{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T02:16:30Z","timestamp":1789697790660,"version":"4.0.1"},"reference-count":66,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2017,6,9]],"date-time":"2017-06-09T00:00:00Z","timestamp":1496966400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["FKZ 01ZX1409B"],"award-info":[{"award-number":["FKZ 01ZX1409B"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["SFB 850"],"award-info":[{"award-number":["SFB 850"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006058","name":"St. Baldrick's Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006058","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2017,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Epithelioid hemangioendothelioma (EHE) is a rare, vascular sarcoma. Visceral forms arise in the liver\/ lungs. We review the clinical and molecular phenotype of pediatric visceral EHE based on the case of a 9\u2010year\u2010old male child with EHE of the liver\/lungs. His tumor expressed the EHE\u2010specific fusion oncogene <jats:italic>WWTR1\u2010CAMTA1<\/jats:italic>. Molecular characterization revealed a low somatic mutation rate and activated interferon signaling, angiogenesis regulation, and blood vessel remodeling. After polychemotherapy and resection of lung tumors, residual disease remained stable on oral lenalidomide. Literature review identified another 24 children with EHE of the liver\/lungs. Most presented with multifocal, systemic disease. Only those who underwent complete resection achieved complete remission. Four children experienced rapid progression and died. In six children, disease remained stable for years without therapy. Two patients died from progressive EHE 21 and 24 years after first diagnosis. Natural evolution of pediatric visceral EHE is variable, and long\u2010term prognosis remains unclear.<\/jats:p>","DOI":"10.1002\/pbc.26675","type":"journal-article","created":{"date-parts":[[2017,6,9]],"date-time":"2017-06-09T14:16:53Z","timestamp":1497017813000},"source":"Crossref","is-referenced-by-count":42,"title":["Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1709-4448","authenticated-orcid":false,"given":"Simone","family":"Hettmer","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine Division of Pediatric Hematology and Oncology, Medical Center, Faculty of Medicine, University of Freiburg  Freiburg Germany"},{"name":"German Cancer Consortium (DKTK) Partner site Freiburg  Freiburg Germany"},{"name":"Comprehensive Cancer Center Freiburg  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geoffroy","family":"Andrieux","sequence":"additional","affiliation":[{"name":"Institute of Molecular Medicine and Cell Research University of Freiburg  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen","family":"Hochrein","sequence":"additional","affiliation":[{"name":"Institute of Molecular Medicine and Cell Research University of Freiburg  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philipp","family":"Kurz","sequence":"additional","affiliation":[{"name":"Comprehensive Cancer Center Freiburg  Freiburg Germany"},{"name":"Institute of Surgical Pathology Medical Center and Faculty of Medicine, University of Freiburg  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen","family":"R\u00f6ssler","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine Division of Pediatric Hematology and Oncology, Medical Center, Faculty of Medicine, University of Freiburg  Freiburg Germany"},{"name":"Comprehensive Cancer Center Freiburg  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silke","family":"Lassmann","sequence":"additional","affiliation":[{"name":"German Cancer Consortium (DKTK) Partner site Freiburg  Freiburg Germany"},{"name":"Comprehensive Cancer Center Freiburg  Freiburg Germany"},{"name":"Institute of Surgical Pathology Medical Center and Faculty of Medicine, University of Freiburg  Freiburg Germany"},{"name":"German Cancer Research Center (DKFZ)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Werner","sequence":"additional","affiliation":[{"name":"German Cancer Consortium (DKTK) Partner site Freiburg  Freiburg Germany"},{"name":"Comprehensive Cancer Center Freiburg  Freiburg Germany"},{"name":"Institute of Surgical Pathology Medical Center and Faculty of Medicine, University of Freiburg  Freiburg Germany"},{"name":"German Cancer Research Center (DKFZ)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikolas","family":"von Bubnoff","sequence":"additional","affiliation":[{"name":"German Cancer Consortium (DKTK) Partner site Freiburg  Freiburg Germany"},{"name":"Comprehensive Cancer Center Freiburg  Freiburg Germany"},{"name":"Department of Hematology, Oncology and Stem Cell Transplantation Medical Center, University of Freiburg  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Peters","sequence":"additional","affiliation":[{"name":"German Cancer Consortium (DKTK) Partner site Freiburg  Freiburg Germany"},{"name":"Comprehensive Cancer Center Freiburg  Freiburg Germany"},{"name":"Institute of Molecular Medicine and Cell Research University of Freiburg  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ewa","family":"Koscielniak","sequence":"additional","affiliation":[{"name":"Klinikum Stuttgart, Zentrum f\u00fcr Kinder und Jugendmedizin Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology, Immunology)  Stuttgart Germany"},{"name":"Department of Pediatric Oncology University Children's Hospital  Tuebingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9551-2399","authenticated-orcid":false,"given":"Monika","family":"Sparber\u2010Sauer","sequence":"additional","affiliation":[{"name":"Klinikum Stuttgart, Zentrum f\u00fcr Kinder und Jugendmedizin Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology, Immunology)  Stuttgart Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte","family":"Niemeyer","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine Division of Pediatric Hematology and Oncology, Medical Center, Faculty of Medicine, University of Freiburg  Freiburg Germany"},{"name":"German Cancer Consortium (DKTK) Partner site Freiburg  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Mentzel","sequence":"additional","affiliation":[{"name":"Dermapathologische Gemeinschaftspraxis  Friedrichshafen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hauke","family":"Busch","sequence":"additional","affiliation":[{"name":"German Cancer Consortium (DKTK) Partner site Freiburg  Freiburg Germany"},{"name":"Comprehensive Cancer Center Freiburg  Freiburg Germany"},{"name":"Institute of Molecular Medicine and Cell Research University of Freiburg  Freiburg Germany"},{"name":"German Cancer Research Center (DKFZ)  Heidelberg Germany"},{"name":"L\u00fcbeck Institute of Experimental Dermatology  L\u00fcbeck Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie","family":"Boerries","sequence":"additional","affiliation":[{"name":"German Cancer Consortium (DKTK) Partner site Freiburg  Freiburg Germany"},{"name":"Comprehensive Cancer Center Freiburg  Freiburg Germany"},{"name":"Institute of Molecular Medicine and Cell Research University of Freiburg  Freiburg Germany"},{"name":"German Cancer Research Center (DKFZ)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,6,9]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19820901)50:5<970::AID-CNCR2820500527>3.0.CO;2-Z"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.22225"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0046-8177(84)80145-8"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0142(19961201)78:11<2318::AID-CNCR8>3.0.CO;2-I"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0142(19990201)85:3<562::AID-CNCR7>3.0.CO;2-T"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.98.12010089"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2006.06.068"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31815bf8e6"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.11-0039"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22073"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1186\/1746-1596-9-131"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/onc.2015.148"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.3002409"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2014.05.029"},{"issue":"1","key":"e_1_2_6_16_1","first-page":"473","article-title":"Systemic treatment options for epithelioid haemangioendothelioma: the Royal Marsden Hospital experience","volume":"35","author":"Yousaf N","year":"2015","journal-title":"Anticancer Res"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bjc.6601720"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002920050369"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e31816e22d1"},{"issue":"12","key":"e_1_2_6_20_1","first-page":"1001","article-title":"A case with pulmonary epithelioid hemangioendothelioma found on school medical check","volume":"42","author":"Nakano Y","year":"2004","journal-title":"Nihon Kokyuki Gakkai Zasshi"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-015-3852-3"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-008-0915-9"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2559.1989.tb03073.x"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.03.00101602"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19871115)60:10<2471::AID-CNCR2820601021>3.0.CO;2-8"},{"issue":"9","key":"e_1_2_6_26_1","doi-asserted-by":"crossref","first-page":"1616","DOI":"10.1183\/09031936.95.08091616","article-title":"Epithelioid haemangioendothelioma of the lung: clinical and pathological pitfalls","volume":"8","author":"Kasteren ME","year":"1995","journal-title":"Eur Respir J"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btq635"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1038\/nmeth.2651"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gku1003"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2105-14-7"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/bts635"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1186\/gb-2009-10-3-r25"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btp616"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btu049"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2164-14-293"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2013.11.002"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0b013e31815c2a70"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2009.01266.x"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2013.01.043"},{"key":"e_1_2_6_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.28109"},{"key":"e_1_2_6_41_1","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mds237"},{"issue":"11","key":"e_1_2_6_42_1","first-page":"462","article-title":"Multifocal epithelioid hemangioendothelioma: case report of a clinical chamaeleon","volume":"11","author":"Bolke E","year":"2006","journal-title":"Eur J Med Res"},{"key":"e_1_2_6_43_1","doi-asserted-by":"publisher","DOI":"10.1159\/000082932"},{"key":"e_1_2_6_44_1","doi-asserted-by":"publisher","DOI":"10.1186\/1752-1947-4-413"},{"issue":"1","key":"e_1_2_6_45_1","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/S1665-2681(19)31596-0","article-title":"Thalidomide for the treatment of metastatic hepatic epithelioid hemangioendothelioma: a case report with a long term follow\u2010up","volume":"10","author":"Salech F","year":"2011","journal-title":"Ann Hepatol"},{"key":"e_1_2_6_46_1","article-title":"Treatment of hepatic epithelioid hemangioendothelioma: finding uses for thalidomide in a new era of medicine","volume":"2015","author":"Soape MP","year":"2015","journal-title":"Case Rep Gastrointest Med"},{"key":"e_1_2_6_47_1","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v20.i22.7049"},{"key":"e_1_2_6_48_1","article-title":"Activity of lenalidomide in metastatic hepatic epithelioid hemangioendothelioma (HEH): a case report","volume":"27","author":"S","year":"2009","journal-title":"J Clin Oncol (Meeting Abstracts)"},{"key":"e_1_2_6_49_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11060-009-0017-z"},{"key":"e_1_2_6_50_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2004.018937"},{"issue":"1","key":"e_1_2_6_51_1","first-page":"145","article-title":"Primary hepatic epithelioid hemangioendothelioma progressively responsive to interferon\u2010alpha: is there room for novel anti\u2010angiogenetic treatments?","volume":"26","author":"Calabro L","year":"2007","journal-title":"J Exp Clin Cancer Res"},{"key":"e_1_2_6_52_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007890-200207150-00022"},{"key":"e_1_2_6_53_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1075-122X.2005.21663.x"},{"issue":"4","key":"e_1_2_6_54_1","first-page":"281","article-title":"Pulmonary epithelioid haemangioendothelioma\u2013interferon 2\u2010alpha treatment\u2014case report","volume":"76","author":"Radzikowska E","year":"2008","journal-title":"Pneumonol Alergol Pol"},{"key":"e_1_2_6_55_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0873-2159(15)30249-X"},{"key":"e_1_2_6_56_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13569-014-0018-3"},{"key":"e_1_2_6_57_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12885-015-1395-6"},{"key":"e_1_2_6_58_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002470050661"},{"key":"e_1_2_6_59_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-009-1532-y"},{"key":"e_1_2_6_60_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12098-010-0083-4"},{"key":"e_1_2_6_61_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.1950110217"},{"key":"e_1_2_6_62_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e3181d413b2"},{"issue":"12","key":"e_1_2_6_63_1","first-page":"FD01","article-title":"Hepatic epithelioid hemangioendothelioma in an eight year old \u2014a case report","volume":"8","author":"Vasudevan G","year":"2014","journal-title":"J Clin Diagn Res"},{"key":"e_1_2_6_64_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1997.02170400103020"},{"key":"e_1_2_6_65_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.rti.0000158403.40711.e8"},{"key":"e_1_2_6_66_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-005-1492-9"},{"key":"e_1_2_6_67_1","doi-asserted-by":"publisher","DOI":"10.1136\/mp.55.1.61"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.26675","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.26675","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T08:55:04Z","timestamp":1695977704000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.26675"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,6,9]]},"references-count":66,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2017,12]]}},"alternative-id":["10.1002\/pbc.26675"],"URL":"https:\/\/doi.org\/10.1002\/pbc.26675","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,6,9]]},"article-number":"e26675"}}