clinical case, it is crucial to perform immunohistochemistry of a liver biopsy to confirm a HEHE diagnosis., Learning Points: Hepatic epithelioid hemangioendothelioma is a rare vascular tumour that is often misdiagnosed and mismanaged.This case emphasises the critical importance of interdisciplinary teamwork and the use of non-invasive and invasive techniques to achieve a definitive diagnosis., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2024.)

Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare malignancy of vascular origin. When most patients with HEHE are diagnosed, they have a diffuse nodular phenotype, which is usually unresectable. A single nodular phenotype is found in only a small proportion (13%) of patients, and most are reported to be located in the right lobe of the liver. Although the prognosis of HEHE is considered more favorable than that of other hepatic malignant tumors, the 5-year survival rate is reported to be 64% after treatment. Herein, we present an unusual case of resectable HEHE of a single nodular type in the left lobe of the liver. The patient survived 15 years without recurrence after the multimodal treatment of radical resection and postoperative chemotherapy with thalidomide. The aim of the present study is to emphasize that multimodal treatment of radical resection followed by chemotherapy with thalidomide may achieve a relatively good survival outcome in patients with resectable HEHE of a single nodular type.

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 (HEHE) is a low-to-intermediate-grade malignant mesenchymal tumor. The diagnostic and prognostic values of 2-[18F] fluoro-2-deoxy-D-glucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) to patients with HEHE have not been fully validated. Patient survival outcomes (including overall survival [OS] and progression-free survival [PFS]), lesions characteristics and semi-quantitative parameters, in terms of maximum standardized uptake value (SUVmax), peak SUV (SUVpeak), total lesion glycolysis (TLG) and metabolic tumor volume (MTV) on 18F-FDG PET/CT of 20 cases with HEHE were measured and analyzed. A total of 310 liver lesions were detected (excluding the diffuse-type lesions in 3 cases). Most lesions had higher SUVmax in delayed imaging than in early imaging (P = 0.013). Patients with multiple organs involved had higher death rate (P = 0.022), higher progression rate (P = 0.020), shorter OS (P = 0.011), larger lesion SUVmax (P = 0.048) and TLG (P = 0.033) than those with only liver involved. The area under curves (AUCs) from the receiver operating characteristic (ROC) curve analysis were 0.960, 0.949, 0.980 and 0.960 for SUVmax, SUVpeak, TLG and MTV, respectively, in predicting OS (P = 0.005, 0.008, 0.001 and 0.024, respectively). For predicting PFS, the AUCs were 0.791, 0.824, 0.857 and 0.813 (P = 0.036, 0.019, 0.010 and 0.024), respectively. Dual-time-point imaging may improve lesions detectability. Patients with multiple organ involved had worse prognosis. The higher SUVmax, SUVpeak, TLG and MTV of lesions, the worse prognosis of patients were found. [ABSTRACT FROM AUTHOR]

Background: Optimal treatment for patients with hepatic epithelioid hemangioendothelioma (HEHE) remains unclear. Laparoscopic repeat liver resection (LR) is a minimally invasive and potentially effective surgical option for multiple HEHEs. Case presentation: A 42-year-old woman with no relevant history was admitted for multiple liver tumors. Six tumors were observed on T2-weighted magnetic resonance imaging (MRI) including one in S2, two in S3, two in S7, and one in S8. Pathological evaluation of percutaneous tumor biopsy tissue suggested a diagnosis of HEHE and laparoscopic LR was planned. The procedure began with partial resection of S7 and partial resection of S8 and left lateral sectionectomy were performed. Another tumor was found intraoperatively on the surface of S6, necessitating removal by partial resection. Pathological evaluation of the resected tumor tissue from all seven tumors concurred with that of the preoperative biopsy. The patient was discharged on postoperative day 6 without any complications. A follow-up MRI 15 months after the primary surgery revealed one tumor each in S4, S6, and S8. Laparoscopic repeat LR was performed. The patient was discharged on postoperative day 5 without any complications. All three recurrent tumors were pathologically confirmed as HEHEs. Conclusions: We successfully treated primary and recurrent HEHEs with laparoscopic LR, which is a reasonable minimally invasive procedure considering the possibility of multiple courses of liver surgery in patients with HEHE. [ABSTRACT FROM AUTHOR]

A 58-year-old man was notified of liver tumors at a medical check up. Contrast-enhanced computed tomography of the abdomen revealed two low density lesions, 35 mm in diameter, in segment 5/8 and, 15 mm in diameter, in segment 6. Gadoxetic acid enhanced magnetic resonance images revealed multiple hypointensity small masses of varying sizes in both lobes of the liver on the hepatobiliary phase in addition to those two lesions. A malignancy could not be ruled out. We underwent partial liver resections for diagnosis and treatment. First of all, two small masses in segment 3 were resected and diagnosed as fibrous mass and nonmalignancy of frozen section analysis. From this result, we did not perform partial liver resections of other small masses in both lobes of the liver, but did only suspecting malignant lesion in segment 5/8 and segment 6. In histopathological examination, all resected masses including small mass in segment 3 were diagnosed with hepatic epithelioid hemangioendothelioma. We reported a case of hepatic epithelioid hemangioendothelioma which was very difficult to be diagnosed by both preoperative imaging and intraoperative frozen section analysis. [ABSTRACT FROM AUTHOR]

Hepatic epithelioid hemangioendothelioma is a rare neoplasm with a variable malignant potential and a high risk of recurrence. No general treatment guidelines have been established. Fortunately, we were able to minimize immunosuppressant after liver transplantation because of a full HLA‐matched case. There was no recurrence 1 year after treatment. [ABSTRACT FROM AUTHOR]

Purpose of Review: Liver tumors, excluding hepatocellular carcinoma and metastatic disease, are rare. However, it is important to understand how to distinguish these lesions from hepatocellular carcinoma. They run the spectrum of benign to malignant, some aggressive with relatively few therapeutic options. The goal of this paper is to review the most recent literature to provide current insights into diagnosis, treatment, and pathogenesis of these tumor types.Recent Findings: Recent literature has focused on oncogenomics and putative targets for therapeutic intervention. Several ongoing studies are elucidating molecular pathways and evaluating novel therapies in these rare tumors and we focus on these findings, particularly in intrahepatic cholangiocarcinoma and fibrolamellar HCC. While these advances are promising, surgical resection continues to be associated with the greatest survival benefit for rare malignant tumors of the liver.Summary: Clinicians must be aware of rare liver tumors to distinguish them from hepatocellular carcinoma and to develop a differential diagnosis in complicated or atypical presentations. In these rare tumors, advances in understanding tumor biology hold the promise of expanding diagnostic and therapeutic possibilities. [ABSTRACT FROM AUTHOR]

Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare malignancy of vascular origin. When most patients with HEHE are diagnosed, they have a diffuse nodular phenotype, which is usually unresectable. A single nodular phenotype is found in only a small proportion (13%) of patients, and most are reported to be located in the right lobe of the liver. Although the prognosis of HEHE is considered more favorable than that of other hepatic malignant tumors, the 5-year survival rate is reported to be 64% after treatment. Herein, we present an unusual case of resectable HEHE of a single nodular type in the left lobe of the liver. The patient survived 15 years without recurrence after the multimodal treatment of radical resection and postoperative chemotherapy with thalidomide. The aim of the present study is to emphasize that multimodal treatment of radical resection followed by chemotherapy with thalidomide may achieve a relatively good survival outcome in patients with resectable HEHE of a single nodular type. [ABSTRACT FROM AUTHOR]

Purpose: To examine the characteristic features of hepatic epithelioid hemangioendothelioma (HEH) on magnetic resonance imaging (MRI) using Gd-EOB-DTPA. Material and methods: 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 (SI) measured at the tumor center, periphery, and liver parenchyma were plotted against the dynamic phases. Differences in SI 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. Results: 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 SI of the center showed slow enhancement starting from the transitional phase, resulting in divergence between the two graphs throughout the entire dynamic study ( p < 0.05). Pathologically, the lesion center consisted of reduced cellularity with myxohyaline stroma and necrosis. Conclusion: Core pattern can be considered a new diagnostic sign of HEH. [ABSTRACT FROM AUTHOR]

A 72-year-old female was referred for further evaluation of epigastralgia. Abdominal contrast computed tomography revealed numerous tumors in the two lobes of the liver. Liver biopsy and immunohistochemical staining revealed that the tumor cells were positive for factor VIII-associated antigen, platelet endothelial cell adhesion molecule 1 and human hematopoietic progenitor cell antigen, concordant with a diagnosis of hepatic epithelioid hemangioendothelioma (HEH). To elucidate the etiology of HEH, particularly the microRNA (miRNA) profiles, tissue samples obtained from normal and tumor tissues were analyzed using a miRNA array system. A total of 14 miRNAs were significantly upregulated and 93 miRNAs were downregulated in the tumor tissues (P<0.01). Additionally, unsupervised hierarchical clustering analysis using Pearson's correlation revealed that the tumor tissues clustered separately from the normal tissues. The miRNA expression profile was analyzed in HEH and compared with angiosarcoma, which exhibits histology similar to HEH. Out of a total of 107 miRNAs, only miR-122-5p and miR-1290 demonstrated a differential expression pattern in angiosarcoma. Therefore, these miRNAs may be novel biological markers for the determination of a diagnosis of HEH in primary mesenchymal tumors of the liver. To the best of our knowledge, this study is the first report of a miRNA microarray analysis in HEH. [ABSTRACT FROM AUTHOR]

Objectives: The aim of the present study was to describe the experience at a single institution in the management of hepatic epithelioid hemangioendothelioma (HEHE)., Methods: We included 51 patients with histologically confirmed HEHE. We performed log-rank (Cox-Mantel) survival analyses using Kaplan-Meier methods to test differences in survival between patients in different groups. Univariate Cox regression analyses and multivariate proportional hazards regression model were carried out to identify independent prognostic factors., Results: Different imaging modalities were used to diagnose HEHE with various presentations. Liver resection (LR), liver transplantation (LT), systemic treatment (ST), and surveillance had been used in our study. A significant difference was noted between the LR group and the surveillance group with respect to mean survival ( p = 0.006), as was in the LR group and the ST group ( p = 0.036), and in surgical approach (LR and LT) and nonsurgical approach (ST and surveillance) ( p = 0.008). The mean survival between the ST group and the surveillance group was not significantly different ( p = 0.851). LR (p = 0.010) and surgical approach (p = 0.014) were favorable predictors of outcome, while macrovascular invasion (MaVI) (p = 0.037), lung metastasis (p = 0.040), and surveillance (p = 0.033) were poor prognostic factors in univariate analysis. Multivariate analysis showed that LR (p = 0.010) and surgical approach (p = 0.014) were independently associated with good OS, while surveillance (p = 0.033) was independently associated with poor OS. After adjusting for confounding factors, patients in the LR group have much better OS than those in the surveillance group (p = 0.013). However, there was no significant difference in OS between the LR group and ST group (p = 0.254), as was in the ST group and the surveillance group (p = 0.857)., Conclusions: The definitive diagnosis of HEHE was dependent on histopathology, and it was not possible to make a specific diagnosis without biopsy because the radiological findings were similar to those in some hepatic malignancies. ST was not recommended for patients who were not candidates for surgical approaches, and surgical approaches should be warranted regardless of disease stage. The retrospective nature and the small size of the data limited the generalizability of the study, designing a worldwide database that contains all data about patients with HEHE independent of their therapy, which was highly recommended., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Feng, Li, Huang and Xu.)