|
1
|
Marak JR, Raj G, Verma S, Gandhi A. Primary hepatic epithelioid hemangioendothelioma masquerading as metastases: A rare case report. Radiol Case Rep 2023; 18:3739-3747. [PMID: 37609067 PMCID: PMC10440529 DOI: 10.1016/j.radcr.2023.07.046] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2023] [Revised: 07/18/2023] [Accepted: 07/19/2023] [Indexed: 08/24/2023] Open
Abstract
Epithelioid hemangioendothelioma (EHE) of the liver is an extremely rare malignant tumor of vascular origin, representing less than 1 % of all vascular tumors. Nearly 260 cases have been reported in English literature. Radiologically it is seen as multifocal lesions. It can be seen at different sites like lungs, bones, lymph nodes, breasts, and soft tissue. Often it is misdiagnosed with metastases, cholangiocarcinoma, or angiosarcoma. No definite treatment protocol is available due to its rarity, however, these malignancies are treated by radical resection of the tumor or liver transplant and/or chemotherapy. Here we present a primary hepatic epithelioid hemangioendothelioma (HEHE) which was mimicking metastases in a 42-year-old male who was treated with chemotherapy and radiotherapy. Sadly the patient expired after 1 year of complete course of treatment. Imaging features can help to improve the diagnostic accuracy of this tumor.
Collapse
Affiliation(s)
- James R. Marak
- Department of Radiodiagnosis, Dr RMLIMS, Lucknow, Uttar Pradesh, 226010, India
| | - Gaurav Raj
- Department of Radiodiagnosis, Dr RMLIMS, Lucknow, Uttar Pradesh, 226010, India
| | - Shashwat Verma
- Department of Nuclear Medicine, Dr RMLIMS, Lucknow, Uttar Pradesh, 226010, India
| | - Ajeet Gandhi
- Department of Radiation Oncology, Dr RMLIMS, Lucknow, Uttar Pradesh, 226010, India
| |
Collapse
|
|
2
|
Feng L, Li M, Huang Z, Xu M. Hepatic epithelioid hemangioendothelioma-a single-institution experience with 51 cases. Front Oncol 2023; 13:1236134. [PMID: 37601682 PMCID: PMC10435877 DOI: 10.3389/fonc.2023.1236134] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2023] [Accepted: 07/17/2023] [Indexed: 08/22/2023] Open
Abstract
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.
Collapse
Affiliation(s)
- Lei Feng
- Division of Biliary Surgery, Department of General Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Manjie Li
- Radiology Department of West China Tianfu Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Zhuo Huang
- Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Mingqing Xu
- Division of Liver Surgery, Department of General Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| |
Collapse
|
|
3
|
Qiu T, Zhu D, Fu R, Luo Y, Ling W. Conventional Ultrasound and Contrast-Enhanced Ultrasound in Hepatic Epithelioid Hemangioendothelioma: Retrospective Evaluation in 20 Cases. Front Oncol 2022; 12:686650. [PMID: 35295996 PMCID: PMC8918488 DOI: 10.3389/fonc.2022.686650] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2021] [Accepted: 02/07/2022] [Indexed: 02/05/2023] Open
Abstract
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.
Collapse
Affiliation(s)
- Tingting Qiu
- Department of Medical Ultrasound, West China Hospital of Sichuan University, Chengdu, China
| | - Dongmei Zhu
- Department of Ultrasound, The Second Clinical Medical College of Jinan University (Shenzhen People’s Hospital), Shenzhen, China
- Department of Ultrasound, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
| | - Rong Fu
- Department of Medical Ultrasound, West China Hospital of Sichuan University, Chengdu, China
| | - Yan Luo
- Department of Medical Ultrasound, West China Hospital of Sichuan University, Chengdu, China
| | - Wenwu Ling
- Department of Medical Ultrasound, West China Hospital of Sichuan University, Chengdu, China
| |
Collapse
|
|
4
|
Wang W, Liu G, Hu P, Pang L, Gu T, Yu H, Luo R, Yang X, Shi H. Imaging characteristics and prognostic values of hepatic epithelioid hemangioendothelioma on 18F-FDG PET/CT. Clin Exp Med 2020; 20:557-567. [PMID: 32797371 DOI: 10.1007/s10238-020-00653-0] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2020] [Accepted: 08/01/2020] [Indexed: 02/07/2023]
Abstract
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.
Collapse
Affiliation(s)
- Weimin Wang
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University, No. 180 in Fenglin Road, Shanghai, 200032, People's Republic of China
| | - Guobing Liu
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University, No. 180 in Fenglin Road, Shanghai, 200032, People's Republic of China
| | - Pengcheng Hu
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University, No. 180 in Fenglin Road, Shanghai, 200032, People's Republic of China
| | - Lifang Pang
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University, No. 180 in Fenglin Road, Shanghai, 200032, People's Republic of China
| | - Taoying Gu
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University, No. 180 in Fenglin Road, Shanghai, 200032, People's Republic of China
| | - Haojun Yu
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University, No. 180 in Fenglin Road, Shanghai, 200032, People's Republic of China
| | - Rongkui Luo
- Department of Pathology, Zhongshan Hospital, Fudan University, No. 180 in Fenglin Road, Shanghai, 200032, People's Republic of China
| | - Xinrong Yang
- Department of Liver Surgery, Liver Cancer Institute, Zhongshan Hospital, Key Laboratory of Carcinogenesis and Cancer Invasion, Ministry of Education, Fudan University, No. 1609 in Xietu Road, Shanghai, 200032, People's Republic of China
| | - Hongcheng Shi
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University, No. 180 in Fenglin Road, Shanghai, 200032, People's Republic of China.
| |
Collapse
|
|
5
|
A Review of the Spectrum of Imaging Manifestations of Epithelioid Hemangioendothelioma. AJR Am J Roentgenol 2020; 215:1290-1298. [PMID: 32841059 DOI: 10.2214/ajr.20.22876] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
OBJECTIVE. The purpose of this article is to review the spectrum of imaging manifestations of epithelioid hemangioendothelioma across different organ systems and briefly describe its current treatment strategies. CONCLUSION. Epithelioid hemangioendothelioma is a rare, locally invasive neoplasm with metastatic potential. Although most commonly occurring in liver, lungs, and bones, it can also present at multiple other sites. Because of its nonspecific clinical and imaging manifestations, it is often misdiagnosed. The possibility of epithelioid hemangioendothelioma must be considered in the presence of a slowly growing mass that invades adjacent structures. Imaging can help plan percutaneous biopsy, detect sites of disease, and identify poor prognostic factors.
Collapse
|
|
6
|
Cieszanowski A, Anysz-Grodzicka A, Podgorska J, Jagielska B, Pałucki J. Magnetic Resonance Imaging of Uncommon Hepatic Mesenchymal Tumours: Haemangioendothelioma and Angiosarcoma. Curr Med Imaging 2020; 15:362-368. [PMID: 31989904 DOI: 10.2174/1573405614666180628160809] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2017] [Revised: 05/31/2018] [Accepted: 06/12/2018] [Indexed: 11/22/2022]
Abstract
BACKGROUND Primary Hepatic Epithelioid Haemangioendothelioma (HEHE) and Primary Hepatic Angiosarcoma (PHA) are rare mesenchymal tumours with different malignant potential. Whereas HEHE demonstrates low to intermediate malignant potential, PHA is an aggressive malignancy with poor prognosis. The knowledge of typical imaging features of these lesions may facilitate correct diagnosis; however, the ultimate diagnosis of HEHE and PHA is based on histopathologic examination. DISCUSSION The most typical findings helpful in diagnosing HEHE are: Presence of multiple, confluent nodules located at the liver periphery (in young to middle-aged woman), retraction of the liver capsule, marked hyperintensity on T2-weighted images, "target-sign" appearance, progressive centripetal contrast enhancement, and relatively high Apparent Diffusion Coefficient (ADC) values. More than ≥50% of nodules are hyper- or isointense on Hepatobiliary Phase (HBP) images. CONCLUSION The imaging features suggestive of PHA are: Occurrence of metastases (lungs, spleen) at the time of diagnosis, presence of a large dominant mass with smaller satellites, heterogeneity and areas of haemorrhage in a dominant mass, progressive contrast enhancement, slightly elevated ADC values as compared to other malignant liver tumours.
Collapse
Affiliation(s)
- Andrzej Cieszanowski
- 1st Department of Radiology, Maria Skłodowska-Curie Memorial Cancer Centre, Institute of Oncology, Ul. Roentgena 5, 02-781 Warszawa, Poland
| | - Agnieszka Anysz-Grodzicka
- 1st Department of Radiology, Maria Skłodowska-Curie Memorial Cancer Centre, Institute of Oncology, Ul. Roentgena 5, 02-781 Warszawa, Poland
| | - Joanna Podgorska
- 2nd Department of Clinical Radiology, Medical University of Warsaw, Ul. Banacha 1a, 02-097 Warszawa, Poland
| | - Beata Jagielska
- Department of Oncological Diagnosis and Cardio-Oncology, Maria Skłodowska-Curie Memorial Cancer Centre, Institute of Oncology, Ul. Roentgena 5, 02-781 Warszawa, Poland
| | - Jakub Pałucki
- 1st Department of Radiology, Maria Skłodowska-Curie Memorial Cancer Centre, Institute of Oncology, Ul. Roentgena 5, 02-781 Warszawa, Poland
| |
Collapse
|
|
7
|
Ganeshan D, Pickhardt PJ, Morani AC, Javadi S, Lubner MG, Elmohr MM, Duran C, Elsayes KM. Hepatic hemangioendothelioma: CT, MR, and FDG-PET-CT in 67 patients-a bi-institutional comprehensive cancer center review. Eur Radiol 2020; 30:2435-2442. [PMID: 32002639 DOI: 10.1007/s00330-019-06637-3] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/23/2019] [Revised: 11/26/2019] [Accepted: 12/16/2019] [Indexed: 12/12/2022]
Abstract
OBJECTIVE To evaluate the imaging features of hepatic epithelioid hemangioendothelioma (HEH) on multiphasic CT, MR, and FDG-PET-CT. METHODS Bi-institutional review identified 67 adults (mean age, 47 years; 23 M/44 F) with pathologically proven HEH and pretreatment multiphasic CT (n = 67) and/or MR (n = 30) and/or FDG-PET-CT (n = 13). RESULTS HEHs were multifocal in 88% (59/67). Mean size of the dominant mass was 4.1 cm (range, 1.4-19 cm). The tumors were located in the peripheral, subcapsular regions of the liver in 96% (64/67). Capsular retraction was present in 81% (54/67 cases) and tumors were coalescent in 61% (41/67). HEH demonstrated peripheral ring enhancement on arterial phase imaging in 33% (21/64) and target appearance on the portal venous phase in 69% (46/67). Persistent peripheral enhancement on the delayed phase was seen in 49% (31/63). On MR, multilayered target appearance was seen on the T2-weighted sequences in 67% (20/30) and on the diffusion-weighted sequences in 61% (11/18). Target appearance on hepatobiliary phase of MRI was seen in 57% (4/7). On pre-therapy FDG-PET-CT, increased FDG uptake above the background liver parenchyma was seen in 62% (8/13). CONCLUSION HEHs typically manifest as multifocal, coalescent hepatic nodules in peripheral subcapsular location, with associated capsular retraction. Peripheral arterial ring enhancement and target appearance on portal venous phase are commonly seen on CT. Similarly, multilayered target appearance correlating with its histopathological composition is typically seen on multiple sequences of MR including T2-weighted, diffusion-weighted, and dynamic contrast-enhanced multiphasic MR. KEY POINTS • Hepatic epithelioid hemangioendotheliomas manifest on CT and MR as multifocal, coalescent hepatic nodules in peripheral subcapsular location, with associated capsular retraction. • Enhancement pattern on contrast-enhanced CT and MR can vary but peripheral ring enhancement on arterial phase and target appearance on portal venous phase are commonly seen. • Retrospective two-center study showed that cross-sectional imaging may help in the diagnosis.
Collapse
Affiliation(s)
- Dhakshinamoorthy Ganeshan
- Division of Diagnostic Imaging, Body Imaging Section, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030-4009, USA.
| | - Perry J Pickhardt
- Department of Radiology, University of Wisconsin School of Medicine and Public Health, E3/311 Clinical Sciences Center, 600 Highland Ave, Madison, WI, 53792, USA
| | - Ajaykumar C Morani
- Division of Diagnostic Imaging, Unit 1473, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030-4009, USA
| | - Sanaz Javadi
- Division of Diagnostic Imaging, Unit 1473, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030-4009, USA
| | - Meghan G Lubner
- Department of Radiology, University of Wisconsin School of Medicine and Public Health, E3/311 Clinical Sciences Center, 600 Highland Ave, Madison, WI, 53792, USA
| | - Mohab M Elmohr
- Department of Imaging Physics, The University of Texas MD Anderson Cancer Center, 3SCR2.3810, 1881 East Rd, Houston, TX, 77054, USA
| | - Cihan Duran
- Division of Diagnostic Imaging, Body Imaging Section, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030-4009, USA
- Department of Imaging Physics, The University of Texas MD Anderson Cancer Center, 3SCR2.3810, 1881 East Rd, Houston, TX, 77054, USA
| | - Khaled M Elsayes
- Division of Diagnostic Imaging, Body Imaging Section, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030-4009, USA
- Department of Imaging Physics, The University of Texas MD Anderson Cancer Center, 3SCR2.3810, 1881 East Rd, Houston, TX, 77054, USA
| |
Collapse
|
|
8
|
Virarkar M, Saleh M, Diab R, Taggart M, Bhargava P, Bhosale P. Hepatic Hemangioendothelioma: An update. World J Gastrointest Oncol 2020; 12:248-266. [PMID: 32206176 PMCID: PMC7081107 DOI: 10.4251/wjgo.v12.i3.248] [Citation(s) in RCA: 17] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2019] [Revised: 02/21/2020] [Accepted: 02/23/2020] [Indexed: 02/05/2023] Open
Abstract
Primary epithelioid hemangioendotheliomas of the liver (EHL) are rare tumors with a low incidence. The molecular background of EHL is still under investigation, with WWTR1-CAMPTA1 mutation may function as a tumor marker. Commonly, this tumor is misdiagnosed with angiosarcoma, cholangiocarcinomas, metastatic carcinoma, and hepatocellular carcinoma (sclerosing variant). Characteristic features on imaging modalities such as ultrasound, computed tomography, magnetic resonance imaging and positron emission tomography/computed tomography guide in diagnosis and staging. The "halo sign" and the "lollipop sign" on computed tomography and magnetic resonance imaging are described in the literature. Currently, there are no standardized guidelines for treating EHL with treatment options are broad including: chemotherapy, ablation, surgery and liver transplantation with inconsistent results.
Collapse
Affiliation(s)
- Mayur Virarkar
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Mohammed Saleh
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Radwan Diab
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Melissa Taggart
- Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Peeyush Bhargava
- Department of Radiology, The University of Medical Branch, Galveston, TX 77555, United States
| | - Priya Bhosale
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| |
Collapse
|
|
9
|
Epelboym Y, Engelkemier DR, Thomas-Chausse F, Alomari AI, Al-Ibraheemi A, Trenor CC, Adams DM, Chaudry G. Imaging findings in epithelioid hemangioendothelioma. Clin Imaging 2019; 58:59-65. [PMID: 31238187 DOI: 10.1016/j.clinimag.2019.06.002] [Citation(s) in RCA: 24] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2019] [Revised: 05/28/2019] [Accepted: 06/11/2019] [Indexed: 12/12/2022]
Abstract
PURPOSE-OBJECTIVE Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy with varying biologic behavior. The purpose of this study was to identify imaging findings most characteristic of EHE. METHODS Retrospective review of clinical and imaging records in patients referred to our Vascular Anomalies Center over a 17 year period with biopsy proven EHE. RESULTS We evaluated 29 patients (17 F) with median age of 16 years (range 2-76 y). The most common presenting symptoms were pain (n = 13) and palpable mass (n = 7). 22 (70%) had multifocal disease. Most common sites of involvement were lung (n = 25), liver (n = 16), bone (n = 12), soft tissue (n = 3) and lymph nodes (n = 1). Of patients with single site disease, 3 had lung, 3 liver, and 1 had bone lesions. In 18/25 with lung disease, there were multiple nodules of varying sizes and characteristics. In 14/16 with hepatic disease there were multiple nodules with predominantly peripheral distribution. Subcapsular retraction was seen in 10/16 and a "lollipop" sign (hepatic or portal vein tapering at the edge of a well-defined hypoenhancing lesion) identified in 5/16. Of 12 osseous lesions, 11 were lytic, 8 involved vertebrae and 9 involved the axial skeleton. CONCLUSION EHE has varied imaging findings. The most common sites are lungs, liver, and bone, with multi-organ involvement seen in most. Lung disease is most commonly characterized by multiple nodules. Hepatic lesions demonstrate the most distinctive findings, with peripheral distribution, lack of early enhancement, subcapsular retraction and "lollipop" sign. Osseous lesions are commonly lytic and more prevalent in the axial skeleton.
Collapse
Affiliation(s)
- Yan Epelboym
- Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, United States of America
| | - Dawn R Engelkemier
- Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, United States of America
| | - Frederic Thomas-Chausse
- Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, United States of America
| | - Ahmad I Alomari
- Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, United States of America; Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, United States of America
| | - Alyaa Al-Ibraheemi
- Department of Pathology, Boston Children's Hospital and Harvard Medical School, United States of America; Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, United States of America
| | - Cameron C Trenor
- Division of Hematology/Oncology, Boston Children's Hospital and Harvard Medical School, United States of America; Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, United States of America
| | - Denise M Adams
- Division of Hematology/Oncology, Boston Children's Hospital and Harvard Medical School, United States of America; Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, United States of America
| | - Gulraiz Chaudry
- Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, United States of America; Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, United States of America.
| |
Collapse
|
|
10
|
Cousin S, Le Loarer F, Crombé A, Karanian M, Minard-Colin V, Penel N. [Epithelioid hemangioendothelioma]. Bull Cancer 2018; 106:73-83. [PMID: 30527817 DOI: 10.1016/j.bulcan.2018.11.004] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/24/2018] [Revised: 10/28/2018] [Accepted: 11/01/2018] [Indexed: 12/14/2022]
Abstract
We describe herein the inaugural manifestations, the radiological and histological diagnosis criteria for and the outcome of epithelioid hemangioendothelioma (EHE). Most of EHE (90%) display a specific reciprocal chromosomic translocation t(1;3)(p36;q23-25), which is associated with the synthesis of fusion protein WWTR1/CAMTA1. EHE are low-grade vascular sarcomas. EHE could be initially localized or multifocal. At localized stage, EHE are best treated with focal treatments. At multifocal stage, the upfront strategy is watchful follow-up. Some multifocal EHE display very indolent course with spontaneous stable disease for years or decades. In case of progressive multifocal EHE, there is no consensual treatment. Diagnostic and clinical management of EHE requires interdisciplinary expertise from labeled centers.
Collapse
Affiliation(s)
- Sophie Cousin
- Institut Bergonié, département d'oncologie médicale, 229, cours de l'Argonne, 33000 Bordeaux, France
| | - François Le Loarer
- Institut Bergonié, département d'anatomopathologie, 229, cours de l'Argonne, 33000 Bordeaux, France
| | - Amandine Crombé
- Institut Bergonié, département d'imagerie médicale, 229, cours de l'Argonne, 33000 Bordeaux, France
| | - Marie Karanian
- Centre Léon Bérard, département d'anatomopathologie, 28, Promenade Léa et Napoléon Bullukian, 69008 Lyon, France
| | - Véronique Minard-Colin
- Institut Gustave Roussy, département d'oncologie pédiatrique, 114, rue Edouard-Vaillant, 94800 Villejuif, France
| | - Nicolas Penel
- Centre Oscar Lambret, département d'oncologie médicale, 3, rue Frédéric Combemale, 59000 Lille, France; Hôpital Claude Huriez, service d'oncologie médicale, rue Michel Polonowski, 59000 Lille, France.
| |
Collapse
|
|
11
|
Tan Y, Yang X, Dong C, Xiao Z, Zhang H, Wang Y. Diffuse hepatic epithelioid hemangioendothelioma with multiple splenic metastasis and delayed multifocal bone metastasis after liver transplantation on FDG PET/CT images: A case report. Medicine (Baltimore) 2018; 97:e10728. [PMID: 29851777 PMCID: PMC6392553 DOI: 10.1097/md.0000000000010728] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
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.
Collapse
Affiliation(s)
| | | | | | | | - Hongbo Zhang
- Department of Pathology, the Second Xiangya Hospital, Central South University, Changsha, Hunan, China
| | | |
Collapse
|
|
12
|
Imaging of Rare Primary Malignant Hepatic Tumors in Adults With Histopathological Correlation. J Comput Assist Tomogr 2017; 40:452-62. [PMID: 26938690 DOI: 10.1097/rct.0000000000000382] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Hepatocellular carcinoma and cholangiocarcinoma are the most common primary liver malignancies in adults (comprising >85%); however, liver is also host to some unusual primary malignant tumors. Some of these tumors show distinct demographic, clinicopathologic, and imaging features. Imaging features of these uncommon primary malignant liver tumors are presented with an attempt to correlate them with histopathology.
Collapse
|
|
13
|
Woo JH, Kim TJ, Lee KS, Kim TS, Kim BT. Epithelioid hemangioendothelioma in the thorax: Clinicopathologic, CT, PET, and prognostic features. Medicine (Baltimore) 2016; 95:e4348. [PMID: 27472721 PMCID: PMC5265858 DOI: 10.1097/md.0000000000004348] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Little is known about prognostic factors in epithelioid hemangioendothelioma (EHE). We aimed to identify prognostic factors among various clinicopathologic and imaging features of thoracic EHEs.Forty-two patients (male:female = 20:22; median age, 49 years) of EHEs with (n = 19) and without (n = 23) thoracic involvement were included. We reviewed electronic medical records for clinical information and computed tomography (CT) features for thoracic involvement. Differences in demographics and survival outcomes of patients with and without thoracic involvement were assessed. We also estimated overall survival.The most common pattern of thoracic involvement was multiple pulmonary nodules (n = 10), followed by parenchymal tumor with pleural invasion (n = 4), reticulonodular opacities (n = 3), and diffuse pleural thickening (n = 2). No significant difference in survival was found between the thoracic EHE group and nonthoracic EHE group (P = 0.68). Among 4 different thoracic involvement types, the lung multinodular pattern tended to demonstrate longer median survival (8.5 months) than other patterns, whereas the shortest median survival (1 month) was observed for the nodule/mass with pleural involvement pattern (P = 0.038).CT manifestations of thoracic EHEs are classified into 4 patterns, of which lung multinodular pattern is associated with longer survival. Survival is not different between patients with and without thoracic involvement.
Collapse
Affiliation(s)
- Jung Han Woo
- Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine
| | - Tae Jung Kim
- Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine
| | - Kyung Soo Lee
- Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine
- Correspondence: Kyung Soo Lee, Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine (SKKU-SOM), 50, Ilwon-Dong, Kangnam-Gu, Seoul 135-710, Korea (e-mail: )
| | - Tae Sung Kim
- Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine
| | - Byung-Tae Kim
- Department of Nuclear Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| |
Collapse
|
|
14
|
Dong Y, Wang WP, Cantisani V, D’Onofrio M, Ignee A, Mulazzani L, Saftoiu A, Sparchez Z, Sporea I, Dietrich CF. Contrast-enhanced ultrasound of histologically proven hepatic epithelioid hemangioendothelioma. World J Gastroenterol 2016; 22:4741-4749. [PMID: 27217705 PMCID: PMC4870080 DOI: 10.3748/wjg.v22.i19.4741] [Citation(s) in RCA: 30] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/08/2016] [Revised: 03/15/2016] [Accepted: 04/07/2016] [Indexed: 02/06/2023] Open
Abstract
AIM To analyze contrast-enhanced ultrasound (CEUS) features of histologically proven hepatic epithelioid hemangioendothelioma (HEHE) in comparison to other multilocular benign focal liver lesions (FLL). METHODS Twenty-five patients with histologically proven HEHE and 45 patients with histologically proven multilocular benign FLL were retrospectively reviewed. Four radiologists assessed the CEUS enhancement pattern in consensus. RESULTS HEHE manifested as a single (n = 3) or multinodular (n = 22) FLL. On CEUS, HEHE showed rim-like (18/25, 72%) or heterogeneous hyperenhancement (7/25, 28%) in the arterial phase and hypoenhancement (25/25, 100%) in the portal venous and late phases (PVLP), a sign of malignancy. Eighteen patients showed central unenhanced areas (18/25, 72%); in seven patients (7/25, 28%), more lesions were detected in the PVLP. In contrast, all patients with hemangioma and focal nodular hyperplasia showed hyperenhancement as the most distinctive feature (P < 0.01). CONCLUSION CEUS allows for characterization of unequivocal FLL. By analyzing the hypoenhancement in the PVLP, CEUS can determine the malignant nature of HEHE.
Collapse
|
|
15
|
Gan LU, Chang R, Jin H, Yang LI. Typical CT and MRI signs of hepatic epithelioid hemangioendothelioma. Oncol Lett 2016; 11:1699-1706. [PMID: 26998064 PMCID: PMC4774430 DOI: 10.3892/ol.2016.4149] [Citation(s) in RCA: 39] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/09/2015] [Accepted: 01/12/2016] [Indexed: 02/06/2023] Open
Abstract
To investigate the typical magnetic resonance imaging (MRI) and computed tomography (CT) features of hepatic epithelioid hemangioendothelioma (HEH), the CT and MRI findings of 14 histopathologically confirmed cases of HEH were retrospectively analyzed. Non-contrast and dynamic contrast-enhanced scans were conducted in all cases. A total of 229 lesions were detected in the 14 cases. All cases were classified as one of three types: (i) Solitary nodular type (1 case, 7%); (ii) multifocal nodular type (11 cases, 79%); or (iii) diffuse type (2 cases, 14%). The diameter of the lesions ranged from 5 to 105 mm. For the first two types (solitary and multifocal nodular types), the CT findings included low density lesions with clear margins on non-contrast scans, centripetal enhancement in arterial phase, and homogeneous enhancement in the portal venous and delay phases. The findings of non-contrast MRI scans for these two types included low signal intensity on T1-weighted images, heterogeneous high signal intensity on T2-weighted images, and heterogeneous high signal intensity on diffusion-weighted images. The lesions were predominantly located in submarginal areas. On contrast-enhanced MRI, the findings for the first two types included peripheral ring-like enhancement with a central low signal intensity (‘black target-like’ sign) and a central enhanced core surrounded by a low signal intensity halo (‘white target-like’ sign). The findings for the third HEH type (diffuse type) on CT and MRI scans included low density or heterogeneous signal intensity lesions involving regions of part or the whole liver, coalescent lesions (‘strip-like’ sign), and gradual enhancement along central vessels (‘lollipop’ sign). Collectively, these findings indicate that the ‘white target-like’ sign, ‘black target-like’ sign, ‘lollipop’ sign and ‘strip-like’ sign, in addition to capsular contraction and submarginal location, on CT and MRI imaging may have implications for the diagnosis of HEH. Furthermore, a variety of MRI sequences may provide additional information for the differential diagnosis of HEH.
Collapse
Affiliation(s)
- L U Gan
- Department of Radiology, Chinese People's Liberation Army General Hospital, Beijing 100853, P.R. China
| | - Ruiping Chang
- Department of Radiology, Chinese People's Liberation Army General Hospital, Beijing 100853, P.R. China
| | - Hualan Jin
- Department of Radiology, Chinese People's Liberation Army General Hospital, Beijing 100853, P.R. China
| | - L I Yang
- Department of Radiology, Chinese People's Liberation Army General Hospital, Beijing 100853, P.R. China
| |
Collapse
|
|
16
|
Abstract
Vascular sarcomas are soft-tissue tumors that arise from the endothelium with a malignant potential. This review discusses the management of epithelioid hemangioendothelioma (EHE) and angiosarcoma. EHE is a vascular tumor of intermediate malignant potential with an indolent course. EHE arising from the liver, lung, or bone tends to be multifocal and the rate of progression is slow and often unpredictable. Treatment should be considered in patients with significant symptomatic deterioration and/or progressive disease on imaging studies. Various cytotoxic and targeted therapies are available for management, with disease stabilization as the most common outcome. Angiosarcoma is an aggressive vascular tumor with a high malignant potential. Multidisciplinary care is critical for the management of localized disease, and the best outcomes are often observed in patients when a combination of systemic and local therapy options is used. Metastatic angiosarcoma is treated primarily with systemic therapy, and several cytotoxic and targeted therapies are available, alone or in combination. The choice of therapy depends on several factors, such as cutaneous location of the tumor, performance status of the patient, toxicity of the treatment, and patient goals.
Collapse
Affiliation(s)
- Vinod Ravi
- Sarcoma Medical Oncology, University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 450, Houston, TX 77030, USA.
| | | |
Collapse
|
|
17
|
Abstract
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.
Collapse
|
|
18
|
Mistry AM, Gorden DL, Busler JF, Coogan AC, Kelly BS. Diagnostic and therapeutic challenges in hepatic epithelioid hemangioendothelioma. J Gastrointest Cancer 2013; 43:521-5. [PMID: 22544493 DOI: 10.1007/s12029-012-9389-y] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
BACKGROUND 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. METHODS 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.
Collapse
Affiliation(s)
- Akshitkumar M Mistry
- Department of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232-0275, USA.
| | | | | | | | | |
Collapse
|
|
19
|
Hsu CY, Liu YC, Li CP, Huang PH, Lin CH, Chao Y. Malignant hepatic epithelioid hemangioendothelioma with high-output heart failure: Successful management of heart failure with transcatheter arterial chemoembolization. Asia Pac J Clin Oncol 2013; 10:e118-21. [DOI: 10.1111/ajco.12056] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/26/2012] [Indexed: 12/13/2022]
Affiliation(s)
- Chien-Yi Hsu
- Division of Cardiology; Taipei Veterans General Hospital; Taipei Taiwan
- Cardiovascular Research Center; National Yang-Ming University; Taipei Taiwan
| | - Yao-Chung Liu
- Division of Hematology and Oncology; Department of Medicine; Taipei Veterans General Hospital; Taipei Taiwan
- Department of Medicine; Taipei City Hospital; Taipei Taiwan
| | - Chung-Pin Li
- Division of Gastroenterology; Taipei Veterans General Hospital; Taipei Taiwan
- School of Medicine; Taipei Taiwan
| | - Po-Hsun Huang
- Division of Cardiology; Taipei Veterans General Hospital; Taipei Taiwan
- Institute of Clinical Medicine; Taipei Taiwan
- Cardiovascular Research Center; National Yang-Ming University; Taipei Taiwan
| | - Chin-Hsuan Lin
- Departments of Pathology and Laboratory Medicine; Taipei Veterans General Hospital; Taipei Taiwan
| | - Yee Chao
- Cancer Center; Taipei Veterans General Hospital; Taipei Taiwan
- School of Medicine; Taipei Taiwan
| |
Collapse
|
|
20
|
Azzam RI, Alshak NS, Pham HP. AIRP best cases in radiologic-pathologic correlation: Hepatic epithelioid hemangioendothelioma. Radiographics 2012; 32:789-94. [PMID: 22582359 DOI: 10.1148/rg.323115010] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Affiliation(s)
- Ramzi I Azzam
- Department of Diagnostic Imaging, Kaiser Permanente Medical Center, 1505 Edgemont Ave, Los Angeles, CA 90027, USA.
| | | | | |
Collapse
|
|
21
|
Agrawal N, Parajuli S, Zhao P, Satoskar R, Laurin J, Azumi N, Matsumoto C, Shetty K. Liver transplantation in the management of hepatic epithelioid hemangioendothelioma: a single-center experience and review of the literature. Transplant Proc 2012; 43:2647-50. [PMID: 21911139 DOI: 10.1016/j.transproceed.2011.06.035] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2011] [Accepted: 06/03/2011] [Indexed: 12/12/2022]
Abstract
Hepatic epitheliod hemangioendothelioma (HEHE) is a rare tumor of vascular origin with unpredictable malignant potential. We describe our experience with four biopsy-proven HEHE cases that were considered for orthotopic liver transplant (OLT). Three patients had preserved hepatic function and despite extensive disease burden did not develop disease progression while awaiting OLT. We were able to utilize the review process allowed by United Network of Organ Sharing to obtain additional priority for OLT for these patients. This led to expedited organ allocation and excellent post-OLT outcomes.
Collapse
Affiliation(s)
- N Agrawal
- Georgetown Transplant Institute, Georgetown University Hospital, Washington, DC 20007, USA
| | | | | | | | | | | | | | | |
Collapse
|
|
22
|
Amin S, Chung H, Jha R. Hepatic epithelioid hemangioendothelioma: MR imaging findings. ACTA ACUST UNITED AC 2011; 36:407-14. [PMID: 21079951 DOI: 10.1007/s00261-010-9662-0] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
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.
Collapse
Affiliation(s)
- Sejal Amin
- Department of Radiology, Georgetown University Hospital, Washington, DC, USA
| | | | | |
Collapse
|
|
23
|
Hepatic epithelioid hemangioendothelioma: findings at CT and MRI including preliminary observations at diffusion-weighted echo-planar imaging. ACTA ACUST UNITED AC 2011; 36:415-24. [PMID: 20730424 DOI: 10.1007/s00261-010-9641-5] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
OBJECTIVE 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). METHODS 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. RESULTS 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. CONCLUSION 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.
Collapse
|
|
24
|
Thin LWY, Wong DD, De Boer BW, Ferguson JM, Adams L, Macquillan G, Delriviere L, Mitchell A, Jeffrey GP. Hepatic epithelioid haemangioendothelioma: challenges in diagnosis and management. Intern Med J 2011; 40:710-5. [PMID: 19712200 DOI: 10.1111/j.1445-5994.2009.02043.x] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
BACKGROUND Hepatic epithelioid haemangioendothelioma (HEH) is a rare, low grade malignant neoplasm of endothelial origin which is difficult to diagnose and has a variable outcome. We review five HEH cases from our centre with the aim of identifying clinical predictors of outcome and various therapeutic options. METHODS A search was made on the WA Liver Transplant registry for cases with histologically confirmed HEH. Their medical records were reviewed. A literature search was conducted through Medline using terms to compare the results from this series with those of other series. RESULTS Five patients were identified to have HEH. The mean age was 44.2years (range 34-53years). Four of five patients presented with dyspepsia and two patients had clinical evidence of portal hypertension with ascites. Two patients had radiologically diffuse disease and three patients had discrete nodular liver involvement. The mean duration from presentation of symptoms to diagnosis of HEH was 26.8months. Liver transplantation was performed in one patient with diffuse HEH who is alive with no disease recurrence at 3years. Three patients with radiologically stable disease followed with 6monthly surveillance imaging are currently alive and well. The median survival of all five patients was 5years (range 1.5-16years) at the time of follow up. CONCLUSIONS These results support the role of surveillance alone for patients with focal and radiologically stable disease. Patients with diffuse HEH with hepatic decompensation should be considered for transplantation. However, numbers are small and an international registry is required to make firm comparisons.
Collapse
Affiliation(s)
- L W Y Thin
- WA Liver Transplant Service, PathWest Laboratory Medicine and Radiology Department, Sir Charles Gairdner Hospital, and School of Medicine and Pharmacology, University of Western Australia, Perth, Western Australia.
| | | | | | | | | | | | | | | | | |
Collapse
|
|
25
|
Chen Y, Yu RS, Qiu LL, Jiang DY, Tan YB, Fu YB. Contrast-enhanced multiple-phase imaging features in hepatic epithelioid hemangioendothelioma. World J Gastroenterol 2011; 17:3544-53. [PMID: 21941423 PMCID: PMC3163254 DOI: 10.3748/wjg.v17.i30.3544] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2010] [Revised: 05/20/2011] [Accepted: 05/27/2011] [Indexed: 02/06/2023] Open
Abstract
AIM: To investigate and review the contrast-enhanced multiple-phase computed tomography (CEMP CT) and magnetic resonance imaging (MRI) findings in patients with pathologically confirmed hepatic epithelioid hemangioendothelioma (HEHE).
METHODS: Findings from imaging examinations in 8 patients (5 women and 3 men) with pathologically confirmed HEHE were retrospectively reviewed (CT images obtained from 7 patients and MR images obtained from 6 patients). The age of presentation varied from 27 years to 60 years (average age 39.8 years).
RESULTS: There were two types of HEHE: multifocal type (n = 7) and diffuse type (n = 1). In the multifocal-type cases, there were 74 lesions on CT and 28 lesions on MRI with 7 lesions found with diffusion weighted imaging; 18 (24.3%) of 74 lesions on plain CT and 26 (92.9%) of 28 lesions on pre-contrast MRI showed the target sign. On CEMP CT, 28 (37.8%) of 74 lesions appeared with the target sign and a progressive-enhancement rim and 9 (12.2%) of 74 lesions displayed progressive enhancement, maintaining a state of persistent enhancement. On CEMP MRI, 27 (96.4%) of 28 lesions appeared with the target sign with a progressive-enhancement rim and 28 (100%) of 28 lesions displayed progressive-enhancement, maintaining a state of persistent enhancement. In the diffuse-type cases, an enlarged liver was observed with a large nodule appearing with persistent enhancement on CEMP CT and MRI.
CONCLUSION: The most important imaging features of HEHE are the target sign and/or progressive enhancement with persistent enhancement on CEMP CT and MRI. MRI is advantageous over CT in displaying these imaging features.
Collapse
|
|
26
|
Kim EY, Kim TS, Han J, Choi JY, Kwon OJ, Kim J. Thoracic epithelioid hemangioendothelioma: imaging and pathologic features. Acta Radiol 2011; 52:161-6. [PMID: 21498344 DOI: 10.1258/ar.2010.100292] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
The purpose of this article is to review the imaging features of thoracic epithelioid hemangioendotheliomas with pathologic correlation. The thoracic manifestation of epithelioid hemangioendotheliomas is one of three different CT patterns: multiple pulmonary nodules, multiple pulmonary reticulonodular opacities, or diffuse infiltrative pleural thickening. Without any evidence of histological malignancy, malignant features (multiplicity of pulmonary nodules, hepatic or bone involvement, lymphangitic tumor spread, and infiltrative pleural masses) are characteristic of these infrequent tumors.
Collapse
Affiliation(s)
- Eun Young Kim
- Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul
- Department of Radiology, Gachon University Gil Hospital, Incheon
| | - Tae Sung Kim
- Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul
| | | | | | - O Jung Kwon
- Division of Pulmonary and Critical Care Medicine, Department of Medicine
| | - Jhingook Kim
- Department of Thoracic Surgery, Sungkyunkwan University School of Medicine, Seoul, Korea
| |
Collapse
|
|
27
|
Mizukami T, Kamiyama T, Nakanishi K, Yokoo H, Tahara M, Fukumori D, Kamachi H, Matsushita M, Todo S. The Case of resected Hepatic Epithelioidhemangio Endothelioma associated with Focal Nodular Hyperplasia and Hepatic Cavernous Hemangioma. THE JAPANESE JOURNAL OF GASTROENTEROLOGICAL SURGERY 2011; 44:131-137. [DOI: 10.5833/jjgs.44.131] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
|
|
28
|
Pareja E, Cortés M, Rayon M, Moya A, Mir J. [Epithelioid hemangioendothelioma: an uncommon liver tumor]. GASTROENTEROLOGIA Y HEPATOLOGIA 2010; 33:445-8. [PMID: 20570012 DOI: 10.1016/j.gastrohep.2010.04.002] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/03/2010] [Revised: 04/13/2010] [Accepted: 04/20/2010] [Indexed: 11/16/2022]
Abstract
We report the case of a female patient who was referred to our unit because of a solid liver tumor, suggestive of metastasis. After biopsy, the patient was diagnosed with epithelioid hemangioendothelioma of the liver. Epithelioid hemangioendothelioma is a rare entity with an unpredictable, potentially fatal, clinical course and outcome. Due to its rarity, this entity should be considered when a solitary hepatic lesion is detected and should be included in the differential diagnosis with liver metastases. We highlight the infrequency of this tumor, its presentation as a solitary hepatic lesion and the indication of surgical treatment. We describe the clinical and pathological characteristics of epithelioid hemangioendothelioma of the liver and report a new case of this entity. The distinct therapeutic options are discussed.
Collapse
Affiliation(s)
- Eugenia Pareja
- Hospital La Fe, Cirugía y Trasplante Hepatico, Valencia, España.
| | | | | | | | | |
Collapse
|
|
29
|
Chang JY, Marks RS, Nagorney DM, Sanderson SO, Kane S. Ulcerative colitis, infliximab, and hepatic epithelioid hemangioendothelioma: who is to blame? Case Report. Therap Adv Gastroenterol 2010; 3:203-6. [PMID: 21180602 PMCID: PMC3002576 DOI: 10.1177/1756283x10363015] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023] Open
|
|
30
|
Komatsu Y, Koizumi T, Yasuo M, Urushihata K, Yamamoto H, Hanaoka M, Kubo K, Kawakami S, Honda T, Fujimoto K, Hachiya T. Malignant hepatic epithelioid hemangioendothelioma with rapid progression and fatal outcome. Intern Med 2010; 49:1149-53. [PMID: 20558933 DOI: 10.2169/internalmedicine.49.3148] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 46-year-old woman was admitted to our hospital because of pain in the right upper quadrant and dyspnea. Abdominal and chest computed tomography (CT) scans revealed areas of low attenuation in both hepatic lobes, left pleural effusion, and multiple nodules in both lungs. Laboratory data indicated disseminated intravascular coagulation. She developed rapidly progressive respiratory and hepatic failure despite intensive treatment including mechanical ventilation and died of respiratory failure 3 weeks after admission. Immunohistochemical analysis of liver necropsy and cytology of the left plural effusion stained positive for factor VIII-related antigen and CD31. Based on these observations, a diagnosis of hemangioendothelioma (EHE), a rare vascular tumor, was made. A rapid clinical course and fatal outcome, as in the present case, are rare clinical manifestations in EHE.
Collapse
Affiliation(s)
- Yoshimichi Komatsu
- The First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
| | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
31
|
|
|
32
|
Oshima N, Terajima H, Hosotani R. Surgical Therapy for a Solitary Form of Hepatic Epithelioid Hemangioendothelioma: A Long-Term Survival Case. Case Rep Gastroenterol 2009; 3:214-221. [PMID: 21103278 PMCID: PMC2988960 DOI: 10.1159/000227734] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/30/2023] Open
Abstract
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare neoplasm of vascular origin. The clinical presentation of HEHE is variable, and the therapeutic criteria are still unclear since its natural history is unpredictable. A 53-year-old woman was admitted to our hospital because she had a 2.5-cm diameter nodule in the segment V of the liver. She had undergone segmental hepatectomy for solitary HEHE in segment VII 10 years before. There had been no recurrence for the 10 years after the first operation. The tumor was diagnosed as a new lesion of HEHE by percutaneous needle liver biopsy, and thereafter repeated hepatectomy was performed. HEHE seems to be resistant to chemotherapy and radiotherapy. Either surgical resection or orthotopic liver transplantation is generally recommended as a curative treatment for this disease. However, HEHE tends to be detected in multiple lesions, and localized disease is rare. Therefore, the chance of resection is very low. Some reports do not recommend local resection because of early aggressive tumor spread even after curative resection. We herein demonstrate a rare case of HEHE in a patient who underwent repeated hepatectomy for a solitary lesion and who survived for 17 years. It is concluded that surgical resection is one of the most effective treatments for a solitary form of HEHE.
Collapse
Affiliation(s)
- Nobu Oshima
- Department of Surgery, Kobe City Medical Center General Hospital, Kobe, Japan
| | | | | |
Collapse
|
|
33
|
Lee WJ. [Epithelioid hemangioendothelioma]. THE KOREAN JOURNAL OF HEPATOLOGY 2009; 15:222-226. [PMID: 19581775 DOI: 10.3350/kjhep.2009.15.2.222] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/28/2023]
Affiliation(s)
- Won Jae Lee
- Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
| |
Collapse
|
|
34
|
Liver transplantation for hepatic epithelioid hemangioendothelioma: the Canadian multicentre experience. CANADIAN JOURNAL OF GASTROENTEROLOGY = JOURNAL CANADIEN DE GASTROENTEROLOGIE 2009; 22:821-4. [PMID: 18925305 DOI: 10.1155/2008/418485] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
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 x 14 cm x 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.
Collapse
|
|
35
|
Mosoia L, Mabrut JY, Adham M, Boillot O, Ducerf C, Partensky C, Baulieux J. Hepatic epithelioid hemangioendothelioma: long-term results of surgical management. J Surg Oncol 2008; 98:432-7. [PMID: 18792957 DOI: 10.1002/jso.21132] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
BACKGROUND AND OBJECTIVES Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular neoplasm of the liver. Its therapeutic management remains difficult to define in curative intent. The aim of this study was to report long-term results of surgically managed patients. METHODS From 1990 to 2006, nine patients (25-64 years) were retrospectively enrolled in this study. Intrahepatic disease extent was monolobar and bilobar in two and seven patients, respectively. As primary treatment, liver resection (LR) and liver transplantation (LT) were performed in three (two monolobar and one bilobar extent) and six patients, respectively. RESULTS Postoperative mortality was nil. During a median follow-up of 117 months, four patients developed intrahepatic and/or extrahepatic recurrence. One resected patient (with bilobar extent) presented with intrahepatic recurrence was secondary treated by LT. At the time of the follow-up, seven out of the nine patients treated (two after LR, and five after LT) were alive and disease-free. CONCLUSIONS Surgical treatment offers good long-term results in patients suffering from HEHE when LR is tailored to the intrahepatic disease extent: LT has to be considered in patients with bilobar intrahepatic disease whereas LR should be strictly limited to patients presenting with localized and monolobar intrahepatic disease.
Collapse
Affiliation(s)
- Liviu Mosoia
- Department of Digestive Surgery and Liver Transplantation, Croix-Rousse University Hospital, Lyon, France
| | | | | | | | | | | | | |
Collapse
|
|
36
|
Primary hepatic sarcomas: CT findings. Eur Radiol 2008; 18:2196-205. [PMID: 18463872 DOI: 10.1007/s00330-008-0997-7] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/11/2007] [Revised: 03/12/2008] [Accepted: 03/22/2008] [Indexed: 12/13/2022]
Abstract
Primary hepatic sarcomas are rare tumors that are difficult to diagnose clinically. Different primary hepatic sarcomas may have different clinical, morphologic, and radiological features. In this pictorial review, we summarized computed tomography (CT) findings of some relatively common types of hepatic sarcomas, including angiosarcoma, epithelioid hemangioendothelioma (EHE), liposarcoma, undifferentiated embryonal sarcoma (UES), leiomyosarcoma, malignant fibrous histiocytoma (MFH), and carcinosarcoma (including cystadenocarcinosarcoma). To our knowledge, hepatic cystadenocarcinosarcoma has not been described in the English literature. The CT findings in our case are similar to that of cystadenocarcinoma, a huge, multilocular cystic mass with a large mural nodule and solid portion. The advent of CT has allowed earlier detection of primary hepatic sarcomas as well as more accurate diagnosis and characterization. In addition, we briefly discuss the MRI findings and diagnostic value of primary hepatic sarcomas.
Collapse
|
|
37
|
The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: report of the European liver transplant registry. Ann Surg 2008; 246:949-57; discussion 957. [PMID: 18043096 DOI: 10.1097/sla.0b013e31815c2a70] [Citation(s) in RCA: 111] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
BACKGROUND Hepatic epitheloid hemangioendothelioma (HEHE) is a rare low-grade vascular tumor. Its treatment algorithm is still unclear mainly due to a lack of larger clinical experiences with detailed long-term follow-up. MATERIAL AND METHODS Fifty-nine patients, reported to the European Liver Transplant Registry, were analyzed to define the role of liver transplantation (LT) in the treatment of this disease. Eleven (19%) patients were asymptomatic. Eighteen (30.5%) patients had pre-LT surgical [hepatic (7 patients) and extrahepatic (3 patients)] and/or systemic or locoregional (10 patients) medical therapy. Ten (16.9%) patients had extrahepatic disease localization before or at the time of LT. Follow-up was complete for all patients with a median of 92.5 (range, 7-369) from moment of diagnosis and a median of 78.5 (range, 1-245) from the moment of LT. RESULTS HEHE was bilobar in 96% of patients; 86% of patients had more than 15 nodules in the liver specimen. Early (<3 months) and late (>3 months) post-LT mortality was 1.7% (1 patient) and 22% (14 patients). Fourteen (23.7%) patients developed disease recurrence after a median time of 49 months (range, 6-98). Nine (15.3%) patients died of recurrent disease and 5 are surviving with recurrent disease. One-, 5-, and 10- year patient survival rates from moment of transplantation for the whole series are 93%, 83%, 72%. Pre-LT tumor treatment (n = 18) (89%, 89%, and 68% 1-, 5-, and 10-year survival rates from moment of LT vs. 95%, 80%, and 73% in case of absence of pre-LT treatment), lymph node (LN) invasion (n = 18) (96%, 81%, and 71% 1-, 5-, and 10-year survival rates vs. 83%, 78%, and 67% in node negative patients) and extrahepatic disease localization (n = 10) (90%, 80%, and 80% 1-, 5-, and 10-year survival rates vs. 94%, 83%, and 70% in case of absence of extrahepatic disease) did not significantly influence patient survival whereas microvascular (n = 24) (96%, 75%, 52% 1-, 5-, and 10-year survival vs. 96%, 92%, 85% in case of absence of microvascular invasion) and combined micro- and macrovascular invasion (n = 28) (90%, 72%, and 54% 1-,5-, and 10-year survival vs. 96%, 92%, and 85% in case of absence of vascular invasion, P = 0.03) did. Disease-free survival rates at 1, 5, and 10 years post-LT are 90%, 82%, and 64%. Disease-free survival is not significantly influenced by pre-LT treatment, LN status, extrahepatic disease localization, and vascular invasion. CONCLUSIONS The results of the largest reported transplant series in the treatment of HEHE are excellent. Preexisting extrahepatic disease localization as well as LN involvement are not contraindications to LT. Microvascular or combined macro-microvascular invasion significantly influence survival after LT. LT therefore should be offered as a valid therapy earlier in the disease course of these, frequently young, patients. Recurrent (allograft) disease should be treated aggressively as good long-term survivals can be obtained. Long-term prospective follow-up multicenter studies as well as the evaluation of antiangiogenic drugs are necessary to further optimize the treatment of this rare vascular hepatic disorder.
Collapse
|
|
38
|
Kamimura K, Yamamoto T, Suda T, Takahashi T, Nomoto M, Sakurada J, Aoyagi Y. A case of hepatic epithelioid hemangioendothelioma with peritoneal dissemination and severe infiltration to the vessels. KANZO 2008; 49:14-21. [DOI: 10.2957/kanzo.49.14] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
|
|
39
|
Economopoulos N, Kelekis NL, Argentos S, Tsompanlioti C, Patapis P, Nikolaou I, Gouliamos A. Bright-dark ring sign in MR imaging of hepatic epithelioid hemangioendothelioma. J Magn Reson Imaging 2008; 27:908-12. [DOI: 10.1002/jmri.21052] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
|
|
40
|
Woodall CE, Scoggins CR, Lewis AM, Mcmasters KM, Martin RC. Hepatic Malignant Epithelioid Hemangioendothelioma: A Case Report and Review of the Literature. Am Surg 2008. [DOI: 10.1177/000313480807400115] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
Abstract
Malignant epithelioid hemangioendothelioma is a rare hepatic tumor of vascular origin. It is most commonly found in young to middle aged women, and the tumors vary in reported malignant potential. Compounds such as oral contraceptive pills, poly vinyl chloride, and Thorotrast have been identified as risk factors for subsequent disease development. Radiologic (“lollipop” sign, capsular flattening) and pathologic (Factor-VIII antigen staining positive) evaluation aids in the diagnosis. As with most mesenchymal tumors, surgical resection is the most effective means of controlling local disease and preventing distant metastasis, though adjuvant therapies have been offered for those that are unresectable or not transplant candidates. We present our case of a hepatic malignant epithelioid hemangioendothelioma and a review of the English-language literature.
Collapse
Affiliation(s)
- Charles E. Woodall
- Department of Surgery, Division of Surgical Oncology, James Graham Brown Cancer Center, University of Louisville School of Medicine, Louisville, Kentucky
| | - Charles R. Scoggins
- Department of Surgery, Division of Surgical Oncology, James Graham Brown Cancer Center, University of Louisville School of Medicine, Louisville, Kentucky
| | - Angela M. Lewis
- Department of Surgery, Division of Surgical Oncology, James Graham Brown Cancer Center, University of Louisville School of Medicine, Louisville, Kentucky
| | - Kelly M. Mcmasters
- Department of Surgery, Division of Surgical Oncology, James Graham Brown Cancer Center, University of Louisville School of Medicine, Louisville, Kentucky
| | - Robert C.G. Martin
- Department of Surgery, Division of Surgical Oncology, James Graham Brown Cancer Center, University of Louisville School of Medicine, Louisville, Kentucky
| |
Collapse
|
|
41
|
Rodriguez JA, Becker NS, O'Mahony CA, Goss JA, Aloia TA. Long-term outcomes following liver transplantation for hepatic hemangioendothelioma: the UNOS experience from 1987 to 2005. J Gastrointest Surg 2008; 12:110-6. [PMID: 17710508 DOI: 10.1007/s11605-007-0247-3] [Citation(s) in RCA: 76] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/21/2007] [Accepted: 07/16/2007] [Indexed: 02/07/2023]
Abstract
INTRODUCTION Hepatic hemangioendothelioma (HEH) is a vascular neoplasm with intermediate malignant potential. Outcomes after liver transplantation have only been reported as small, single-institution experiences. The purpose of this study was to evaluate patient and allograft survivals after liver transplantation in a large, multi-institutional cohort of patients with HEH. METHODS Using the United Network for Organ Sharing (UNOS) database, we identified 110 patients with a diagnosis of HEH who underwent 126 transplants between 1987 and 2005. Patient and allograft survivals were calculated using Kaplan-Meier survival curves. Log rank tests were used to determine the influence of study variables on outcomes. RESULTS Of the 110 transplanted patients, 75 patients (68%) were female, 80 patients (73%) were Caucasian, and the median age was 36 years old (23%<4 y.o., 71%>18 y.o.). The 30-day posttransplant mortality rate was 2.4%. At a median patient follow-up interval of 24 months, 1- and 5-year patient and allograft survivals were 80% and 64%, and 70% and 55%, respectively. Pretransplant medical status, but not age, was found to statistically correlate with patient survival. CONCLUSION These data indicate that survivals after transplantation for HEH are favorable. Given the propensity for recurrence after resection, these data support consideration of liver transplantation for all patients with significant intrahepatic tumor burden.
Collapse
Affiliation(s)
- Joel A Rodriguez
- Michael E. DeBakey Department of Surgery, Baylor College of Medicine, 1709 Dryden, Suite 15.37, Houston, Texas 77030, USA
| | | | | | | | | |
Collapse
|
|
42
|
Mehrabi A, Kashfi A, Fonouni H, Schemmer P, Schmied BM, Hallscheidt P, Schirmacher P, Weitz J, Friess H, Buchler MW, Schmidt J. Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer 2006; 107:2108-2121. [PMID: 17019735 DOI: 10.1002/cncr.22225] [Citation(s) in RCA: 301] [Impact Index Per Article: 15.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Malignant hepatic epithelioid hemangioendothelioma (HEH) is a rare malignant tumor of vascular origin with unknown etiology and a variable natural course. The authors present a comprehensive review of the literature on HEH with a focus on clinical outcome after different therapeutic strategies. All published series on patients with HEH (n = 434 patients) were analyzed from the first description in 1984 to the current literature. The reviewed parameters included demographic data, clinical manifestations, therapeutic modalities, and clinical outcome. The mean age of patients with HEH was 41.7 years, and the male-to-female ratio was 2:3. The most common clinical manifestations were right upper quadrant pain, hepatomegaly, and weight loss. Most patients presented with multifocal tumor that involved both lobes of the liver. Lung, peritoneum, lymph nodes, and bone were the most common sites of extrahepatic involvement at the time of diagnosis. The most common management has been liver transplantation (LTx) (44.8% of patients), followed by no treatment (24.8% of patients), chemotherapy or radiotherapy (21% of patients), and liver resection (LRx) (9.4% of patients). The 1-year and 5-year patient survival rates were 96% and 54.5%, respectively, after LTx; 39.3% and 4.5%, respectively, after no treatment, 73.3% and 30%, respectively, after chemotherapy or radiotherapy; and 100% and 75%, respectively, after LRx. LRx has been the treatment of choice in patients with resectable HEH. However, LTx has been proposed as the treatment of choice because of the hepatic multicentricity of HEH. In addition, LTx is an acceptable option for patients who have HEH with extrahepatic manifestation. Highly selected patients may be able to undergo living-donor LTx, preserving the donor pool. The role of different adjuvant therapies for patients with HEH remains to be determined.
Collapse
Affiliation(s)
- Arianeb Mehrabi
- Department of General, Visceral, and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany.
| | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
43
|
Affiliation(s)
- Frank Earnest
- Department of Radiology, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 55905, USA.
| | | |
Collapse
|
|
44
|
Mehrabi A, Kashfi A, Schemmer P, Sauer P, Encke J, Fonouni H, Friess H, Weitz J, Schmidt J, Büchler MW, Kraus TW. Surgical treatment of primary hepatic epithelioid hemangioendothelioma. Transplantation 2005; 80:S109-S112. [PMID: 16286886 DOI: 10.1097/01.tp.0000186904.15029.4a] [Citation(s) in RCA: 38] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Epithelioid hemangioendothelioma is a very rare tumor of vascular origin. It can develop in different tissues such as soft tissue, lung, or liver. Hepatic epithelioid hemangioendothelioma (HEH) mostly affects females. The malignant potential of HEH often remains unclear in the individual patient. It can range from benign hemangioma to malignant hemangioendotheliosarcoma. Here we present our experience with five patients with primary HEH, who were treated with curative intention in our department. All patients in our series with confirmed histological HEH did not show extrahepatic extension and consequently underwent surgical treatment. In three patients, liver transplantation (LTx) was performed (two cadaveric and one living related). In one patient, a right-sided hemihepatectomy with partial resection of the diaphragm was performed. One patient died while she was on the waiting list for LTx due to rapid tumor progression. Postoperative follow-up ranged from 1 to 13 years. No adjuvant chemotherapy was applied. Until now, no recurrence of local tumor or distant metastases could be observed during follow-up in our series. Early detection and surgical intervention in case of HEH can potentially offer curative treatment. The treatment of first choice appears to be radical liver resection. In our view, LTx represents a potentially important option for patients with a nonresectable tumor. Despite the long waiting time, its often unclear dignity, and a proven progressive growth pattern, living related LTx also plays a potentially important role. The 5-year overall survival rate of patients with HEH in the literature varies from 43% to 55%. Long-term survival of patients with HEH is significantly higher compared to other hepatic malignancies. The role of adjuvant therapy currently remains unclear.
Collapse
Affiliation(s)
- Arianeb Mehrabi
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
45
|
Ponferrada A, Salcedo M, Matilla A, Núñez O, Bañares R, Alvarez E, Clemente G. Heterogeneidad clínica y evolutiva del hemangioendotelioma epitelioide hepático. GASTROENTEROLOGIA Y HEPATOLOGIA 2005; 28:321-5. [PMID: 15989812 DOI: 10.1157/13076348] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
Epithelioid hemangioendothelioma is an infrequent mesenchymal tumor of unknown etiology and variable clinical symptoms. It usually affects black, middle-aged women. The most frequent symptoms are right hypochondrium pain, abdominal discomfort and constitutional syndrome with progressive liver damage. Diagnosis is difficult and detailed pathological analysis is required. Controlled clinical studies are lacking and there are no factors that predict the clinical course or serve as a guide to the most effective treatment. We report 3 cases with distinct clinical course ranging from clinical stability to dissemination and death. Treatment differed in the three patients.
Collapse
Affiliation(s)
- A Ponferrada
- Sección de Hepatología, Servicio de Aparato Digestivo, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
| | | | | | | | | | | | | |
Collapse
|
|
46
|
Mascarenhas RCV, Sanghvi AN, Friedlander L, Geyer SJ, Beasley HS, Van Thiel DH. Thalidomide inhibits the growth and progression of hepatic epithelioid hemangioendothelioma. Oncology 2005; 67:471-5. [PMID: 15714004 DOI: 10.1159/000082932] [Citation(s) in RCA: 47] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2004] [Accepted: 04/28/2004] [Indexed: 12/12/2022]
Abstract
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.
Collapse
Affiliation(s)
- Ranjan C V Mascarenhas
- Department of Medicine, Loyola University Chicago, Stritch School of Medicine, Maywood, Ill., USA
| | | | | | | | | | | |
Collapse
|
|
47
|
Alobaidi M, Shirkhoda A. Malignant cystic and necrotic liver lesions: A pattern approach to discrimination. Curr Probl Diagn Radiol 2004; 33:254-68. [PMID: 15549051 DOI: 10.1067/j.cpradiol.2004.08.002] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
Malignant primary and metastatic lesions of the liver have a myriad of imaging appearances. Discriminating between the various lesions requires an understanding of the underlying pathophysiology and imaging characteristics that lead to their malignant appearances. A pattern approach of recognition by imaging with understanding of why particular lesions behave the way they do on sonography, CT, MRI, and nuclear scintigraphy can be developed. A broad selection of malignant cystic liver lesions will be displayed in a multimodality pictorial style including hepatocellular carcinoma, cholangiocarcinoma, cystic and necrotic hepatic metastases, biliary cystadenoma/cystadenocarcinoma, epitheloid hemangioendothelioma, hepatoblastoma, and cystic sarcomas. Characteristic imaging features, clinical symptoms, and prognosis will be discussed. Emphasis will be placed on consistent features of these malignant lesions that help to discriminate them from other hepatic disorders.
Collapse
|
|
48
|
Mermuys K, Vanhoenacker PK, Roskams T, D'Haenens P, Van Hoe L. Epithelioid hemangioendothelioma of the liver: radiologic-pathologic correlation. ACTA ACUST UNITED AC 2004; 29:221-3. [PMID: 15290949 DOI: 10.1007/s00261-003-0094-y] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
Epithelioid hemangioendothelioma of the liver is a rare vascular tumor with intermediate malignant potential. On imaging studies, the lesion has a solid appearance and may mimic metastatic disease. We present a case in which the morphologic features (multifocal aspect, peripheral location, and capsular retraction) and the clinical history aided in including this entity in the differential diagnosis.
Collapse
Affiliation(s)
- K Mermuys
- Department of Radiology, Onze Lieve Vrouwe Hospital Aalst, Moorselbaan 164, 9300 Aalst, Belgium.
| | | | | | | | | |
Collapse
|
|
49
|
Lerut JP, Orlando G, Sempoux C, Ciccarelli O, Van Beers BE, Danse E, Horsmans Y, Rahier J, Roggen F. Hepatic haemangioendothelioma in adults: excellent outcome following liver transplantation. Transpl Int 2004. [PMID: 15114438 DOI: 10.1111/j.1432-2277.2004.tb00429.x] [Citation(s) in RCA: 34] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
Hepatic epithelioid haemangioendotheliomas (HEHEs) are rare, low-grade vascular tumours. Five adults with HEHEs and one adult with a vascular tumour showing combined features of haemangioma and haemangioendothelioma underwent liver transplantation. Two HEHE patients had extrahepatic metastases at the time of transplantation. Median survival time following diagnosis was 10.7 years (range 40 months to 195 months). One patient needed resection of a HEHE in the breast 13 years post-transplantation. All six patients are surviving free from disease 22 to 166 months after transplantation (median 77 months). One HEHE-patient who had been treated for 8 years for vertebral and cerebral localisations is free of disease without immunosuppression 56 months after transplantation. We can conclude that liver transplantation is a valuable treatment for hepatic haemangioendothelioma, even in cases of extrahepatic localisation of the disease.
Collapse
Affiliation(s)
- Jan P Lerut
- Liver Transplant Programme, Department of Digestive Surgery, Cliniques Universitaires St-Luc/1400, Université Catholique de Louvain (UCL), Av. Hippocrate 10, 1200 Brussels, Belgium.
| | | | | | | | | | | | | | | | | |
Collapse
|
|
50
|
Joshua AM, Stockler MR, Boyer MJ. Prolonged survival after the diagnosis of metastatic hepatic epitheliod haemangioendothelioma. Intern Med J 2004; 34:70-1. [PMID: 14748919 DOI: 10.1111/j.1444-0903.2004.00514.x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
|