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For: Alomari AI. The lollipop sign: a new cross-sectional sign of hepatic epithelioid hemangioendothelioma. Eur J Radiol 2006;59:460-4. [PMID: 16644166 DOI: 10.1016/j.ejrad.2006.03.022] [Citation(s) in RCA: 57] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [What about the content of this article? (0)] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2005] [Revised: 03/19/2006] [Accepted: 03/20/2006] [Indexed: 02/07/2023]

For:	Alomari AI. The lollipop sign: a new cross-sectional sign of hepatic epithelioid hemangioendothelioma. Eur J Radiol 2006;59:460-4. [PMID: 16644166 DOI: 10.1016/j.ejrad.2006.03.022] [Citation(s) in RCA: 57] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [What about the content of this article? (0)] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2005] [Revised: 03/19/2006] [Accepted: 03/20/2006] [Indexed: 02/07/2023]

Number

Cited by Other Article(s)

Malik M, Idrees RB, Mirza Z, Anwar S, Ahmad B, Chaudhary MH. Rare Encounter With Hepatic Epithelioid Hemangioendothelioma: A Case Report. Cureus 2025;17:e80567. [PMID: 40225499 PMCID: PMC11994122 DOI: 10.7759/cureus.80567] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/14/2025] [Indexed: 04/15/2025] Open

Matteini F, Cannella R, Dioguardi Burgio M, Torrisi C, Sartoris R, Brancatelli G, Vilgrain V, Ronot M, Vernuccio F. Discontinuous peripheral enhancement of focal liver lesions on CT and MRI: outside the box of typical cavernous hemangioma. Abdom Radiol (NY) 2025;50:693-709. [PMID: 39192088 PMCID: PMC11794645 DOI: 10.1007/s00261-024-04522-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/29/2024] [Accepted: 08/06/2024] [Indexed: 08/29/2024]

Meng K, Yang X, Guo S, Tao J. Hepatic epithelioid hemangioendothelioma with TFE3 rearrangement: a case report and literature review. Front Oncol 2025;15:1442233. [PMID: 39917171 PMCID: PMC11799294 DOI: 10.3389/fonc.2025.1442233] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2024] [Accepted: 01/03/2025] [Indexed: 02/09/2025] Open

Khasawneh H, O'Brien C, Czeyda-Pommersheim F, Qayyum A, Miller FH, Arif Tiwari H, Paspulati RM, Kierans AS. Beyond cholangiocarcinoma: imaging features of mimicking pathologies in the biliary tract. Abdom Radiol (NY) 2024:10.1007/s00261-024-04749-z. [PMID: 39710762 DOI: 10.1007/s00261-024-04749-z] [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: 10/16/2024] [Revised: 12/02/2024] [Accepted: 12/03/2024] [Indexed: 12/24/2024]

Vadlamani SP, Mahato A, Madakshira MG. Multifocal epithelioid hemangioendothelioma of liver. Med J Armed Forces India 2024;80:738-740. [PMID: 39990531 PMCID: PMC11842925 DOI: 10.1016/j.mjafi.2022.03.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2022] [Accepted: 03/31/2022] [Indexed: 11/25/2022] Open

Wang B, Chen X, Li R, Ai B, Ye F, Zhao J, Zhang Y, Huang Z, Li Z, Bi X, Zhao H, Cao D, Cai J, Zhou J, Yan T. Comprehensive evaluation of clinical outcomes in hepatic epithelioid hemangioendothelioma subsets: insights from SEER Database and departmental cohort analysis. Front Immunol 2024;15:1491922. [PMID: 39502705 PMCID: PMC11534872 DOI: 10.3389/fimmu.2024.1491922] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2024] [Accepted: 10/07/2024] [Indexed: 11/08/2024] Open

Abstract

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.

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Affiliation(s)

Bingchen Wang Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Xiao Chen Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Rongxuan Li Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Bolun Ai Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Feng Ye Department of Radiology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Jianjun Zhao Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Yefan Zhang Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Zhen Huang Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Zhiyu Li Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Xinyu Bi Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Hong Zhao Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Dayong Cao Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Jianqiang Cai Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Jianguo Zhou Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Tao Yan Department of Anesthesiology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

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Dong Y, Cekuolis A, Schreiber-Dietrich D, Augustiniene R, Schwarz S, Möller K, Nourkami-Tutdibi N, Chen S, Cao JY, Huang YL, Wang Y, Taut H, Grevelding L, Dietrich CF. Review on Pediatric Malignant Focal Liver Lesions with Imaging Evaluation: Part II. Diagnostics (Basel) 2023;13:3659. [PMID: 38132242 PMCID: PMC10743166 DOI: 10.3390/diagnostics13243659] [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: 10/17/2023] [Revised: 12/04/2023] [Accepted: 12/06/2023] [Indexed: 12/23/2023] Open

Affiliation(s)

Yi Dong Department of Ultrasound, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200092, China; (Y.D.); (S.C.); (J.-Y.C.); (Y.-L.H.); (Y.W.)
Andrius Cekuolis Ultrasound Section, Department of Pediatric Radiology, Radiology and Nuclear Medicine Centre, Vilnius University Hospital Santaros Klinikos, 08661 Vilnius, Lithuania; (A.C.); (R.A.)
Dagmar Schreiber-Dietrich Localinomed and Pediatric Department, Kliniken Salem, 3013 Bern, Switzerland;
Rasa Augustiniene Ultrasound Section, Department of Pediatric Radiology, Radiology and Nuclear Medicine Centre, Vilnius University Hospital Santaros Klinikos, 08661 Vilnius, Lithuania; (A.C.); (R.A.)
Simone Schwarz Department of Neonatology and Pediatric Intensive Care Medicine, Sana Kliniken Duisburg GmbH, 47055 Duisburg, Germany;
Kathleen Möller Medical Department I/Gastroenterology, SANA Hospital Lichtenberg, 10365 Berlin, Germany;
Nasenien Nourkami-Tutdibi Saarland University Medical Center, Hospital of General Pediatrics and Neonatology, 66421 Homburg, Germany;
Sheng Chen Department of Ultrasound, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200092, China; (Y.D.); (S.C.); (J.-Y.C.); (Y.-L.H.); (Y.W.)
Jia-Ying Cao Department of Ultrasound, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200092, China; (Y.D.); (S.C.); (J.-Y.C.); (Y.-L.H.); (Y.W.)
Yun-Lin Huang Department of Ultrasound, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200092, China; (Y.D.); (S.C.); (J.-Y.C.); (Y.-L.H.); (Y.W.)
Ying Wang Department of Ultrasound, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200092, China; (Y.D.); (S.C.); (J.-Y.C.); (Y.-L.H.); (Y.W.)
Heike Taut Children’s Hospital, Universitätsklinikum Dresden, Technische Universität Dresden, 01069 Dresden, Germany;
Lara Grevelding Department of Pediatrics, Division of Pneumology, Allergology, Infectious Diseases and Gastroenterology, University Hospital Frankfurt, Goethe University, 60323 Frankfurt, Germany
Christoph F. Dietrich Department of Ultrasound, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200092, China; (Y.D.); (S.C.); (J.-Y.C.); (Y.-L.H.); (Y.W.) Department Allgemeine Innere Medizin (DAIM), Kliniken Hirslanden Beau Site, Salem und Permanence, 3013 Bern, Switzerland

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Abdelmogod A, Papadopoulos L, Riordan S, Wong M, Weltman M, Lim R, McEvoy C, Fellowes A, Fox S, Bedő J, Penington J, Pham K, Hofmann O, Vissers JHA, Grimmond S, Ratnayake G, Christie M, Mitchell C, Murray WK, McClymont K, Luk P, Papenfuss AT, Kee D, Scott CL, Goldstein D, Barker HE. A Matched Molecular and Clinical Analysis of the Epithelioid Haemangioendothelioma Cohort in the Stafford Fox Rare Cancer Program and Contextual Literature Review. Cancers (Basel) 2023;15:4378. [PMID: 37686662 PMCID: PMC10487006 DOI: 10.3390/cancers15174378] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2023] [Revised: 08/21/2023] [Accepted: 08/29/2023] [Indexed: 09/10/2023] Open

Abstract

BACKGROUND

Epithelioid haemangioendothelioma (EHE) is an ultra-rare malignant vascular tumour with a prevalence of 1 per 1,000,000. It is typically molecularly characterised by a WWTR1::CAMTA1 gene fusion in approximately 90% of cases, or a YAP1::TFE3 gene fusion in approximately 10% of cases. EHE cases are typically refractory to therapies, and no anticancer agents are reimbursed for EHE in Australia.

METHODS

We report a cohort of nine EHE cases with comprehensive histologic and molecular profiling from the Walter and Eliza Hall Institute of Medical Research Stafford Fox Rare Cancer Program (WEHI-SFRCP) collated via nation-wide referral to the Australian Rare Cancer (ARC) Portal. The diagnoses of EHE were confirmed by histopathological and immunohistochemical (IHC) examination. Molecular profiling was performed using the TruSight Oncology 500 assay, the TruSight RNA fusion panel, whole genome sequencing (WGS), or whole exome sequencing (WES).

RESULTS

Molecular analysis of RNA, DNA or both was possible in seven of nine cases. The WWTR1::CAMTA1 fusion was identified in five cases. The YAP1::TFE3 fusion was identified in one case, demonstrating unique morphology compared to cases with the more common WWTR1::CAMTA1 fusion. All tumours expressed typical endothelial markers CD31, ERG, and CD34 and were negative for pan-cytokeratin. Cases with a WWTR1::CAMTA1 fusion displayed high expression of CAMTA1 and the single case with a YAP1::TFE3 fusion displayed high expression of TFE3. Survival was highly variable and unrelated to molecular profile.

CONCLUSIONS

This cohort of EHE cases provides molecular and histopathological characterisation and matching clinical information that emphasises the molecular patterns and variable clinical outcomes and adds to our knowledge of this ultra-rare cancer. Such information from multiple studies will advance our understanding, potentially improving treatment options.

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Affiliation(s)

Arwa Abdelmogod Limestone Coast Local Health Network, Flinders University, Bedford Park, SA 5042, Australia;
Lia Papadopoulos The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC 3052, Australia; (L.P.); (R.L.); (J.B.); (J.P.); (A.T.P.); (D.K.); (C.L.S.) The Australian Rare Cancer Portal, BioGrid, Parkville, VIC 3051, Australia; Eastern Health Clinical School, Monash University, Box Hill, VIC 3128, Australia
Stephen Riordan Prince of Wales Clinical School, University of NSW, Randwick, NSW 2031, Australia; Gastrointestinal and Liver Unit, Prince of Wales Hospital, Randwick, NSW 2031, Australia
Melvin Wong Radiology Department, Prince of Wales Hospital, Randwick, NSW 2031, Australia;
Martin Weltman Department of Gastroenterology, Nepean Hospital, Kingswood, NSW 2747, Australia;
Ratana Lim The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC 3052, Australia; (L.P.); (R.L.); (J.B.); (J.P.); (A.T.P.); (D.K.); (C.L.S.)
Christopher McEvoy Peter MacCallum Cancer Centre, Melbourne, VIC 3000, Australia; (C.M.); (A.F.)
Andrew Fellowes Peter MacCallum Cancer Centre, Melbourne, VIC 3000, Australia; (C.M.); (A.F.)
Stephen Fox Peter MacCallum Cancer Centre, Melbourne, VIC 3000, Australia; (C.M.); (A.F.)
Justin Bedő The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC 3052, Australia; (L.P.); (R.L.); (J.B.); (J.P.); (A.T.P.); (D.K.); (C.L.S.)
Jocelyn Penington The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC 3052, Australia; (L.P.); (R.L.); (J.B.); (J.P.); (A.T.P.); (D.K.); (C.L.S.)
Kym Pham Centre for Cancer Research and Department of Clinical Pathology, University of Melbourne, Melbourne, VIC 3010, Australia; (K.P.); (O.H.); (J.H.A.V.); (S.G.)
Oliver Hofmann Centre for Cancer Research and Department of Clinical Pathology, University of Melbourne, Melbourne, VIC 3010, Australia; (K.P.); (O.H.); (J.H.A.V.); (S.G.)
Joseph H. A. Vissers Centre for Cancer Research and Department of Clinical Pathology, University of Melbourne, Melbourne, VIC 3010, Australia; (K.P.); (O.H.); (J.H.A.V.); (S.G.)
Sean Grimmond Centre for Cancer Research and Department of Clinical Pathology, University of Melbourne, Melbourne, VIC 3010, Australia; (K.P.); (O.H.); (J.H.A.V.); (S.G.)
Gayanie Ratnayake The Royal Womens’ Hospital, Parkville, VIC 3052, Australia;
Michael Christie The Royal Melbourne Hospital, Parkville, VIC 3052, Australia;
Catherine Mitchell Department of Pathology, Peter MacCallum Cancer Centre, Melbourne, VIC 3000, Australia; (C.M.); (W.K.M.)
William K. Murray Department of Pathology, Peter MacCallum Cancer Centre, Melbourne, VIC 3000, Australia; (C.M.); (W.K.M.)
Kelly McClymont Sullivan Nicolaides Pathology, Brisbane, QLD 4000, Australia;
Peter Luk Royal Prince Alfred Hospital, Camperdown, NSW 2050, Australia;
Anthony T. Papenfuss The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC 3052, Australia; (L.P.); (R.L.); (J.B.); (J.P.); (A.T.P.); (D.K.); (C.L.S.) Department of Gastroenterology, Nepean Hospital, Kingswood, NSW 2747, Australia; Sir Peter MacCallum Cancer Centre, Department of Oncology, University of Melbourne, Parkville, VIC 3000, Australia
Damien Kee The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC 3052, Australia; (L.P.); (R.L.); (J.B.); (J.P.); (A.T.P.); (D.K.); (C.L.S.) The Australian Rare Cancer Portal, BioGrid, Parkville, VIC 3051, Australia; Sir Peter MacCallum Cancer Centre, Department of Oncology, University of Melbourne, Parkville, VIC 3000, Australia Austin Health, Heidelberg, VIC 3084, Australia
Clare L. Scott The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC 3052, Australia; (L.P.); (R.L.); (J.B.); (J.P.); (A.T.P.); (D.K.); (C.L.S.) The Australian Rare Cancer Portal, BioGrid, Parkville, VIC 3051, Australia; The Royal Womens’ Hospital, Parkville, VIC 3052, Australia; Sir Peter MacCallum Cancer Centre, Department of Oncology, University of Melbourne, Parkville, VIC 3000, Australia Department of Medical Biology, University of Melbourne, Melbourne, VIC 3010, Australia Department of Obstetrics and Gynaecology, University of Melbourne, Parkville, VIC 3010, Australia
David Goldstein The Australian Rare Cancer Portal, BioGrid, Parkville, VIC 3051, Australia; Eastern Health Clinical School, Monash University, Box Hill, VIC 3128, Australia Nelune Center, Prince of Wales Hospital, Randwick, NSW 2031, Australia
Holly E. Barker The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC 3052, Australia; (L.P.); (R.L.); (J.B.); (J.P.); (A.T.P.); (D.K.); (C.L.S.) Department of Medical Biology, University of Melbourne, Melbourne, VIC 3010, Australia

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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.

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Carney BW, Larson MC, Corwin MT, Lamba R. Imaging of Hepatobiliary Cancer. Curr Probl Cancer 2023:100964. [PMID: 37321910 DOI: 10.1016/j.currproblcancer.2023.100964] [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: 12/29/2022] [Revised: 05/04/2023] [Accepted: 05/20/2023] [Indexed: 06/17/2023]

Tan H, Zhou R, Yu H, Teng F, Si S, Liu L, Yang S, Han D, Liu X. CT appearances and classification of hepatic epithelioid hemangioendothelioma. Insights Imaging 2023;14:56. [PMID: 37005950 PMCID: PMC10067783 DOI: 10.1186/s13244-023-01410-z] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2022] [Accepted: 03/20/2023] [Indexed: 04/04/2023] Open

Luo L, Cai Z, Zeng S, Wang L, Kang Z, Yang N, Zhang Y. CT and MRI features of hepatic epithelioid haemangioendothelioma: a multi-institutional retrospective analysis of 15 cases and a literature review. Insights Imaging 2023;14:2. [PMID: 36600110 PMCID: PMC9813315 DOI: 10.1186/s13244-022-01344-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2022] [Accepted: 12/08/2022] [Indexed: 01/06/2023] Open

Ajay PS, Tsagkalidis V, Casabianca A, Burchard PR, Melucci AD, Chacon A, Goyal S, Switchenko JM, Kooby DA, Carpizo DR, Shah MM. A review of hepatic epithelioid hemangioendothelioma-Analyzing patient characteristics and treatment strategies. J Surg Oncol 2022;126:1423-1429. [PMID: 35975699 PMCID: PMC9836828 DOI: 10.1002/jso.27066] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2022] [Accepted: 07/30/2022] [Indexed: 01/14/2023]

Chu K, Li Z, Tang W, Jiang X. Updated information regarding management of hepatic epithelioid hemangioendothelioma. Intractable Rare Dis Res 2022;11:211-214. [PMID: 36457586 PMCID: PMC9709622 DOI: 10.5582/irdr.2022.01113] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/12/2022] [Revised: 11/04/2022] [Accepted: 11/06/2022] [Indexed: 11/10/2022] Open

Cross-Sectional Imaging Findings of Atypical Liver Malignancies and Diagnostic Pitfalls. Radiol Clin North Am 2022;60:775-794. [DOI: 10.1016/j.rcl.2022.05.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]

Mo WF, Tong YL. Hepatic epithelioid hemangioendothelioma after thirteen years’ follow-up: A case report and review of literature. World J Clin Cases 2022;10:6119-6127. [PMID: 35949854 PMCID: PMC9254202 DOI: 10.12998/wjcc.v10.i18.6119] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2021] [Revised: 01/24/2022] [Accepted: 04/23/2022] [Indexed: 02/06/2023] Open

Bisbee CA, Burstiner L, Natcheva H. Hepatic Epithelioid Hemangioendothelioma With Unique Metastasis to the Superior Vena Cava Found on CT Imaging: A Case Study. Cureus 2022;14:e26155. [PMID: 35891869 PMCID: PMC9302553 DOI: 10.7759/cureus.26155] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/21/2022] [Indexed: 11/09/2022] Open

Liu X, Yu H, Zhang Z, Si S, Huang J, Tan H, Teng F, Yang Z. MRI appearances of hepatic epithelioid hemangioendothelioma: a retrospective study of 57 patients. Insights Imaging 2022;13:65. [PMID: 35380293 PMCID: PMC8982790 DOI: 10.1186/s13244-022-01213-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2021] [Accepted: 03/21/2022] [Indexed: 11/17/2022] Open

Kolleri JJ, Khaliq A, Ladumor SB, Habtezghi AB, Koshy SM, Petkar M. Primary Hepatic Epithelioid Hemangioendothelioma Masquerading as a Hepatic Abscess With Infective Picture: A Case Report. Cureus 2022;14:e22859. [PMID: 35392441 PMCID: PMC8977811 DOI: 10.7759/cureus.22859] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/28/2022] [Indexed: 11/09/2022] Open

An incidental hepatic lesion in a cirrhotic liver. Clin Res Hepatol Gastroenterol 2022;46:101825. [PMID: 34757154 DOI: 10.1016/j.clinre.2021.101825] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/23/2021] [Revised: 09/16/2021] [Accepted: 10/21/2021] [Indexed: 02/04/2023]

Kovač JD, Janković A, Đikić-Rom A, Grubor N, Antić A, Dugalić V. Imaging Spectrum of Intrahepatic Mass-Forming Cholangiocarcinoma and Its Mimickers: How to Differentiate Them Using MRI. Curr Oncol 2022;29:698-723. [PMID: 35200560 PMCID: PMC8870737 DOI: 10.3390/curroncol29020061] [Citation(s) in RCA: 22] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/29/2021] [Revised: 01/23/2022] [Accepted: 01/29/2022] [Indexed: 12/14/2022] Open

Wang X, Liang P, Lv P, Li R, Hou P, Gao J. Clinical characteristics and CT features of hepatic epithelioid haemangioendothelioma and comparison with those of liver metastases. Insights Imaging 2022;13:9. [PMID: 35050424 PMCID: PMC8776937 DOI: 10.1186/s13244-021-01143-x] [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: 08/11/2021] [Accepted: 12/14/2021] [Indexed: 11/10/2022] Open

Che F, Yang CW, Hu X, Li Q, Wei Y, Liu XJ, Song B. Massive Calcified Epithelioid Hemangioendothelioma With Multifocal Involvement: An Imaging Diagnosis Dilemma and a Rare Case Report. Front Oncol 2021;11:782970. [PMID: 34976822 PMCID: PMC8718394 DOI: 10.3389/fonc.2021.782970] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2021] [Accepted: 11/30/2021] [Indexed: 02/05/2023] Open

Serji B, Ramdani A, Mirali H, Bouhout T, Bennani A, El Harroudi T. Hepatic epithelioid hemangioendothelioma simulating liver metastasis: A case report. Ann Med Surg (Lond) 2021;70:102885. [PMID: 34691426 PMCID: PMC8519773 DOI: 10.1016/j.amsu.2021.102885] [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: 08/26/2021] [Revised: 09/20/2021] [Accepted: 09/21/2021] [Indexed: 12/02/2022] Open

Abstract

Introduction

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular origin. Liver involvement is often multinodular simulating metastases. Herein, we report a rare case of HEHE mimicking liver metastases in a female patient.

Case presentation

A 43-years-old female patient, presented with complaints of pain in the right upper quadrant. Laboratory tests were all within the normal range. Abdominal ultrasound revealed multiple heterogeneous hypoechoic liver lesions. A thoracic and abdominopelvic computed tomography showed bilateral pulmonary micronodules with multiple hypodense hepatic nodules involving both lobes. Percutaneous ultrasound-guided biopsy with pathological study and immunohistochemistry staining revealed the diagnosis of hepatic epithelioid hemangioendothelioma.

Discussion

HEHE usually involves both liver lobes with three radiological presentations: single nodular, multiple nodular, or diffuse types, the diffuse type reflects an advanced stage. 3/4 of the cases are initially misdiagnosed as liver metastases or primary liver tumors. The pathological study with the immunochemistry stainings confirms the diagnosis. There is no standard treatment for HEHE due to its rarity and lack of prospective randomized studies.

Conclusion

HEHE is a rare tumor of vascular origin of unknown etiology with malignant potential and unpredictable course. The therapeutic management of this rare condition is not codified and is discussed on a case-by-case basis. Surgical treatment remains the best option with an excellent outcome.

•

Hepatic epithelioid hemangioendothelioma is a rare tumor of vascular origin.

•

The Clinical presentation and the radiological findings are not specific.

•

Final Diagnosis is made upon pathological study and immunohistochemistry staining.

•

Surgical treatment remain the treatment of choice.

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Stacchiotti S, Miah AB, Frezza AM, Messiou C, Morosi C, Caraceni A, Antonescu CR, Bajpai J, Baldini E, Bauer S, Biagini R, Bielack S, Blay JY, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Callegaro D, De Alava E, Deoras-Sutliff M, Dufresne A, Eriksson M, Errani C, Fedenko A, Ferraresi V, Ferrari A, Fletcher CDM, Garcia Del Muro X, Gelderblom H, Gladdy RA, Gouin F, Grignani G, Gutkovich J, Haas R, Hindi N, Hohenberger P, Huang P, Joensuu H, Jones RL, Jungels C, Kasper B, Kawai A, Le Cesne A, Le Grange F, Leithner A, Leonard H, Lopez Pousa A, Martin Broto J, Merimsky O, Merriam P, Miceli R, Mir O, Molinari M, Montemurro M, Oldani G, Palmerini E, Pantaleo MA, Patel S, Piperno-Neumann S, Raut CP, Ravi V, Razak ARA, Reichardt P, Rubin BP, Rutkowski P, Safwat AA, Sangalli C, Sapisochin G, Sbaraglia M, Scheipl S, Schöffski P, Strauss D, Strauss SJ, Sundby Hall K, Tap WD, Trama A, Tweddle A, van der Graaf WTA, Van De Sande MAJ, Van Houdt W, van Oortmerssen G, Wagner AJ, Wartenberg M, Wood J, Zaffaroni N, Zimmermann C, Casali PG, Dei Tos AP, Gronchi A. Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts. ESMO Open 2021;6:100170. [PMID: 34090171 PMCID: PMC8182432 DOI: 10.1016/j.esmoop.2021.100170] [Citation(s) in RCA: 106] [Impact Index Per Article: 26.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2021] [Revised: 05/07/2021] [Accepted: 05/11/2021] [Indexed: 12/22/2022] Open

Affiliation(s)

S Stacchiotti Adult Mesenchymal Tumor and Rare Cancer Unit, Cancer Medicine Department, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy.
A B Miah The Royal Marsden Hospital and The Institute of Cancer Research, London, UK
A M Frezza Adult Mesenchymal Tumor and Rare Cancer Unit, Cancer Medicine Department, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
C Messiou Department of Radiology, The Royal Marsden Hospital and The Institute of Cancer Research, London, UK
C Morosi Radiology Department, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
A Caraceni Palliative Care Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
C R Antonescu Department of Pathology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, USA
J Bajpai Medical Oncology Department, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India
E Baldini Department of Radiation Oncology, Dana-Farber Cancer Center/Brigham and Women's Hospital, Boston, USA
S Bauer Department of Medical Oncology, West German Cancer Center, Sarcoma Center, University of Duisburg-Essen, University Hospital, Essen, Germany
R Biagini Orthopaedic Department, Regina Elena National Cancer Institute, Rome, Italy
S Bielack Klinikum Stuttgart - Olgahospital, Zentrum für Kinder-, Jugend- und Frauenmedizin, Stuttgart Cancer Center, Pädiatrische Onkologie, Hämatologie, Immunologie, Stuttgart, Germany
J Y Blay Department of Medical Oncology, Centre Leon Berard, Université Claude Bernard Lyon, Unicancer, Lyon, France
S Bonvalot Department of Surgical Oncology, Institut Curie, Université Paris Sciences et Lettres, Paris, France
I Boukovinas Nordix, Athens, Greece
J V M G Bovee Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands
K Boye Department of Oncology, Oslo University Hospital, The Norwegian Radium Hospital, Oslo, Norway
T Brodowicz Medical University Vienna & General Hospital Department of Internal Medicine 1/Oncology, Vienna, Austria
D Callegaro Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
E De Alava Institute of Biomedicine of Sevilla (IBiS), Virgen del Rocio University Hospital/CSIC/University of Sevilla/CIBERONC, Seville, Spain; Department of Normal and Pathological Cytology and Histology, School of Medicine, University of Seville, Seville, Spain
M Deoras-Sutliff The EHE Foundation, Wisconsin, USA
A Dufresne Department of Medical Oncology, Centre Leon Berard, Université Claude Bernard Lyon, Unicancer, Lyon, France
M Eriksson Department of Oncology, Skane University Hospital and Lund University, Lund, Sweden
C Errani Orthopaedic Service, Musculoskeletal Oncology Department, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
A Fedenko Medical Oncology Division, P.A. Herzen Cancer Research Institute, Moscow, Russian Federation
V Ferraresi Sarcomas and Rare Tumors Unit, IRCCS Regina Elena National Cancer Institute, Rome, Italy
A Ferrari Paediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
C D M Fletcher Department of Pathology Brigham & Women's Hospital, Boston, USA
X Garcia Del Muro University of Barcelona and Genitourinary Cancer and Sarcoma Unit Institut Català d'Oncologia, Hospitalet, Barcelona, Spain
H Gelderblom Department of Medical Oncology, Leiden University Medical Center, Leiden, The Netherlands
R A Gladdy University of Toronto and Lunenfeld-Tanenbaum Research Institute, Toronto, Canada
F Gouin Department of Surgery, Centre Leon Berard, Lyon, France
G Grignani Division of Medical Oncology, Candiolo Cancer Institute, FPO - IRCCS, Candiolo, Italy
J Gutkovich The EHE Foundation, Wisconsin, USA; NUY Langone Medical Center, New York, USA
R Haas Department of Radiotherapy, the Netherlands Cancer Institute, Amsterdam, the Netherlands; Department of Radiotherapy, the Leiden University Medical Center, Leiden, the Netherlands
N Hindi Group of Advanced Therapies and Biomarkers in Sarcoma, Institute of Biomedicine of Seville (IBIS, HUVR, CSIC, Universidad de Sevilla), Seville, Spain
P Hohenberger Division of Surgical Oncology & Thoracic Surgery, Mannheim University Medical Center, University of Heidelberg, Heidelberg, Germany
P Huang Division of Molecular Pathology, The Institute of Cancer Research, London, UK
H Joensuu Department of Oncology, Helsinki University Hospital & Helsinki University, Helsinki, Finland
R L Jones Department of Cancer, The Royal Marsden Hospital and The Institute of Cancer Research, London, UK
C Jungels Institut Jules Bordet, Université Libre de Bruxelles, Brussels, Belgium
B Kasper University of Heidelberg, Mannheim University Medical Center, Sarcoma Unit, Mannheim, Germany
A Kawai Musculoskeletal Oncology and Rehabilitation Medicine, Rare Cancer Center National Cancer Center Hospital, Tokyo, Japan
A Le Cesne International Department, Gustave Roussy, Villejuif, France
F Le Grange UCLH - University College London Hospitals NHS Foundation Trust, London, UK
A Leithner Department of Orthopaedics and Trauma Medical University Graz, Graz, Austria
H Leonard Chair of Trustees of the EHE Rare Cancer Charity (UK), Charity number 1162472
A Lopez Pousa Medical Oncology Department, Hospital Universitario Santa Creu i Sant Pau, Barcelona, Spain
J Martin Broto Hospital Universitario Fundación Jimenez Diaz, Madrid, Spain
O Merimsky Unit of Soft Tissue and Bone Oncology, Division of Oncology, Tel-Aviv Medical Center affiliated with Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
P Merriam Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, USA
R Miceli Department of Clinical Epidemiology and Trial Organisation, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
O Mir Sarcoma Group, Gustave Roussy, Villejuif, France
M Molinari University of Pittsburgh Medical Center, Thomas Starzl Transplant Institute, Pittsburgh, USA
M Montemurro Oncology, CHUV Lausanne, Lausanne, Switzerland
G Oldani Division of Abdominal Surgery, University Hospitals of Geneva, Geneva, Switzerland
E Palmerini Chemotherapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
M A Pantaleo Division of Oncology, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
S Patel Sarcoma Center, The University of Texas M.D. Anderson Cancer Center, Houston, USA
S Piperno-Neumann Department of Medical Oncology, Institut Curie, Paris, France
C P Raut Department of Surgery, Brigham and Women's Hospital, Boston, USA; Center for Sarcoma and Bone Oncology, Harvard Medical School, Boston, USA; Dana Farber Cancer Center, Harvard Medical School, Boston, USA
V Ravi Sarcoma Center, The University of Texas M.D. Anderson Cancer Center, Houston, USA
A R A Razak Princess Margaret Cancer Centre and Sinai Healthcare System & Faculty of Medicine, University of Toronto, Toronto, Canada
P Reichardt Helios Klinikum Berlin-Buch, Department of Oncology and Palliative Care, Berlin, Germany
B P Rubin Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, USA
P Rutkowski Maria Sklodowska-Curie National Research Institute of Oncology, Department of Soft Tissue/Bone Sarcoma and Melanoma, Warsaw, Poland
A A Safwat Aarhus University Hospital, Aarhus, Denmark
C Sangalli Department of Radiation Oncology, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
G Sapisochin Multi-Organ Transplant and HPB Surgical Oncology, Division of General Surgery, Department of Surgery, University of Toronto, Toronto, Canada
M Sbaraglia Department of Pathology, Azienda Ospedaliera Università Padova, Padua, Italy
S Scheipl Department of Orthopaedics and Trauma, Medical University of Graz, Graz, Austria
P Schöffski Leuven Cancer Institute, Leuven, Belgium
D Strauss Department of Surgery, The Royal Marsden Hospital and The Institute of Cancer Research, London, UK
S J Strauss University College London Hospital, London, UK
K Sundby Hall Department of Oncology, Oslo University Hospital, The Norwegian Radium Hospital, Oslo, Norway
W D Tap Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, USA
A Trama Department of Research, Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
A Tweddle Palliative Care, The Royal Marsden Hospital and The Institute of Cancer Research London
W T A van der Graaf Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands
M A J Van De Sande Department of Orthopedic Surgery Bone and Soft Tissue Tumor Unit, Leiden University Medical Center, Leiden, The Netherlands
W Van Houdt Sarcoma and Melanoma Unit, The Netherlands Cancer Institute, Amsterdam, The Netherlands
G van Oortmerssen Co-Chair of Sarcoma Patients EuroNet (SPAEN), Woelfersheim, Germany & Chairman of the Dutch organisation for sarcoma patients (Patiëntenplatform Sarcomen), Guest researcher at Leiden University (Leiden Institute for Advanced Computer Science), Leiden University, Leiden, The Netherlands
A J Wagner Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, USA
M Wartenberg Chair of the Board of Directors of Sarcoma Patients EuroNet (SPAEN), Sarcoma Patients EuroNet (SPAEN), Woelfersheim, Germany
J Wood The Royal Marsden NHS Foundation Trust, London, UK
N Zaffaroni Molecular Pharmacology Unit, Department of Applied Research and Technological Development, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
C Zimmermann Department of Supportive Care, Princess Margaret Cancer Centre and Division of Palliative Medicine, Department of Medicine, University of Toronto, Toronto, Canada
P G Casali Adult Mesenchymal Tumor and Rare Cancer Unit, Cancer Medicine Department, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
A P Dei Tos Department of Pathology, Azienda Ospedaliera Università Padova, Padua, Italy
A Gronchi Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

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Kanno H, Sato T, Midorikawa R, Kojima S, Fukutomi S, Goto Y, Nomura Y, Yoshitomi M, Kawahara R, Sakai H, Hisaka T, Akagi Y, Okuda K. Hepatic Epithelioid Hemangioendothelioma Presenting Synchronously with Hepatocellular Carcinoma. Case Rep Gastroenterol 2021;15:344-351. [PMID: 33790724 PMCID: PMC7989811 DOI: 10.1159/000513803] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/15/2020] [Accepted: 12/11/2020] [Indexed: 11/19/2022] Open

Wittschieber D, Muggenthaler H, Mall G, Mentzel HJ. [Bridging vein injuries in shaken baby syndrome : Forensic-radiological meta-analysis with special focus on the tadpole sign]. Radiologe 2021;61:71-79. [PMID: 33289862 PMCID: PMC7810648 DOI: 10.1007/s00117-020-00780-5] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]

Razik A, Malla S, Goyal A, Gamanagatti S, Kandasamy D, Das CJ, Sharma R, Gupta AK. Unusual Primary Neoplasms of the Adult Liver: Review of Imaging Appearances and Differential Diagnosis. Curr Probl Diagn Radiol 2020;51:73-85. [PMID: 33199074 DOI: 10.1067/j.cpradiol.2020.10.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/16/2020] [Accepted: 10/16/2020] [Indexed: 12/24/2022]

Abstract

The radiological appearance of common primary hepatic tumors such as hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) is widely recognized. Hepatic masses with unusual histology are occasionally encountered, but seldom suspected on imaging. However, many possess characteristic imaging findings, which when assessed along with the clinical and demographic background and serum tumor markers, may enable a prospective diagnosis. This review attempts to familiarize the reader with the clinicopathological characteristics, imaging manifestations, and differential diagnosis of these unusual liver tumors in adults. Biphenotypic primary liver carcinoma is suspected in masses showing distinct areas of HCC and CCA-type enhancement pattern in cirrhotic livers. Fibrolamellar carcinoma occurs in young individuals without underlying chronic liver disease and shows a characteristic T2-hypointense scar frequently showing calcification. Perivascular epithelioid cell tumors are differentials for any arterial hyperenhancing mass in the noncirrhotic liver, particularly in patients with tuberous sclerosis. Multifocal subcapsular tumors showing target-like morphology, capsular retraction and "lollipop" sign are suspicious for epithelioid hemangioendothelioma. On the other hand, multiple hemorrhagic lesions showing patchy areas of bizarre-shaped arterial phase hyperenhancement are suspicious for angiosarcoma. Primary hepatic lymphoma (PHL) is suspected when patients with immunosuppression present with solitary or multifocal masses that insinuate around vessels and bile ducts without causing luminal narrowing. Intense diffusion restriction and low-level homogeneous or target-like enhancement are also ancillary features of PHL. Primary hepatic neuroendocrine tumor shows uptake on Ga-68 DOTANOC PET/CT. Although a straightforward diagnosis may be difficult in these cases, awareness of the characteristic imaging appearances is helpful in suspecting the diagnosis.

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Terasaki F, Yamamoto Y, Sugiura T, Okamura Y, Ito T, Ashida R, Ohgi K, Uesaka K. Laparoscopic repeat liver resection for hepatic epithelioid hemangioendothelioma. Surg Case Rep 2020;6:254. [PMID: 33001327 PMCID: PMC7530154 DOI: 10.1186/s40792-020-01036-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/21/2020] [Accepted: 09/25/2020] [Indexed: 01/23/2023] Open

Kou K, Chen YG, Zhou JP, Sun XD, Sun DW, Li SX, Lv GY. Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy. World J Clin Cases 2020;8:3978-3987. [PMID: 33024754 PMCID: PMC7520791 DOI: 10.12998/wjcc.v8.i18.3978] [Citation(s) in RCA: 30] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/06/2020] [Revised: 08/12/2020] [Accepted: 08/21/2020] [Indexed: 02/05/2023] Open

Mamone G, Di Piazza A, Carollo V, Crinò F, Vella S, Cortis K, Miraglia R. Imaging of primary malignant tumors in non-cirrhotic liver. Diagn Interv Imaging 2020;101:519-535. [PMID: 32029387 DOI: 10.1016/j.diii.2020.01.010] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2019] [Revised: 01/13/2020] [Accepted: 01/13/2020] [Indexed: 02/07/2023]

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]

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.

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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

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]

Mamone G, Miraglia R. The "Target sign" and the "Lollipop sign" in hepatic epithelioid hemangioendothelioma. Abdom Radiol (NY) 2019;44:1617-1620. [PMID: 30367214 DOI: 10.1007/s00261-018-1820-9] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]

Studer LL, Selby DM. Hepatic Epithelioid Hemangioendothelioma. Arch Pathol Lab Med 2019;142:263-267. [PMID: 29372848 DOI: 10.5858/arpa.2016-0171-rs] [Citation(s) in RCA: 44] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]

Kubo N, Harimoto N, Araki K, Hagiwara K, Yamanaka T, Ishii N, Tsukagoshi M, Igarashi T, Watanabe A, Miyazaki M, Yokoo H, Kuwano H, Shirabe K. The Feature of Solitary Small Nodular Type of Hepatic Epithelioid Hemangioendothelioma. Case Rep Gastroenterol 2018;12:402-410. [PMID: 30186092 PMCID: PMC6120404 DOI: 10.1159/000490524] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/12/2018] [Accepted: 05/29/2018] [Indexed: 01/29/2023] Open

Liver Calcifications and Calcified Liver Masses: Pattern Recognition Approach on CT. AJR Am J Roentgenol 2018;211:76-86. [DOI: 10.2214/ajr.18.19704] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]

Durot I, Wilson SR, Willmann JK. Contrast-enhanced ultrasound of malignant liver lesions. Abdom Radiol (NY) 2018;43:819-847. [PMID: 29094174 DOI: 10.1007/s00261-017-1360-8] [Citation(s) in RCA: 32] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]

Yikilmaz A, George M, Lee EY. Pediatric Hepatobiliary Neoplasms. Radiol Clin North Am 2017;55:741-766. [DOI: 10.1016/j.rcl.2017.02.003] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]

Yang JW, Li Y, Xie K, Dong W, Cao XT, Xiao WD. Spontaneous rupture of hepatic epithelioid hemangioendothelioma: A case report. World J Gastroenterol 2017;23:185-190. [PMID: 28104995 PMCID: PMC5221283 DOI: 10.3748/wjg.v23.i1.185] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/21/2016] [Revised: 09/27/2016] [Accepted: 10/27/2016] [Indexed: 02/06/2023] Open

Wu CH, Chiu NC, Yeh YC, Kuo Y, Yu SS, Weng CY, Liu CA, Chou YH, Chiou YY. Uncommon liver tumors: Case report and literature review. Medicine (Baltimore) 2016;95:e4952. [PMID: 27684838 PMCID: PMC5265931 DOI: 10.1097/md.0000000000004952] [Citation(s) in RCA: 32] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open

Abstract

BACKGROUND

Beside hepatocellular carcinoma, metastasis, and cholangiocarcinoma, the imaging findings of other relatively uncommon hepatic lesions are less discussed in the literature. Imaging diagnosis of these lesions is a daily challenge. In this article, we review the imaging characteristics of these neoplasms.

METHODS

From January 2003 to December 2014, 4746 patients underwent liver biopsy or hepatic surgical resection in our hospital. We reviewed the pathological database retrospectively. Imaging of these lesions was reviewed.

RESULTS

Imaging findings of uncommon hepatic lesions vary. We discuss the typical imaging characteristics with literature review. Clinical and pathological correlations are also described. Primary hepatic lymphoma consists only of 1% of the extranodal non-Hodgkin lymphoma, and is defined as the one involving only the liver and perihepatic lymph nodes within 6 months after diagnosis. Combined hepatocellular and cholangiocarcinoma (cHCC-CC) shares some overlapping imaging characteristics with both HCC and cholangiocarcinoma because of being an admixture of them. Angiosarcoma is the most common hepatic mesenchymal tumor and is hypervascular in nature. Inflammatory pseudotumor is often heterogeneous on ultrasonography and with enhanced septations and rims in the portovenous phase after contrast medium. Angiomyolipoma (AML) typically presents with macroscopic fat components with low signal on fat-saturated magnetic resonance imaging (MRI) and presence of drainage vessels. Intraductal papillary neoplasm of the bile duct (IPNB) is thought of as a counterpart to the pancreatic intraductal papillary mucinous neoplasm. Most of the IPNBs secrete mucin and cause disproportional dilatation of the bile ducts. Mucinous cystic neoplasm (MCN) contains proteinaceous and colloidal components without ductal communication and characterizes with hyperintensity on T1-weighted imaging. Other extremely rare lesions, including epithelioid hemangioendothelioma and inflammatory pseudotumor-like follicular dendritic cell sarcoma, are also discussed. Hepatoblastoma and mesenchymal hamartoma, mostly in children, are also briefly reviewed as well.

CONCLUSION

It is important for radiologists to be familiar with the typical imaging features of the uncommon hepatic neoplasms. If imaging findings are not typical or diagnostic, further biopsy is required.

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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

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.

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Zhou L, Cui MY, Xiong J, Dong Z, Luo Y, Xiao H, Xu L, Huang K, Li ZP, Feng ST. Spectrum of appearances on CT and MRI of hepatic epithelioid hemangioendothelioma. BMC Gastroenterol 2015;15:69. [PMID: 26088585 PMCID: PMC4474347 DOI: 10.1186/s12876-015-0299-x] [Citation(s) in RCA: 33] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/19/2015] [Accepted: 06/05/2015] [Indexed: 02/08/2023] Open

Abstract

Background

This study aims to analyze the computed tomography (CT) and magnetic resonance imaging(MRI) characteristics of hepatic epithelioid hemangioendothelioma (HEHE).

Methods

Eleven patients with histopathologically confirmed HEHE via surgical excision or biopsy were included. Imaging findings of these 11 patients were retrospectively analyzed (CT images obtained from all patients and MR images from five patients). Patterns of growth, characteristics of distribution, density/signal features, patterns of contrast enhancement, and changes of adjacent tissues were evaluated.

Results

HEHE is characterized by multiple lesions in the liver. HEHE could be further categorized as three types when considering patterns of growth: nodular type(5 cases), coalescent type(1 case) and mixed type(5 cases). In this study, a total of 312 lesions were detected, 214(74.3 %) of which were subcapsular. All lesions appeared as hypodense while round lower density were found within 10 lesions(<2 cm) on unenhanced CT images. On MRI, all lesions demonstrated low signal intensity on T1 weighted images and high heterogeneous signal intensity on T2 weighted images when compared to the normal liver parenchyma. Other imaging features included “lollipop sign”(6 cases) and capsular retraction(6 cases). On contrast-enhanced CT and MRI, lesions smaller than 2.0 cm mostly showed mild homogeneous enhancement (214/227, 94.3 %); lesions measuring 2.0–3.0 cm in diameter showed ring-like enhancement (16/53,30.2 %) and heterogeneous delayed enhancement (29/53,54.7 %); lesions larger than 3.0 cm demonstrated heterogeneous delayed enhancement (26/32, 81.3 %).

Conclusion

The imaging findings of HEHE showed some typical imaging features and size-dependent patterns with contrast enhancement on both CT and MR images, these features can be used for accurate imaging diagnosis of HEHE.

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Pedrassa BC, da Rocha EL, Kierszenbaum ML, Bormann RL, Torres LR, D'Ippolito G. Uncommon hepatic tumors: iconographic essay - Part 1. Radiol Bras 2015;47:310-6. [PMID: 25741106 PMCID: PMC4341401 DOI: 10.1590/0100-3984.2013.1760] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2013] [Accepted: 10/14/2013] [Indexed: 01/04/2023] Open

Lamba R, Fananazapir G, Corwin MT, Khatri VP. Diagnostic Imaging of Hepatic Lesions in Adults. Surg Oncol Clin N Am 2014;23:789-820. [DOI: 10.1016/j.soc.2014.07.003] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]

Fan F, Yang X, Zhu B, Zhang Y. Clinical and radiological characteristics of Chinese patients with hepatic epithelioid hemangioendothelioma. Ann Saudi Med 2013;33:334-8. [PMID: 24060710 PMCID: PMC6078508 DOI: 10.5144/0256-4947.2013.334] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/16/2023] Open

Abstract

BACKGROUND AND OBJECTIVES

To study clinical features, imaging findings, and prognosis of hepatic epithelioid hemangioendothelioma (HEHE) in Eastern Hepatobiliary Surgical Hospital and to improve the level of recognition and preoperative diagnosis of HEHE.

DESIGN AND SETTINGS

This is a retrospective study. The study was conducted at Eastern Hepatobiliary Surgical Hospital in Shanghai, China from 1999 to 2012.

METHODS

Clinical data of 15 pathology-confirmed HEHE patients admitted in our hospital from 1999-2010 were collected and analyzed retrospectively.

RESULTS

Besides 7 cases of abdominal discomfort, this cohort of HEHE patients does not show common typical manifestations. Laboratory examinations found 8 low ALB/GLB cases, 3 mild anemia cases, and 1 high alanine aminotransferase case, but no abnormal a-fetoprotein, carcinoembryonic antigen, or CA19-9 cases. Plain computed tomography (CT) scanning found uneven multiple low-density lesions in most cases, dynamic enhanced CT scanning demonstrated peripheral enhancement in the hepatic arterial HA phase and more peripheral enhancement in the portal vein PV phase, and ring enhancement were detected in the delayed phase. Magnetic resonance imaging (MRI) demonstrated low signal intensity and even lower lesion centers in T1-weighted im.ages, but slightly high signal intensity lesions and higher lesion centers in T2-weighted images. Enhanced MRI scanning showed ring enhancements but no obvious enhanced centers. Lesions were further enhanced in delayed MRI scanning. Prognosis of this cohort: 10 of these 15 patients were alive, including 4 cases with a tumor.

CONCLUSION

Comprehending the clinical and radiological characteristics of HEHE facilitates the level of recognition and preoperative diagnosis of this disease, and promotes surgeons to take active and appropriate surgeries.

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Lakkis Z, Kim S, Delabrousse E, Jary M, Nguyen T, Mantion G, Heyd B, Lassabe C, Borg C. Metronomic cyclophosphamide: an alternative treatment for hepatic epithelioid hemangioendothelioma. J Hepatol 2013;58:1254-7. [PMID: 23402747 DOI: 10.1016/j.jhep.2013.01.043] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/27/2012] [Revised: 01/28/2013] [Accepted: 01/31/2013] [Indexed: 02/07/2023]