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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
Abstract
Malignant hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor with variable malignant potential, often affecting middle-aged individuals. Hepatic involvement is uncommon, presenting diagnostic challenges due to overlapping imaging features with other liver pathologies. We report a case of a 35-year-old female with HEHE initially presenting with epigastric pain and an incidental liver mass. Imaging revealed multifocal hepatic lesions, confirmed as HEHE through biopsy and immunohistochemistry. Despite initial stability on tamoxifen, disease progression occurred after seven years, manifesting as an increase in the size and number of hepatic lesions. Hepatic transplantation was planned, which was refused by the patient. Subsequently, the disease progressed to a large confluent hepatic mass with calcifications, lymphadenopathy, and pulmonary metastases. Systemic chemotherapy was henceforth initiated. This case underscores the importance of a multimodal approach integrating imaging, histopathology, and tailored therapeutic strategies for diagnosing and managing HEHE. Early detection and comprehensive management are critical to improving outcomes where liver transplantation offers a potential cure.
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Affiliation(s)
- Mariam Malik
- Department of Radiology, Nuclear Medicine, Oncology and Radiotherapy Institute-Atomic Energy Cancer Hospital (NORI-AECH), Islamabad, PAK
| | - Rana Bilal Idrees
- Department of Radiology, Institute of Nuclear Medicine and Oncology-Atomic Energy Cancer Hospital (INMOL-AECH), Lahore, PAK
| | - Zeeshan Mirza
- Department of Radiology, Institute of Nuclear Medicine and Oncology-Atomic Energy Cancer Hospital (INMOL-AECH), Lahore, PAK
| | - Sadia Anwar
- Department of Radiology, Institute of Nuclear Medicine and Oncology-Atomic Energy Cancer Hospital (INMOL-AECH), Lahore, PAK
| | - Barira Ahmad
- Department of Radiology, Institute of Nuclear Medicine and Oncology-Atomic Energy Cancer Hospital (INMOL-AECH), Lahore, PAK
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Nie Y, Jing W, Zheng X, He X, Chen M, Zhang H. CAMTA1-immunonegative epithelioid hemangioendotheliomas of the liver: a clinicopathological and molecular analysis of seven cases. Front Oncol 2025; 15:1478036. [PMID: 40040722 PMCID: PMC11876046 DOI: 10.3389/fonc.2025.1478036] [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: 08/09/2024] [Accepted: 01/27/2025] [Indexed: 03/06/2025] Open
Abstract
Background Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor. Most EHEs (>90%) cases harbor WWTR1::CAMTA1 fusion gene, and CAMTA1 immunohistochemistry (IHC) is a highly sensitive and specific tool for EHE diagnosis. However, there exist CAMTA1-immunonegative cases, the majority of which harbor YAP1::TFE3 fusion, with a few cases having more rare fusions. Liver is one of the most common sites of EHE, where the CAMTA1 subtype dominates, and the other variants are extremely rare. Hence, we focused on the hepatic CAMTA1-immunonegative EHEs to analyze the clinicopathological and molecular features of these peculiar cases. Methods The SNOMED search of the hospital pathology files between January 2016 to November 2023 identified 57 hepatic EHEs and 7 cases were CAMTA1-immunonegative. Fluorescence in situ hybridization (FISH), next generation sequencing (NGS) and Sanger sequencing were performed to identify the genetic change of the 7 cases. Results This series included 3 females and 4 males, aged from 33 to 64 years. All the 7 cases were negative for CAMTA1 IHC. Four cases were positive for TFE3 IHC and exhibited YAP1::TFE3 fusion. Another 3 cases were also negative for TFE3, while WWTR1::CAMTA1 fusion were detected by NGS in 1 case and demonstrated by FISH in all the 3 cases. Morphologically, among the 4 TFE3 rearrangement cases, 3 cases showed the TFE3-sutype morphologic appearance, while the histology of 1 case was similar to that of CAMTA1- subtype. In the 3 CAMTA1-rearranged lesions, 2 cases had classic EHE morphology, and 1 case exhibited atypical histology, with higher atypia and well-formed vessels. Surgical resection was performed on five cases and two cases were biopsied and received chemotherapy. Follow-up information was available in 6 patients (median 46 months), including 4 patients were alive without disease and 2 patients were alive with disease. Conclusion Our study reported 7 CAMTA1-immunonegative hepatic EHEs and most of them were TFE3-rearranged EHEs with morphology variation. Moreover, there does exist the CAMTA1-immunonegative but CAMTA1-rearranged EHE cases. Therefore, the diagnosis of EHE should be based on morphology, combined with CAMTA1 and TFE3 IHC, and if necessary, supplemented by genetic analysis including FISH and NGS, to establish correct diagnosis.
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Affiliation(s)
| | | | | | | | | | - Hongying Zhang
- Department of Pathology, West China Hospital, Sichuan University,
Chengdu, China
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3
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Sawma T, Sultan A, Abdulmoneim S, Grotz T, Rosen CB, Taner T, Heimbach JK, Warner SG, Siontis BL, Ho TP, Robinson SI, Thiels CA. Management and Long-Term Outcomes of Patients With Hepatic Epithelioid Hemangioendothelioma. J Surg Oncol 2024; 130:1062-1069. [PMID: 39318157 DOI: 10.1002/jso.27807] [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: 07/13/2024] [Accepted: 07/26/2024] [Indexed: 09/26/2024]
Abstract
BACKGROUND AND OBJECTIVES Hepatic epithelioid hemangioendothelioma (HEHE) is an uncommon vascular neoplasm characterized by variable clinical behavior. Our aim was to describe the therapeutic approach for HEHE at diagnosis and define clinicopathological characteristics associated with tumor progression and long-term survival. METHODS This is a retrospective study that includes patients with HEHE who received treatment at Mayo Clinic Rochester between 1984 and 2023. RESULTS Eighty patients were included in the study (median age: 44 years; 62.5% female), 24 underwent liver transplantation, 26 underwent liver resection, and 30 were managed medically. The 3-year overall survival rates were 86.7%, 80.9%, and 51.1%, respectively. Notably, 26 patients had extrahepatic metastases at the time of diagnosis, four (16.7%) in the transplantation group, four (15.4%) in the resection group, and 18 (69.2%) in the nonsurgical group. On multivariable modeling, bone metastasis was independently associated with long-term mortality (HR 6.3, p < 0.001) while lung metastasis and surgical intervention were not associated with long-term mortality (HR 0.8, p = 0.8; HR 1.1, p = 0.9, respectively). CONCLUSION Bone metastasis emerged as a strong predictor of poor survival. Hence, aggressive surgical intervention may not be advantageous in patients with skeletal metastases but can still be offered for those with other extrahepatic metastases.
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Affiliation(s)
- Tedy Sawma
- Division of Hepatobiliary & Pancreas Surgery, Mayo Clinic, Rochester, Minnesota, USA
| | - Ahmer Sultan
- Division of Hepatobiliary & Pancreas Surgery, Mayo Clinic, Rochester, Minnesota, USA
| | - Samer Abdulmoneim
- Division of Hepatobiliary & Pancreas Surgery, Mayo Clinic, Rochester, Minnesota, USA
| | - Travis Grotz
- Division of Hepatobiliary & Pancreas Surgery, Mayo Clinic, Rochester, Minnesota, USA
| | - Charles B Rosen
- Division of Transplantation Surgery, Mayo Clinic, Rochester, Minnesota, USA
| | - Timucin Taner
- Division of Transplantation Surgery, Mayo Clinic, Rochester, Minnesota, USA
| | - Julie K Heimbach
- Division of Transplantation Surgery, Mayo Clinic, Rochester, Minnesota, USA
| | - Susanne G Warner
- Division of Hepatobiliary & Pancreas Surgery, Mayo Clinic, Rochester, Minnesota, USA
| | | | - Thanh P Ho
- Department of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA
| | - Steven I Robinson
- Department of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA
| | - Cornelius A Thiels
- Division of Hepatobiliary & Pancreas Surgery, Mayo Clinic, Rochester, Minnesota, USA
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4
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Narayanan G, Spano A, Gentile NT, Shnayder-Adams MM, Gurusamy V, Levi DM, Wilky BA, Mora RA, Noman R, Peddu P, Dijkstra M. Irreversible Electroporation as a Valid Treatment Option for Hepatic Epithelioid Hemangioendothelioma: An International Multicenter Experience. Cardiovasc Intervent Radiol 2024; 47:883-890. [PMID: 38844684 PMCID: PMC11239779 DOI: 10.1007/s00270-024-03770-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/05/2024] [Accepted: 05/22/2024] [Indexed: 07/12/2024]
Abstract
PURPOSE Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor with currently no established standard of care. This international multicenter retrospective study assesses the use of percutaneous irreversible electroporation (IRE) as an ablative tool to treat HEHE and provides a clinical overview of the current management and role of IRE in HEHE treatment. MATERIAL AND METHODS Between 2017 and 2023, 14 patients with 47 HEHE tumors were treated with percutaneous IRE using CT-scan guidance in 23 procedures. Baseline patient and tumor characteristics were evaluated. Primary outcome measures included safety and effectiveness, analyzed using Common Terminology Criteria for Adverse Events (CTCAE) and treatment response by mRECIST criteria. Secondary outcome measures included technical success, post-treatment tumor sizes and length of hospital stay. Technical success was defined as complete ablation with an adequate ablative margin (intentional tumor free ablation margin > 5 mm). RESULTS IRE treatment resulted in technical success in all tumors. Following a median follow-up of 15 months, 30 tumors demonstrated a complete response according to mRECIST criteria. The average tumor size pre-treatment was 25.8 mm, accompanied by an average reduction in tumor size by 7.5 mm. In 38 out of 47 tumors, there was no evidence of local recurrence. In nine tumors, residual tumor was present. There were no cases of progressive disease. Median length of hospital stay was one day. Only one grade 3 CTCAE event occurred, a pneumothorax requiring chest tube placement. CONCLUSION The current study provides evidence that IRE is a safe and efficacious minimally invasive treatment option for HEHE.
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Affiliation(s)
- Govindarajan Narayanan
- Herbert Wertheim College of Medicine, Florida International University, Miami, FL, USA
- Department of Interventional Oncology, Miami Cancer Institute, Baptist Health South Florida, Miami, FL, USA
- Department of Interventional Radiology, Miami Cardiac and Vascular Institute, Baptist Health South Florida, Miami, FL, USA
| | - Anthony Spano
- Department of Interventional Radiology, Miami Cardiac and Vascular Institute, Baptist Health South Florida, Miami, FL, USA
| | - Nicole T Gentile
- Department of Interventional Oncology, Miami Cancer Institute, Baptist Health South Florida, Miami, FL, USA
- Department of Interventional Radiology, Miami Cardiac and Vascular Institute, Baptist Health South Florida, Miami, FL, USA
| | - Michelle M Shnayder-Adams
- Division of Vascular and Interventional Radiology, University of Michigan Hospital, Ann Arbor, MI, USA
| | - Varshana Gurusamy
- Department of Interventional Radiology, MD Anderson Cancer Center, Houston, TX, USA
| | - David M Levi
- Division of Abdominal Transplant Surgery, Atrium Health Carolinas Medical Center, Charlotte, NC, USA
| | | | - Ronald A Mora
- Department of Interventional Radiology, Miami Cardiac and Vascular Institute, Baptist Health South Florida, Miami, FL, USA
| | - Raihan Noman
- Department of Radiology, Rutgers New Jersey Medical School, Newark, NJ, USA
| | - Praveen Peddu
- Department of Radiology, King's College Hospital NHS Trust, London, UK
| | - Madelon Dijkstra
- Department of Interventional Oncology, Miami Cancer Institute, Baptist Health South Florida, Miami, FL, USA.
- Department of Interventional Radiology, Miami Cardiac and Vascular Institute, Baptist Health South Florida, Miami, FL, USA.
- Department of Radiology and Nuclear Medicine, Amsterdam UMC, Location VUmc, Cancer Center Amsterdam, Amsterdam, The Netherlands.
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Fang J, Ji Y, Zhao W, Pu C, Mi S, Zhang B. Three cases of hepatic epithelioid hemangioendothelioma evaluated using conventional and contrast-enhanced ultrasound: Case reports. JOURNAL OF CLINICAL ULTRASOUND : JCU 2022; 50:826-831. [PMID: 35018654 PMCID: PMC9540860 DOI: 10.1002/jcu.23141] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/15/2021] [Revised: 01/04/2022] [Accepted: 01/05/2022] [Indexed: 06/14/2023]
Abstract
Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare vascular endothelial cell tumor, which lacks typical clinical manifestations and specificity of imaging features. Whether the background of fatty liver and the difference in Contrast enhanced ultrasound (CEUS) characteristics between large and small lesions has not been well defined. In this case reports, we described the ultrasound image features of three patients with HEHE. These three patients with HEHE have certain similar characteristics of conventional ultrasound and CEUS. CEUS imaging features include large nodules show earlier perfusion than liver parenchyma, with rim-enhancement, nonenhancing regions in the center, while small nodules show earlier perfusion than liver parenchyma, with hyperenhancement. All nodules show faster washout than hepatic parenchyma, showing heterogeneous hypoenhancement, and more washout lesions can be found in the PVP and LP. Conventional ultrasound and CEUS not only help to improve the diagnostic confidence of HEHE of rare liver tumors, but also can guide the biopsy area, making it easier to make accurate pathological diagnosis.
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Affiliation(s)
- Jian‐Qiang Fang
- Department of Interventional UltrasoundXianyang Central HospitalXianyangChina
| | - Ya‐Yun Ji
- Department of Interventional UltrasoundXianyang Central HospitalXianyangChina
| | - Wei‐An Zhao
- Department of Interventional UltrasoundXianyang Central HospitalXianyangChina
| | - Cui Pu
- Department of Interventional UltrasoundXianyang Central HospitalXianyangChina
| | - Si‐Yuan Mi
- Department of Interventional UltrasoundXianyang Central HospitalXianyangChina
| | - Bin‐Yu Zhang
- Department of Interventional UltrasoundXianyang Central HospitalXianyangChina
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6
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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
Abstract
BACKGROUND Hepatic epithelioid hemangioendothelioma (EHE) is a rare vascular endothelial cell tumor of the liver, consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. Immunohistochemistry is usually positive for vascular markers, such as factor VIII-related antigen, CD31, and CD34. Hepatic EHE can have a varied clinical course; treatment includes liver transplantation, liver resection, chemotherapy, and radiation therapy.
CASE SUMMARY A 46-year-old woman with abdominal discomfort and elevated serum carcinoembryonic antigen was found to have multiple low-density lesions in the liver and lung on computed tomography (CT) evaluation. An ultrasound-guided fine needle aspiration biopsy revealed a fibrous stroma with dendritic cells, containing intracellular vacuoles. Immunohistochemical staining found that the tumor cells were positive for CD34, CD31, and factor VIII-related antigen. The patient received four courses of combined chemotherapy and was followed-up for 13 years, at which time the patient was in stable condition without disease progression and a confined neoplasm, as evidenced by CT scans.
CONCLUSION The histology and immunohistochemical characteristics of hepatic EHE are well described. Chemotherapy may be effective in patients with extrahepatic lesions.
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Affiliation(s)
- Wei-Fang Mo
- Department of General Practice, The 2nd Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou 310009, Zhejiang Province,China
| | - Yu-Ling Tong
- Department of General Practice, The 2nd Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou 310009, Zhejiang Province,China
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7
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Khan N, Waheed S, Alkhateb R. Triple whammy: a rare case of epithelioid hemangioendothelioma with synchronous angiomyomatous hamartoma complicated by Actinomyces meyeri. Arch Clin Cases 2022; 8:56-61. [PMID: 34984227 PMCID: PMC8717002 DOI: 10.22551/2021.32.0803.10187] [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] [Indexed: 11/25/2022] Open
Abstract
Epithelioid hemangioendothelioma is a rare vascular malignancy that originates from vascular endothelial or pre-endothelial cells and is composed of epithelioid or histiocytoid cells. This malignancy has an incidence of approximately one per one million individuals and can occur in various regions of the body including the lungs, liver, bones, and soft tissues. The behavior of this cancer can range from indolent to aggressive and diagnosis and treatment are often delayed due to variable presentations and lack of established treatment guidelines. Here we present the case of a 27-year-old Hispanic male that presented with right groin pain, abdominal pain, and a fifty-pound weight loss over one year. The patient had a complex hospital course during which he was found to have an angiomyomatous hamartoma of his right groin area, postsurgical right inguinal wound infection with Actinomyces meyeri, and epithelioid hemangioendothelioma distal to the right iliac bifurcation. The patient is currently pending further imaging studies to evaluate candidacy for surgical resection and following with oncology for chemotherapeutic options.
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Affiliation(s)
- Nariman Khan
- Department of Internal Medicine, University of Texas Health Science Center San Antonio, TX, USA
| | - Saadia Waheed
- Department of Internal Medicine, University of Texas Health Science Center San Antonio, TX, USA
| | - Rahaf Alkhateb
- Department of Pathology and Laboratory Medicine, University of Texas Health Science Center San Antonio, TX, USA
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8
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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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Affiliation(s)
- Badr Serji
- Surgical Oncology Department, Mohammed VI University Hospital, Regional Oncology Center, Oujda, Morocco.,Mohammed First University Oujda, Faculty of Medicine and Pharmacy Oujda, Oujda, Morocco
| | - Abdelbassir Ramdani
- Surgical Oncology Department, Mohammed VI University Hospital, Regional Oncology Center, Oujda, Morocco.,Mohammed First University Oujda, Faculty of Medicine and Pharmacy Oujda, Oujda, Morocco
| | - Houda Mirali
- Mohammed First University Oujda, Faculty of Medicine and Pharmacy Oujda, Oujda, Morocco.,Radiology Department, Mohammed VI University Hospital, Oujda, Morocco
| | - Tariq Bouhout
- Surgical Oncology Department, Mohammed VI University Hospital, Regional Oncology Center, Oujda, Morocco.,Mohammed First University Oujda, Faculty of Medicine and Pharmacy Oujda, Oujda, Morocco
| | - Amal Bennani
- Mohammed First University Oujda, Faculty of Medicine and Pharmacy Oujda, Oujda, Morocco.,Pathology Department, Mohammed VI University Hospital, Oujda, Morocco
| | - Tijani El Harroudi
- Surgical Oncology Department, Mohammed VI University Hospital, Regional Oncology Center, Oujda, Morocco.,Mohammed First University Oujda, Faculty of Medicine and Pharmacy Oujda, Oujda, Morocco
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9
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Witte S, Weidema M, Kaal S, Versleijen-Jonkers Y, Flucke U, van der Graaf W, Desar I. The heterogeneity of Epithelioid Hemangioendothelioma (EHE): A case series and review of the literature with emphasis on treatment options. Semin Oncol 2021; 48:111-118. [PMID: 34176654 DOI: 10.1053/j.seminoncol.2021.04.002] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/16/2019] [Revised: 04/07/2021] [Accepted: 04/14/2021] [Indexed: 12/28/2022]
Abstract
Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular sarcoma with a very heterogeneous presentation and prognosis. We here present five cases of EHE emphasizing diversity in presentation, treatment, and prognosis. Furthermore, we present a review of the literature on EHE treatment options.
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Affiliation(s)
- Stijn Witte
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | - Marije Weidema
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | - Suzanne Kaal
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | | | - Uta Flucke
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | - Winette van der Graaf
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | - Ingrid Desar
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands.
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10
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Huda T, Parwez MM, Pandya B. Metastatic Hepatic Epitheloid Hemangioendothelioma in a Young Male: A Rare Presentation. Gastrointest Tumors 2021; 8:58-62. [PMID: 33981683 DOI: 10.1159/000513963] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/26/2020] [Accepted: 12/22/2020] [Indexed: 12/19/2022] Open
Abstract
Hepatic EHE (epithelioid hemangioendothelioma) is an uncommon entity of vascular origin and a low-grade malignant tumor. Primary hepatic EHE is rare. These tumors can be multifocal at presentation like in the soft tissues, bones, brain, liver, and small intestine. First described by Weiss and Enzinger in 1982 as a malignant vascular neoplasm with indolent behavior. We report the case of a 23-year-old male, known case of chronic liver disease, who presented with incisional hernia following exploratory laparotomy 8 months back, performed for intestinal obstruction. Contrast-enhanced computed tomography abdomen revealed an incisional hernia with a large defect along with multiple lesions in the liver (suspected metastases) and peritoneal deposits and a few discrete lung nodules. Some areas of interloop collections were also noted. Biopsies were taken from the liver which revealed benign cirrhotic lesion. Relevant to the findings, multiple biopsies were performed and fluid was sent for evaluation. The liver biopsy came out positive for borderline vascular malignancy (epithelioid hemangioendothelioma). This was confirmed with the immunohistochemistry report. Epithelioid hemangioendothelioma occurs mostly in soft tissues of extremity and lungs. The involvement of the liver may be seen as metastasis or rarely as a primary tumor. The incidence of primary malignant hepatic hemangioendothelioma is about 0.1/100,000; the mean age at the time of diagnosis is 41.7 years, and male:female ratio is 2:3. Liver transplantation, hepatectomy, chemoembolization, radiotherapy, and chemotherapeutic agents are reported treatment regimens. Malignant EHE of liver presents as multiple hepatic nodules. Being locally aggressive, it can invade the peritoneum, gut, and lungs. Orthoptic liver transplantation appears to be the only remedy because of the multifocal nature of the disease. Partial hepatectomy is possible for localized tumors.
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Affiliation(s)
- Tanweerul Huda
- Department of General Surgery, AIIMS, Bhopal, Madhya Pradesh, India
| | | | - Bharati Pandya
- Department of General Surgery, AIIMS, Bhopal, Madhya Pradesh, India
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11
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Aktas A, Probst D, Van Tine B, Marlow K. Hepatic epithelioid hemangioendothelioma associated with acute disseminated encephalomyelitis by a possible paraneoplastic process. Rare Tumors 2020; 12:2036361320977012. [PMID: 33294143 PMCID: PMC7705382 DOI: 10.1177/2036361320977012] [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: 05/14/2019] [Accepted: 11/05/2020] [Indexed: 11/16/2022] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is a low-grade, malignant vascular neoplasm that frequently involves the liver, lungs, bone, and soft tissue. Although not commonly associated with a paraneoplastic syndrome, paraneoplastic syndromes in the setting of EHE have been reported. Acute disseminated encephalomyelitis (ADEM) is an acute, autoimmune, demyelinating disorder of the central nervous system that most commonly occurs after an infection or vaccination. We present the case of a 23 year old female who developed the acute onset of fevers, tremors, right sided hemiplegia, global aphasia, and incontinence of urine and stool. MRI demonstrated findings consistent with a demyelinating disorder and brain biopsy confirmed the diagnosis of ADEM. The patient's work up revealed multiple liver lesions which were biopsy proven EHE. This case report discusses the diagnosis and treatment of two concurrent rare disease processes and the possible association of the processes via a paraneoplastic syndrome.
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Affiliation(s)
- Adem Aktas
- Division of Neurorehabilitation, Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA
| | - Daniel Probst
- Division of Neurorehabilitation, Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA
| | - Brian Van Tine
- Division of Oncology, Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA
| | - Kathryn Marlow
- Division of Neurorehabilitation, Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA
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12
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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
Abstract
With an estimated incidence of only 1-2 cases in every 1 million people, hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. HEHE is characterized as a low-to-moderate grade malignant tumor and is classified into three types: solitary, multiple, and diffuse. Both the etiology and characteristic clinical manifestations of HEHE are unclear. However, HEHE has a characteristic appearance on imaging including ultrasound, magnetic resonance imaging, and positron emission tomography/computerized tomography. Still, its diagnosis depends mainly on pathological findings, with immunohistochemical detection of endothelial markers cluster of differentiation 31 (CD31), CD34, CD10, vimentin, and factor VIII antigen as the basis of diagnosis. Hepatectomy and/or liver transplantation are the first choice for treatment, but various chemotherapeutic drugs are reportedly effective, providing a promising treatment option. In this review, we summarize the literature related to the diagnosis and treatment of HEHE, which provides future perspectives for the clinical management of HEHE.
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Affiliation(s)
- Kai Kou
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - Yu-Guo Chen
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - Jian-Peng Zhou
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - Xiao-Dong Sun
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - Da-Wei Sun
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - Shu-Xuan Li
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - Guo-Yue Lv
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
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Din NU, Rahim S, Asghari T, Abdul-Ghafar J, Ahmad Z. Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases. Diagn Pathol 2020; 15:120. [PMID: 32977811 PMCID: PMC7519523 DOI: 10.1186/s13000-020-01039-2] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2020] [Accepted: 09/21/2020] [Indexed: 11/10/2022] Open
Abstract
Background Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature. Methods All cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained. Results Seven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon. Conclusion Clinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.
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Affiliation(s)
- Nasir Ud Din
- Section of Histopathology, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
| | - Shabina Rahim
- Section of Histopathology, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
| | - Tamana Asghari
- Section of Histopathology, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
| | - Jamshid Abdul-Ghafar
- Department of Pathology and Clinical Laboratory, French Medical Institute for Mothers and Children (FMIC), Kabul, Afghanistan.
| | - Zubair Ahmad
- Section of Histopathology, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
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Lee SS, Lee JH. Surgical treatment of asymptomatic epithelioid hemangioendothelioma originating from the superior vena cava: A case report. Medicine (Baltimore) 2020; 99:e19859. [PMID: 32312011 PMCID: PMC7220394 DOI: 10.1097/md.0000000000019859] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
Abstract
RATIONALE Epithelioid hemangioendothelioma is a rare endothelial tumor with a low-grade malignancy. This tumor can be treated with complete resection. PATIENT CONCERNS A 20-year-old Korean man visited our hospital due to an abnormal finding on standing chest PA X-ray. He did not have any past medical history. DIAGNOSIS Chest computed tomography shows a well-defined, oval-shaped tumor invading the brachiocephalic vein and superior vena cava. A malignant tumor of vascular origin was diagnosed by a percutaneous needle biopsy. INTERVENTIONS We performed en-bloc resection including the great vessels for complete resection of the tumor. Histologic evaluation confirmed the lesion to be a hemangioendothelioma and the surgical margins were free from tumor invasion. OUTCOMES Fourteen days later, the patient was discharged without any complication. Thirty months after surgery, recurrences, or metastasis were not detected. LESSONS Epithelioid hemangioendothelioma is a rare malignant endothelial tumor in the central vein. Surgery is the treatment of choice and shows good results. We introduce and appropriate surgical method to ensure successful treatment for rare disease.
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Affiliation(s)
- Seok Soo Lee
- Department of Thoracic and Cardiovascular Surgery, Yeungnam University Medical Center
| | - Jang Hoon Lee
- Department of Thoracic and Cardiovascular Surgery, Yeungnam University college of Medicine, Daegu, Republic of Korea
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Kyriazoglou A, Koutsoukos K, Zagouri F, Liontos M, Dimitriadis E, Tiniakos D, Dimopoulos MA. Metastatic Hepatic Epithelioid Hemangioendothelioma Treated with Olaratumab: A Falling Star Rising? Ther Clin Risk Manag 2020; 16:141-146. [PMID: 32161464 PMCID: PMC7051805 DOI: 10.2147/tcrm.s220804] [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: 06/25/2019] [Accepted: 11/12/2019] [Indexed: 11/23/2022] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare vascular malignant tumor with indolent course. Liver transplantation for local disease is the treatment of choice. In the metastatic setting there is no consensus regarding the appropriate systemic treatment. We present two cases of metastatic hepatic epithelioid hemangioendothelioma (hEHE) treated with the combination of Doxorubicin and Olaratumab. Both patients showed Stable Disease (SD) as a response, after the completion of six cycles of this combination therapy.
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Affiliation(s)
| | | | - Flora Zagouri
- Department of Clinical Therapeutics, General Hospital Alexandra, Athens, Greece
| | - Michalis Liontos
- Department of Clinical Therapeutics, General Hospital Alexandra, Athens, Greece
| | | | - Dina Tiniakos
- Department of Pathology Aretaion Hospital, National & Kapodistrian University of Athens, Athens, Greece.,Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK
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Noh OK, Kim SS, Yang MJ, Lim SG, Hwang JC, Cho HJ, Cheong JY, Cho SW. Treatment and prognosis of hepatic epithelioid hemangioendothelioma based on SEER data analysis from 1973 to 2014. Hepatobiliary Pancreat Dis Int 2020; 19:29-35. [PMID: 31822393 DOI: 10.1016/j.hbpd.2019.11.006] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/09/2019] [Accepted: 11/25/2019] [Indexed: 02/06/2023]
Abstract
BACKGROUND Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of vascular origin with an unknown etiology, a low incidence, and a variable natural course. We evaluated the management and prognosis of HEH from the Surveillance, Epidemiology and End Results (SEER) program and changes in treatment modalities of HEH over 30 years. METHODS From 1973 to 2014 in the SEER database, we selected patients diagnosed with HEH. We analyzed the clinical characteristics, patterns of management, and clinical outcomes of patients with HEH. RESULTS We identified 79 patients with HEH (median age: 54.0 years; male to female ratio: 1:2.6). The initial extent of disease was local in 22 (27.8%) patients, regional metastasis in 22 (27.8%), distant metastasis in 31 (39.2%) and unknown in 4 (5.1%). The median size of primary tumor was 3.85 cm (interquartile range, 2.50-7.93 cm). Among 74 patients with available management data, the most common management was no treatment (29/74, 39.2%), followed by chemotherapy only (22/74, 29.7%), liver resection-based (13/74, 17.6%), and transplantation-based therapy (6/74, 8.1%). The 5-year cancer-specific survival rate was 57.8%. Patients who underwent surgical treatment had significantly higher survival than those who underwent non-surgical treatment (5-year survival; 88% vs. 49%, P = 0.019). Multivariate analysis revealed that surgical therapy was the only independent prognostic factor for survival (hazard ratio: 0.20, P = 0.040). CONCLUSIONS Resection or liver transplantation is worth considering for treatment of patients with HEH.
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Affiliation(s)
- O Kyu Noh
- Department of Radiation Oncology, Ajou University School of Medicine, Suwon, Korea; Department of Biomedical Informatics, Ajou University School of Medicine, Suwon, Korea; Office of Biostatistics, Ajou Research Institute for Innovative Medicine, Ajou University Medical Center, Suwon, Korea
| | - Soon Sun Kim
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea.
| | - Min Jae Yang
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Sun Gyo Lim
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Jae Chul Hwang
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Hyo Jung Cho
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Jae Youn Cheong
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Sung Won Cho
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
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Lund PS, Willemoe GL, Hansen LB, Warnecke M. Hepatic epitheloid haemangioendothelioma: a rare malignant tumour. BMJ Case Rep 2020; 13:13/1/e232794. [PMID: 31996387 DOI: 10.1136/bcr-2019-232794] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Our case concerns a 66-year-old man. After experiencing recurrent episodes of abdominal pain, an initial CT scan, ultrasound and gastroscopy was carried out. All of which showed normal findings.As a consequence of persisting symptoms, another CT scan was performed. This scan revealed a hypodense area in the right lobe of the liver. This was interpreted as a possible haemangioma. Subsequent MRI scans indicated an intrahepatic cholangiocarcinoma. A final ultrasound-guided liver biopsy was performed and histology demonstrated epitheloid haemangioendothelioma, which was locally advanced and inoperable.
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Affiliation(s)
| | | | | | - Mads Warnecke
- Pathology, Zealand University Hospital Koge, Koge, Denmark
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Should living donor liver transplant selection be subject to the same restrictions as deceased donor transplant? Curr Opin Organ Transplant 2019; 25:47-51. [PMID: 31834006 DOI: 10.1097/mot.0000000000000728] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
PURPOSE OF REVIEW In the United States, most of the liver allografts come from deceased donors, and our current liver recipient selection process is heavily centered on the ethical principle of utility to maximize the net benefit to the liver recipient community as a group rather than individuals due to the organ scarcity. Although living donor liver transplantation contributes less than 5% of total liver transplant in the United States, these living donor recipients are being subjected to the same selection process designed to benefit the group as a whole rather than the individuals. We would like to examine if these recipients who have living donors should be subjected to the same selection process. RECENT FINDINGS There are several disease processes where liver transplantation is the only curative option, and recent studies have shown clear survival benefits with liver transplantation. SUMMARY For those who have living donors, different selection criteria based on their specific disease, not based on the principle of utilization should be used to evaluate their candidacy.
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Cao L, Hong J, Zhou L, Ye Y, Liu Y, Yu J, Zheng S. Selection of treatment for hepatic epithelioid hemangioendothelioma: a single-center experience. World J Surg Oncol 2019; 17:183. [PMID: 31699108 PMCID: PMC6839190 DOI: 10.1186/s12957-019-1729-y] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2019] [Accepted: 10/15/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Hepatic epithelioid hemangioendothelioma (HEHE) is a rare angiogenic tumor with no recognized effective treatment. Treatment options used worldwide include liver transplantation (LT), liver resection (LR), radiofrequency ablation (RFA), chemotherapy, and observation. The aim of this study was to describe the efficacy of different treatment options used for HEHE at our center. METHODS The medical charts of 12 patients with HEHE (9 women and 3 men) who were diagnosed and treated at the First Affiliated Hospital of Zhejiang University, China, between January 2011 and December 2017 were retrospectively reviewed. RESULTS The patients were diagnosed by postoperative histopathology or fine needle aspiration biopsy. Two patients with diffuse lesions received LT and were alive without recurrence at the last follow-up. Three patients received LR as the initial treatment, and all of them developed recurrence during the follow-up period. One patient received RFA and remained free of disease, while the remaining six patients opted for simple observation rather than treatment. One of the patients who received LR passed away because of tumor recurrence within 32 months after surgery; the other patients showed no significant disease activity after treatments for their recurrent lesions. As of April 2018, the mean follow-up duration was 39.6 ± 20.1 months (15-82 months). CONCLUSIONS There are multiple strategies for HEHE. Considering its indolent course, initial observation for assessment of the lesion behavior may aid in the selection of appropriate treatment. Surgery or LT is suitable for patients with disease progression during the observation period. However, our sample size was small, and further studies are required to gather more information that can aid in optimal treatment selection.
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Affiliation(s)
- Linping Cao
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310003, China
| | - Jiawei Hong
- Key Laboratory of Combined Multi-Organ Transplantation, Ministry of Public Health, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310003, China
| | - Lingfeng Zhou
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310003, China
| | - Yufu Ye
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310003, China
| | - Yuanxing Liu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310003, China
| | - Jun Yu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310003, China
| | - Shusen Zheng
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310003, China. .,Key Laboratory of Combined Multi-Organ Transplantation, Ministry of Public Health, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310003, China.
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Lazăr DC, Avram MF, Romoșan I, Văcariu V, Goldiș A, Cornianu M. Malignant hepatic vascular tumors in adults: Characteristics, diagnostic difficulties and current management. World J Clin Oncol 2019; 10:110-135. [PMID: 30949442 PMCID: PMC6441663 DOI: 10.5306/wjco.v10.i3.110] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2019] [Revised: 02/23/2019] [Accepted: 03/12/2019] [Indexed: 02/06/2023] Open
Abstract
Malignant vascular tumors of the liver include rare primary hepatic mesenchymal tumors developed in the background of a normal liver parenchyma. Most of them are detected incidentally by the increased use of performing imaging techniques. Their diagnosis is challenging, involving clinical and imaging criteria, with final confirmation by histology and immunohistochemistry. Surgery represents the mainstay of treatment. Liver transplantation (LT) has improved substantially the prognosis of hepatic epithelioid hemangioendothelioma (HEHE), with 5-year patient survival rates of up to 81%, based on the European Liver Intestine Transplantation Association-European Liver Transplant Registry study. Unfortunately, the results of surgery and LT are dismal in cases of hepatic angiosarcoma (HAS). Due to the disappointing results of very short survival periods of approximately 6-7 mo after LT, because of tumor recurrence and rapid progression of the disease, HAS is considered an absolute contraindication to LT. Recurrences after surgical resection are high in cases of HEHE and invariably present in cases of HAS. The discovery of reliable prognostic markers and the elaboration of prognostic scores following LT are needed to provide the best therapeutic choice for each patient. Studies on a few patients have demonstrated the stabilization of the disease in a proportion of patients with hepatic vascular tumors using novel targeted antiangiogenic agents, cytokines or immunotherapy. These new approaches, alone or in combination with other therapeutic modalities, such as surgery and classical chemotherapy, need further investigation to assess their role in prolonging patient survival. Personalized therapeutic algorithms according to the histopathological features, behavior, molecular biology and genetics of the tumors should be elaborated in the near future for the management of patients diagnosed with primary malignant vascular tumors of the liver.
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Affiliation(s)
- Daniela Cornelia Lazăr
- Department of Internal Medicine I, University Medical Clinic, University of Medicine and Pharmacy “Victor Babeş”, Timişoara 300041, Romania
| | - Mihaela Flavia Avram
- Department of Surgery X, 1st Surgery Clinic, University of Medicine and Pharmacy “Victor Babeş”, Timişoara 300041, Romania
| | - Ioan Romoșan
- Department of Internal Medicine I, University Medical Clinic, University of Medicine and Pharmacy “Victor Babeş”, Timişoara 300041, Romania
| | - Violetta Văcariu
- Department of Internal Medicine I, University Medical Clinic, University of Medicine and Pharmacy “Victor Babeş”, Timişoara 300041, Romania
| | - Adrian Goldiș
- Department of Gastroenterology and Hepatology, University of Medicine and Pharmacy “Victor Babeş”, Timişoara 300041, Romania
| | - Mărioara Cornianu
- Department of Pathology, University of Medicine and Pharmacy “Victor Babeş”, Timişoara 300041, Romania
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