{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T17:31:27Z","timestamp":1761845487597,"version":"build-2065373602"},"reference-count":109,"publisher":"MDPI AG","issue":"24","license":[{"start":{"date-parts":[[2021,12,9]],"date-time":"2021-12-09T00:00:00Z","timestamp":1639008000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Malignant vascular tumors of the head and neck are rare neoplasms with variable clinical presentation, wide age distribution, and variable clinical courses. The heterogeneous presentation of angiosarcomas and epithelioid hemangioendothelioma often leads to misdiagnosis and unsuitable treatment. While risk factors for angiosarcomas are previous radiation, chronic lymphedema, and exposure to arsenic, thorium oxide, or vinyl chloride, there are only limited and retrospective data available on prognostic factors in EHE. In both angiosarcomas and EHE, surgery is the mainstay of treatment. There is limited evidence regarding the role of radiotherapy in EHE, although EHE is considered relatively radiosensitive. In angiosarcomas, adjuvant radiotherapy is recommended according to retrospective case series. A standard medical therapy for metastasized malignant vascular tumors is lacking. Chemotherapy, which is effective in angiosarcoma, is mostly ineffective in EHE. Targeted therapy, antiangiogenetic drugs and immunotherapy have been studied as new treatment options. The goal of this review is to summarize the current data regarding malignant vascular tumors along with their diagnosis and management.<\/jats:p>","DOI":"10.3390\/cancers13246201","type":"journal-article","created":{"date-parts":[[2021,12,9]],"date-time":"2021-12-09T21:52:32Z","timestamp":1639086752000},"page":"6201","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Malignant Vascular Tumors of the Head and Neck\u2014Which Type of Therapy Works Best?"],"prefix":"10.3390","volume":"13","author":[{"given":"Susanne","family":"Wiegand","sequence":"first","affiliation":[{"name":"Department of Otolaryngology, Head and Neck Surgery, University Hospital Leipzig, 04103 Leipzig, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Dietz","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology, Head and Neck Surgery, University Hospital Leipzig, 04103 Leipzig, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6191-2095","authenticated-orcid":false,"given":"Gunnar","family":"Wichmann","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology, Head and Neck Surgery, University Hospital Leipzig, 04103 Leipzig, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,12,9]]},"reference":[{"unstructured":"(2021, December 07). ISSVA Classification 2018. Available online: http:\/\/www.issva.org\/UserFiles\/file\/ISSVA-Classification-2018.pdf.","key":"ref_1"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/j.jvs.2016.03.329","article-title":"Malignant Vascular Tumors: A Nationwide Analysis","volume":"63","author":"Alarhayem","year":"2016","journal-title":"J. Vasc. Surg."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1007\/s00784-003-0233-4","article-title":"Sarcomas of the oral and maxillofacial region: A review of 32 cases in 25 years","volume":"8","author":"Yamaguchi","year":"2004","journal-title":"Clin. Oral Investig."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"S30","DOI":"10.1038\/modpathol.2013.176","article-title":"Malignant vascular tumors\u2014An update","volume":"27","author":"Antonescu","year":"2014","journal-title":"MOD. Pathol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1016\/S1470-2045(10)70023-1","article-title":"Angiosarcoma","volume":"11","author":"Young","year":"2010","journal-title":"Lancet Oncol."},{"unstructured":"Weiss, S.W., and Goldblum, J.R. (2008). Enzinger & Weiss\u2019s Soft Tissue Tumors, Elsevier Health Sciences, Mosby. [5th ed.].","key":"ref_6"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1953","DOI":"10.1245\/s10434-006-9335-y","article-title":"Treatment and Outcome of 82 Patients with Angiosarcoma","volume":"14","author":"Abraham","year":"2007","journal-title":"Ann. Surg. Oncol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1002\/(SICI)1096-9098(199603)61:3<170::AID-JSO2>3.0.CO;2-8","article-title":"Prognostic factors in angiosarcoma: A multivariate analysis of 55 cases","volume":"61","author":"Naka","year":"1996","journal-title":"J. Surg. Oncol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2030","DOI":"10.1093\/annonc\/mdm381","article-title":"Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: A retrospective study of 161 cases","volume":"18","author":"Fayette","year":"2007","journal-title":"Ann. Oncol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1604","DOI":"10.6004\/jnccn.2020.0058","article-title":"NCCN Guidelines Insights: Soft Tissue Sarcoma, Version 1.2021","volume":"18","author":"Kane","year":"2020","journal-title":"J. Natl. Compr. Cancer Netw."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1923","DOI":"10.1148\/rg.317115095","article-title":"Soft Tissue Tumors of the Head and Neck: Imaging-based Review of the WHO Classification","volume":"31","author":"Razek","year":"2011","journal-title":"Radiographics"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1007\/s00259-016-3456-8","article-title":"Pretreatment tumor SUVmax predicts disease-specific and overall survival in patients with head and neck soft tissue sarcoma","volume":"44","author":"Ha","year":"2017","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"649","DOI":"10.2214\/AJR.19.21635","article-title":"Prognostic Value of Quantitative Parameters of 18F-FDG PET\/CT for Patients With Angiosarcoma","volume":"214","author":"Kato","year":"2020","journal-title":"Am. J. Roentgenol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"5878","DOI":"10.1002\/cncr.27614","article-title":"Alterations of the p53 and PIK3CA\/AKT\/mTOR pathways in angiosarcomas","volume":"118","author":"Italiano","year":"2012","journal-title":"Cancer"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1716","DOI":"10.1002\/cncr.11667","article-title":"Cutaneous angiosarcoma of the scalp","volume":"98","author":"Pawlik","year":"2003","journal-title":"Cancer"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"2433","DOI":"10.1016\/j.ejca.2008.07.037","article-title":"Paclitaxel in patients with advanced angiosarcomas of soft tissue: A retrospective study of the EORTC soft tissue and bone sarcoma group","volume":"44","author":"Schlemmer","year":"2008","journal-title":"Eur. J. Cancer"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1002\/hed.21513","article-title":"Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp","volume":"33","author":"Guadagnolo","year":"2010","journal-title":"Head Neck"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"e1127","DOI":"10.1259\/bjr\/31655219","article-title":"Treatment and prognosis of angiosarcoma of the scalp and face: A retrospective analysis of 48 patients","volume":"85","author":"Ogawa","year":"2012","journal-title":"Br. J. Radiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"2400","DOI":"10.1002\/(SICI)1097-0142(19960601)77:11<2400::AID-CNCR32>3.0.CO;2-Z","article-title":"Angiosarcoma. A report of 67 patients and a review of the literature","volume":"77","author":"Mark","year":"1996","journal-title":"Cancer"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1097\/COC.0b013e3182436ea3","article-title":"Radiation Therapy for Angiosarcoma","volume":"36","author":"Scott","year":"2013","journal-title":"Am. J. Clin. Oncol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"627113","DOI":"10.3389\/fonc.2021.627113","article-title":"The Effectiveness of Different Treatment Modalities of Cutaneous Angiosarcoma: Results From Meta-Analysis and Observational Data From SEER Database","volume":"11","author":"Bi","year":"2021","journal-title":"Front. Oncol."},{"key":"ref_22","first-page":"1247","article-title":"Radiation Therapy for Angiosarcoma of the Scalp: Total Scalp Irradiation and Local Irradiation","volume":"38","author":"Hata","year":"2018","journal-title":"Anticancer Res."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"921","DOI":"10.21873\/invivo.11014","article-title":"Definitive Radiation Therapy for Angiosarcoma of the Face and Scalp","volume":"30","author":"Suzuki","year":"2016","journal-title":"Vivo"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"2034","DOI":"10.1002\/(SICI)1097-0142(19991115)86:10<2034::AID-CNCR21>3.0.CO;2-P","article-title":"Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face","volume":"86","author":"Fata","year":"1999","journal-title":"Cancer"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1007\/s11912-002-0066-3","article-title":"Management of angiosarcoma","volume":"4","author":"Budd","year":"2002","journal-title":"Curr. Oncol. Rep."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1002\/cncr.22822","article-title":"Docetaxel: A therapeutic option in the treatment of cutaneous angiosarcoma","volume":"110","author":"Nagano","year":"2007","journal-title":"Cancer"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1097\/00130404-200505000-00011","article-title":"A 14-Year Retrospective Review of Angiosarcoma","volume":"11","author":"Fury","year":"2005","journal-title":"Cancer J."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"10532","DOI":"10.1200\/jco.2008.26.15_suppl.10532","article-title":"Phase II intergroup study of sorafenib (S) in advanced soft tissue sarcomas (STS): SWOG 0505","volume":"26","author":"Ryan","year":"2008","journal-title":"J. Clin. Oncol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"3154","DOI":"10.1200\/JCO.2008.20.9890","article-title":"Multicenter Phase II Trial of Sunitinib in the Treatment of Nongastrointestinal Stromal Tumor Sarcomas","volume":"27","author":"George","year":"2009","journal-title":"J. Clin. Oncol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"3133","DOI":"10.1200\/JCO.2008.20.4495","article-title":"Phase II Study of Sorafenib in Patients With Metastatic or Recurrent Sarcomas","volume":"27","author":"Maki","year":"2009","journal-title":"J. Clin. Oncol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1093\/annonc\/mdr138","article-title":"Metastatic angiosarcomas: Doxorubicin-based regimens, weekly paclitaxel and metastasectomy significantly improve the outcome","volume":"23","author":"Penel","year":"2012","journal-title":"Ann. Oncol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1093\/annonc\/mdr066","article-title":"Gemcitabine in advanced angiosarcoma: A retrospective case series analysis from the Italian Rare Cancer Network","volume":"23","author":"Stacchiotti","year":"2012","journal-title":"Ann. Oncol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1634\/theoncologist.2011-0237","article-title":"Sorafenib for Patients with Advanced Angiosarcoma: A Phase II Trial from the French Sarcoma Group (GSF\/GETO)","volume":"17","author":"Italiano","year":"2012","journal-title":"Oncologist"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"3330","DOI":"10.1002\/cncr.26599","article-title":"Comparison of doxorubicin and weekly paclitaxel efficacy in metastatic angiosarcomas","volume":"118","author":"Italiano","year":"2012","journal-title":"Cancer"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1093\/annonc\/mds237","article-title":"An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas","volume":"24","author":"Agulnik","year":"2013","journal-title":"Ann. Oncol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"3178","DOI":"10.1016\/j.ejca.2014.10.004","article-title":"First-line anthracycline-based chemotherapy for angiosarcoma and other soft tissue sarcoma subtypes: Pooled analysis of eleven European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group trials","volume":"50","author":"Young","year":"2014","journal-title":"Eur. J. Cancer"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1159\/000381917","article-title":"Outcomes of Systemic Therapy for Patients with Metastatic Angiosarcoma","volume":"89","author":"Munhoz","year":"2015","journal-title":"Oncology"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"2797","DOI":"10.1200\/JCO.2015.60.8505","article-title":"Paclitaxel Given Once Per Week With or Without Bevacizumab in Patients With Advanced Angiosarcoma: A Randomized Phase II Trial","volume":"33","author":"Domont","year":"2015","journal-title":"J. Clin. Oncol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1080\/0284186X.2016.1234068","article-title":"Pazopanib in advanced vascular sarcomas: An EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis","volume":"56","author":"Jones","year":"2017","journal-title":"Acta Oncol."},{"doi-asserted-by":"crossref","unstructured":"Lebellec, L., Bertucci, F., Tresch-Bruneel, E., Ray-Coquard, I., Le Cesne, A., Bompas, E., Blay, J.-Y., Italiano, A., Mir, O., and Ryckewaert, T. (2018). Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: An ancillary study derived from a randomized clinical trial. BMC Cancer, 18.","key":"ref_40","DOI":"10.1186\/s12885-018-4828-1"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/j.ejca.2021.06.027","article-title":"An open-label single-arm phase II study of regorafenib for the treatment of angiosarcoma","volume":"154","author":"Agulnik","year":"2021","journal-title":"Eur. J. Cancer"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"e002990","DOI":"10.1136\/jitc-2021-002990","article-title":"Multicenter phase II trial (SWOG S1609, cohort 51) of ipilimumab and nivolumab in metastatic or unresectable angiosarcoma: A substudy of dual anti-CTLA-4 and anti-PD-1 blockade in rare tumors (DART)","volume":"9","author":"Wagner","year":"2021","journal-title":"J. Immunother. Cancer"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1007\/s10637-006-9035-2","article-title":"Phase I\/II study of docetaxel, ifosfamide, and doxorubicin in advanced, recurrent, or metastatic soft tissue sarcoma (STS)","volume":"24","author":"Suppiah","year":"2006","journal-title":"Investig. New Drugs"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1879","DOI":"10.1016\/S0140-6736(12)60651-5","article-title":"Pazopanib for metastatic soft-tissue sarcoma (PALETTE): A randomised, double-blind, placebo-controlled phase 3 trial","volume":"379","author":"Blay","year":"2012","journal-title":"Lancet"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1186\/s12885-015-1160-x","article-title":"Efficacy of pazopanib monotherapy in patients who had been heavily pretreated for metastatic soft tissue sarcoma: A retrospective case series","volume":"15","author":"Yoo","year":"2015","journal-title":"BMC Cancer"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1007\/s12032-016-0831-z","article-title":"Pazopanib treatment slows progression and stabilizes disease in patients with taxane-resistant cutaneous angiosarcoma","volume":"33","author":"Ogata","year":"2016","journal-title":"Med. Oncol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.ebiom.2016.02.026","article-title":"Effective Management of Advanced Angiosarcoma by the Synergistic Combination of Propranolol and Vinblastine-based Metronomic Chemotherapy: A Bench to Bedside Study","volume":"6","author":"Pasquier","year":"2016","journal-title":"EBioMedicine"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"360","DOI":"10.1200\/JGO.2016.005843","article-title":"Positive PD-L1 Expression Predicts Worse Outcome in Cutaneous Angiosarcoma","volume":"3","author":"Shimizu","year":"2017","journal-title":"J. Glob. Oncol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1001\/jamaoncol.2017.1617","article-title":"Use of PD-1 Targeting, Macrophage Infiltration, and IDO Pathway Activation in Sarcomas","volume":"4","author":"Toulmonde","year":"2018","journal-title":"JAMA Oncol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1177\/000313480807400115","article-title":"Hepatic Malignant Epithelioid Hemangioendothelioma: A Case Report and Review of the Literature","volume":"74","author":"Woodall","year":"2008","journal-title":"Am. Surg."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"970","DOI":"10.1002\/1097-0142(19820901)50:5<970::AID-CNCR2820500527>3.0.CO;2-Z","article-title":"Epithelioid hemangioendothelioma a vascular tumor often mistaken for a carcinoma","volume":"50","author":"Weiss","year":"1982","journal-title":"Cancer"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/0030-4220(86)90204-5","article-title":"Epithelioid hemangioendothelioma of the head and neck: A clinicopathologic report of twelve cases","volume":"61","author":"Ellis","year":"1986","journal-title":"Oral Surg. Oral Med. Oral Pathol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1002\/1097-0142(19940201)73:3<610::AID-CNCR2820730318>3.0.CO;2-3","article-title":"Epithelioid hemangioendothelioma with multiple site involvement. Literature review and observations","volume":"73","author":"Bollinger","year":"1994","journal-title":"Cancer"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1111\/j.1600-051X.1986.tb01408.x","article-title":"Hemangioendothelioma of the gingiva. Histopathologic and therapeutic considerations","volume":"13","author":"Ebo","year":"1986","journal-title":"J. Clin. Periodontol."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1299","DOI":"10.1288\/00005537-198711000-00009","article-title":"Epithelioid hemangioendothelioma (histiocytoid hemangioma) of the palate","volume":"97","author":"Moran","year":"1987","journal-title":"Laryngoscope"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1016\/0030-4220(87)90261-1","article-title":"Epithelioid hemangioendothelioma of the gingiva: Case report and ultrastructural study","volume":"63","author":"Marcucci","year":"1987","journal-title":"Oral Surg. Oral Med. Oral Pathol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"1480","DOI":"10.1002\/hep.24617","article-title":"Unusual extrahepatic metastasis to the soft tissue of the left cervical neck area from hepatic epithelioid hemangioendothelioma","volume":"54","author":"Kim","year":"2011","journal-title":"Hepatology"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1177\/030089169908500618","article-title":"Epithelioid Hemangioendothelioma of the Larynx","volume":"85","author":"Boscaino","year":"1999","journal-title":"Tumori J."},{"unstructured":"Fletcher, C.D.M. (1997). International Academy of P (2013) WHO classification of tumours of soft tissue and bone. World Health Or-ganization Classification of Tumours, IARC Press. [4th ed.].","key":"ref_59"},{"key":"ref_60","first-page":"259","article-title":"Epithelioid hemangioendothelioma: An overview and update on a rare vascular tumor","volume":"8","author":"Sardaro","year":"2014","journal-title":"Oncol. Rev."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1312","DOI":"10.1378\/chest.11-0039","article-title":"Clinical Patterns and Outcome in Epithelioid Hemangioendothelioma With or Without Pulmonary Involvement","volume":"140","author":"Lau","year":"2011","journal-title":"Chest"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1002\/gcc.20886","article-title":"A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites","volume":"50","author":"Errani","year":"2011","journal-title":"Genes Chromosom. Cancer"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"e1912416","DOI":"10.1001\/jamanetworkopen.2019.12416","article-title":"Common Secondary Genomic Variants Associated With Advanced Epithelioid Hemangioendothelioma","volume":"2","author":"Seligson","year":"2019","journal-title":"JAMA Netw. Open"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1002\/gcc.22073","article-title":"NovelYAP1-TFE3fusion defines a distinct subset of epithelioid hemangioendothelioma","volume":"52","author":"Antonescu","year":"2013","journal-title":"Genes Chromosom. Cancer"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"4006","DOI":"10.1128\/JCM.05527-11","article-title":"Bartonella Species Bacteremia in Two Patients with Epithelioid Hemangioendothelioma","volume":"49","author":"Mascarelli","year":"2011","journal-title":"J. Clin. Microbiol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s13244-019-0818-3","article-title":"Vascular lesions of the head and neck: An update on classification and imaging review","volume":"11","author":"Brahmbhatt","year":"2020","journal-title":"Insights Imaging"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"2108","DOI":"10.1002\/cncr.22225","article-title":"Primary malignant hepatic epithelioid hemangioendothelioma","volume":"107","author":"Mehrabi","year":"2006","journal-title":"Cancer"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"924","DOI":"10.1097\/PAS.0b013e31815bf8e6","article-title":"Epithelioid Hemangioendothelioma of Soft Tissue: A Proposal for Risk Stratification Based on 49 Cases","volume":"32","author":"Deyrup","year":"2008","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"100170","DOI":"10.1016\/j.esmoop.2021.100170","article-title":"Epithelioid hemangioendothelioma, an ultra-rare cancer: A consensus paper from the community of experts","volume":"6","author":"Stacchiotti","year":"2021","journal-title":"ESMO Open"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1007\/s10238-020-00653-0","article-title":"Imaging characteristics and prognostic values of hepatic epithelioid hemangioendothelioma on 18F-FDG PET\/CT","volume":"20","author":"Wang","year":"2020","journal-title":"Clin. Exp. Med."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"e66","DOI":"10.1097\/RLU.0b013e318266ceca","article-title":"MRI and FDG PET\/CT Findings of Hepatic Epithelioid Hemangioendothelioma","volume":"38","author":"Dong","year":"2013","journal-title":"Clin. Nucl. Med."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1046\/j.1365-2559.1998.00384.x","article-title":"Epithelioid haemangioendothelioma of the thyroid gland: A case report and review of literature","volume":"32","author":"Siddiqui","year":"1998","journal-title":"Histopathology"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1377","DOI":"10.1089\/thy.2005.15.1377","article-title":"An Authentic Malignant Epithelioid Hemangioendothelioma of the Thyroid: A Case Report and Review of the Literature","volume":"15","author":"Hassan","year":"2005","journal-title":"Thyroid"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1007\/s12105-007-0035-0","article-title":"Epithelioid Hemangioendothelioma of the Head and Neck: Role of Podoplanin in the Differential Diagnosis","volume":"2","author":"Naqvi","year":"2007","journal-title":"Head Neck Pathol."},{"key":"ref_75","first-page":"308","article-title":"Epithelioid haemangioendothelioma of the anterior skull base: What is the optimal treatment?","volume":"15","author":"Wong","year":"2009","journal-title":"Hong Kong Med. J."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"111","DOI":"10.4103\/0974-2727.72214","article-title":"Epithelioid Hemangioendothelioma of Nasal Cavity","volume":"2","author":"Patnayak","year":"2010","journal-title":"J. Lab. Physicians"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"142","DOI":"10.4103\/0974-620X.91272","article-title":"Hemangioendothelioma of the eyelid can mimic chalazion","volume":"4","author":"Raddaoui","year":"2011","journal-title":"Oman J. Ophthalmol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1111\/j.1440-1789.2010.01180.x","article-title":"Primary epithelioid hemangioendothelioma in the clival region: A case report and literature review","volume":"31","author":"Ma","year":"2010","journal-title":"Neuropathology"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"120","DOI":"10.47210\/bjohns.2015.v23i3.59","article-title":"A Rare Case of A Giant Hemangioendothelioma of Neck","volume":"23","author":"Banerjee","year":"2015","journal-title":"Bengal J. Otolaryngol. Head Neck Surg."},{"key":"ref_80","first-page":"1","article-title":"Epithelioid Hemangioendothelioma of the Mastoid: Resection for Recurrence and Adjuvant Radiation with 8-Year Followup","volume":"2013","author":"Drazin","year":"2013","journal-title":"Case Rep. Surg."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1016\/j.ijscr.2015.06.041","article-title":"Epithelioid Haemangioendothelioma of the mandibular gingiva: Case report and literature review","volume":"14","author":"Ali","year":"2015","journal-title":"Int. J. Surg. Case Rep."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1007\/s12022-016-9428-5","article-title":"Epithelioid Hemangioendothelioma: A Rare Primary Thyroid Tumor with Confirmation of WWTR1 and CAMTA1 Rearrangements","volume":"27","author":"Shah","year":"2016","journal-title":"Endocr. Pathol."},{"key":"ref_83","first-page":"155","article-title":"Epithelioid hemangioendothelioma of the nasal septum","volume":"12","author":"Hanege","year":"2016","journal-title":"B ENT"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"109","DOI":"10.4103\/0975-962X.180314","article-title":"Epithelioid hemangioendothelioma of hypopharynx: A rare presentation","volume":"7","author":"Jain","year":"2016","journal-title":"Indian J. Dent."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"53","DOI":"10.4103\/2152-7806.181902","article-title":"Nasal cavity epithelioid hemangioendothelioma invading the anterior skull base","volume":"7","author":"Endo","year":"2016","journal-title":"Surg. Neurol. Int."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"137","DOI":"10.4103\/0973-029X.180975","article-title":"Epithelioid hemangioendothelioma of the mandibular gingiva: A rare case of metastasis 4 years after radical excision and literature review","volume":"20","author":"Salgarelli","year":"2016","journal-title":"J. Oral Maxillofac. Pathol. JOMFP"},{"key":"ref_87","first-page":"1","article-title":"A Case of an Epithelioid Hemangioendothelioma Arising from the Innominate Vein Mimicking Cervical Metastatic Lymphadenopathy","volume":"2016","author":"Brill","year":"2016","journal-title":"Case Rep. Surg."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"e638","DOI":"10.1097\/SCS.0000000000003749","article-title":"An Epithelioid Hemangioendothelioma of the Head and Neck","volume":"28","author":"Duzer","year":"2017","journal-title":"J. Craniofac. Surg."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1007\/s13691-018-0351-0","article-title":"Epithelioid hemangioendothelioma of the parotid gland: A case report","volume":"8","author":"Koide","year":"2019","journal-title":"Int. Cancer Conf. J."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/j.jormas.2017.10.001","article-title":"Epithelioid hemangioendothelioma, an uncommon tumor of the eyelid: A case report","volume":"119","author":"Ennouhi","year":"2018","journal-title":"J. Stomatol. Oral Maxillofac. Surg."},{"key":"ref_91","first-page":"50","article-title":"Successful Total Resection of an Orbital Epithelioid Hemangioendothelioma with the Aid of Endovascular Embolization","volume":"5","author":"Eshraghi","year":"2018","journal-title":"Ocul. Oncol. Pathol."},{"key":"ref_92","first-page":"260","article-title":"Epithelioid hemangioendothelioma of the parotid gland: A case report in an unusual location with a review of the literature","volume":"52","year":"2019","journal-title":"Rev. Esp. Patol."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12957-020-01999-1","article-title":"Primary epithelioid angiosarcoma originating from the mandibular gingiva: A case report of an extremely rare oral lesion","volume":"18","author":"Komatsu","year":"2020","journal-title":"World J. Surg. Oncol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"S433","DOI":"10.1177\/0145561319873914","article-title":"Epithelioid Hemangioendothelioma Presenting as Unilateral Vocal Fold Paralysis: A Case Report and Literature Review","volume":"100","author":"Lui","year":"2021","journal-title":"Ear Nose Throat J."},{"doi-asserted-by":"crossref","unstructured":"Cirkin, D.S., Secinti, I.E., Dogan, E., and Ozler, G.S. (2021). Epithelioid hemangioendothelioma in the tongue: A rare case report. Turk. J. Pathol., 1.","key":"ref_95","DOI":"10.5146\/tjpath.2021.01560"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"103061","DOI":"10.1016\/j.amjoto.2021.103061","article-title":"3D endoscopic endonasal craniectomy for intenstinal type adeno-carcinoma (ITAC) of the nasal cavity","volume":"42","author":"Mevio","year":"2021","journal-title":"Am. J. Otolaryngol."},{"doi-asserted-by":"crossref","unstructured":"Meccariello, G., Maniaci, A., Bianchi, G., Cammaroto, G., Iannella, G., Catalano, A., Sgarzani, R., de Vito, A., Capaccio, P., and Pelucchi, S. (2021). Neck dissection and trans oral robotic surgery for oropharyngeal squamous cell carcinoma. Auris Nasus Larynx, 3.","key":"ref_97","DOI":"10.1016\/j.anl.2021.05.007"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"104770","DOI":"10.1016\/j.oraloncology.2020.104770","article-title":"Transoral robotic surgery and neck dissection for HPV-positive oropharyngeal carcinoma: Importance of nodal count in survival","volume":"109","author":"Viet","year":"2020","journal-title":"Oral Oncol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"841","DOI":"10.1097\/00007632-199504000-00020","article-title":"Seven-Year Follow-up of Vertebral Excision and Reconstruction for Malignant Hemangioendothelioma of Bone","volume":"20","author":"Sybert","year":"1995","journal-title":"Spine"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1183\/09031936.98.12010089","article-title":"Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression","volume":"12","author":"Kitaichi","year":"1998","journal-title":"Eur. Respir. J."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"2645","DOI":"10.1002\/cam4.3807","article-title":"Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature","volume":"10","author":"Frezza","year":"2021","journal-title":"Cancer Med."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1002\/cncr.33247","article-title":"Activity of sirolimus in patients with progressive epithelioid hemangioendothelioma: A case-series analysis within the Italian Rare Cancer Network","volume":"127","author":"Stacchiotti","year":"2021","journal-title":"Cancer"},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"e8507","DOI":"10.1097\/MD.0000000000008507","article-title":"Apatinib for the treatment of pulmonary epithelioid hemangioendothelioma","volume":"96","author":"Zheng","year":"2017","journal-title":"Medicine"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1159\/000443986","article-title":"Sorafenib Monotherapy in a Patient with Unresectable Hepatic Epithelioid Hemangioendothelioma","volume":"9","author":"Kobayashi","year":"2016","journal-title":"Case Rep. Oncol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1186\/s12885-015-1395-6","article-title":"Pazopanib for metastatic pulmonary epithelioid hemangioendothelioma\u2014A suitable treatment option: Case report and review of anti-angiogenic treatment options","volume":"15","author":"Semenisty","year":"2015","journal-title":"BMC Cancer"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1159\/000082932","article-title":"Thalidomide Inhibits the Growth and Progression of Hepatic Epithelioid Hemangioendothelioma","volume":"67","author":"Mascarenhas","year":"2004","journal-title":"Oncology"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1097\/MPH.0b013e31816e22d1","article-title":"Metastatic Hepatic Epithelioid Hemangioendothelioma in a Teenage Girl","volume":"30","author":"Kassam","year":"2008","journal-title":"J. Pediatr. Hematol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1007\/s00268-008-9882-4","article-title":"Clinical Experience with Infantile Hepatic Hemangioendothelioma","volume":"33","author":"Moon","year":"2009","journal-title":"World J. Surg."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"281","DOI":"10.5603\/ARM.27887","article-title":"Pulmonary epithelioid haeman-gioendothelioma--interferon 2-alpha treatment\u2014Case report","volume":"76","author":"Radzikowska","year":"2008","journal-title":"Adv. Respir. Med."}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/13\/24\/6201\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T07:44:01Z","timestamp":1760168641000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/13\/24\/6201"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12,9]]},"references-count":109,"journal-issue":{"issue":"24","published-online":{"date-parts":[[2021,12]]}},"alternative-id":["cancers13246201"],"URL":"https:\/\/doi.org\/10.3390\/cancers13246201","relation":{},"ISSN":["2072-6694"],"issn-type":[{"type":"electronic","value":"2072-6694"}],"subject":[],"published":{"date-parts":[[2021,12,9]]}}}