{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T18:17:55Z","timestamp":1788459475966,"version":"build-2803163510"},"reference-count":22,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,12,13]],"date-time":"2025-12-13T00:00:00Z","timestamp":1765584000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,12,13]],"date-time":"2025-12-13T00:00:00Z","timestamp":1765584000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100010109","name":"Medical University of Graz","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100010109","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Eur J Pediatr"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The European Reference Network (ERN) on Rare Multisystemic Vascular Diseases (VASCERN) is dedicated to gathering the best expertise in Europe in order to improve and provide high-level healthcare to all patients with rare vascular diseases and provide accessible cross-border healthcare to patients with rare vascular diseases. Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour with local aggressiveness, often associated with a life-threatening coagulopathy known as the Kasabach-Merritt phenomenon (KMP). The Vascular Anomalies Working Group (VASCA-WG) developed diagnostic and therapeutic pathways for the management of KHE using the nominal group technique (NGT), a well-established, structured, multi-stage, facilitated group meeting technique used to generate consensus statements. The pathways were developed following two face-to-face meetings and several web meetings to facilitate discussion.<\/jats:p>\n                  <jats:p>\n                    <jats:italic>Conclusion<\/jats:italic>\n                    : VASCA has produced this expert opinion, which reflects the strategies developed by experts and patient representatives on how to approach patients with Kaposiform Hemangiothelioma in a practical manner; here, we present an algorithmic flow chart of this work.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:table-wrap>\n                      <jats:table>\n                        <jats:tbody>\n                          <jats:tr>\n                            <jats:td align=\"left\">\n                              <jats:bold>What is Known:<\/jats:bold>\n                              \u2022\u00a0\n                              <jats:italic>Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor, often associated with a life threatening coagulopathy, and can be challenging to manage.<\/jats:italic>\n                            <\/jats:td>\n                          <\/jats:tr>\n                          <jats:tr>\n                            <jats:td align=\"left\">\n                              <jats:bold>What is New:<\/jats:bold>\n                              \u2022\u00a0\n                              <jats:italic>The diagnostic and therapeutic pathway for KHE, developed by a multidisciplinary group of experts (VASCA-VASCERN Group), offers concrete offers concrete help in making decisions about managing this condition.<\/jats:italic>\n                            <\/jats:td>\n                          <\/jats:tr>\n                        <\/jats:tbody>\n                      <\/jats:table>\n                    <\/jats:table-wrap>\n                  <\/jats:p>","DOI":"10.1007\/s00431-025-06631-6","type":"journal-article","created":{"date-parts":[[2025,12,13]],"date-time":"2025-12-13T11:05:15Z","timestamp":1765623915000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma"],"prefix":"10.1007","volume":"185","author":[{"given":"Paolo","family":"Gasparella","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emir Q.","family":"Haxhija","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rune","family":"Andersen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Barea","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eulalia","family":"Baselga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel Bejarano","family":"Serrano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sigurd","family":"Berger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annouk Anne","family":"Bisdorff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivia","family":"Boccara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra","family":"Borgards","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Bom-Sucesso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurence M.","family":"Boon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anca Maria","family":"Cimpean","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Diociaiuti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronika","family":"Dvorakova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"May El","family":"Hachem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sofia","family":"Frisk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nader","family":"Ghaffarpour","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annegret","family":"Holm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan D.","family":"Irvine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikkel","family":"Kaltoft","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Friedrich G.","family":"Kapp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olga","family":"Koskova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristiina","family":"Kyrklund","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Madureira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darius","family":"Palionis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Przemys\u0142aw","family":"Przewratil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bitten","family":"Sch\u00f6newolf-Greulich","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria-Corina","family":"Stanciulescu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaroslav","family":"\u0160t\u011brba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jukka","family":"Tolonen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Birute","family":"Vaisnyte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carine","family":"van der Vleuten","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dariusz","family":"Wyrzykowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leo Schultze","family":"Kool","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miikka","family":"Vikkula","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,12,13]]},"reference":[{"issue":"1","key":"6631_CR1","doi-asserted-by":"publisher","first-page":"285","DOI":"10.1016\/j.jpeds.2013.03.080","volume":"163","author":"BA Drolet","year":"2013","unstructured":"Drolet BA, Trenor CC 3rd, Brandao LR, Chiu YE, Chun RH, Dasgupta R et al (2013) Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr 163(1):285\u2013291","journal-title":"J Pediatr"},{"issue":"1","key":"6631_CR2","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1016\/j.jpeds.2012.06.044","volume":"162","author":"SE Croteau","year":"2013","unstructured":"Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB et al (2013) Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 162(1):142\u2013147","journal-title":"J Pediatr"},{"issue":"Suppl 3","key":"6631_CR3","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.29353","volume":"69","author":"JM Mack","year":"2022","unstructured":"Mack JM, Crary SE (2022) How we approach coagulopathy with vascular anomalies. Pediatr Blood Cancer 69(Suppl 3):e29353","journal-title":"Pediatr Blood Cancer"},{"issue":"2","key":"6631_CR4","doi-asserted-by":"publisher","first-page":"e20153257","DOI":"10.1542\/peds.2015-3257","volume":"137","author":"DM Adams","year":"2016","unstructured":"Adams DM, Trenor CC 3rd, Hammill AM, Vinks AA, Patel MN, Chaudry G et al (2016) Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics 137(2):e20153257","journal-title":"Pediatrics"},{"issue":"6","key":"6631_CR5","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0302437","volume":"19","author":"SH Lee","year":"2024","unstructured":"Lee SH, Ten Cate O, Gottlieb M, Horsley T, Shea B, Fournier K et al (2024) The use of virtual nominal groups in healthcare research: an extended scoping review. PLoS ONE 19(6):e0302437","journal-title":"PLoS ONE"},{"issue":"4","key":"6631_CR6","doi-asserted-by":"publisher","first-page":"616","DOI":"10.1016\/j.jaad.2004.10.880","volume":"52","author":"A Gruman","year":"2005","unstructured":"Gruman A, Liang MG, Mulliken JB, Fishman SJ, Burrows PE, Kozakewich HP et al (2005) Kaposiform hemangioendothelioma without Kasabach-merritt phenomenon. J Am Acad Dermatol 52(4):616\u2013622","journal-title":"J Am Acad Dermatol"},{"issue":"4","key":"6631_CR7","doi-asserted-by":"publisher","first-page":"322","DOI":"10.1007\/s12519-018-0171-5","volume":"14","author":"I Schmid","year":"2018","unstructured":"Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Haberle B (2018) Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. World J Pediatr 14(4):322\u2013329","journal-title":"World J Pediatr"},{"issue":"10","key":"6631_CR8","doi-asserted-by":"publisher","first-page":"835","DOI":"10.1111\/1346-8138.15025","volume":"46","author":"X Gong","year":"2019","unstructured":"Gong X, Ying H, Zhang Z, Wang L, Li J, Ding A et al (2019) Ultrasonography and magnetic resonance imaging features of kaposiform hemangioendothelioma and tufted angioma. J Dermatol 46(10):835\u2013842","journal-title":"J Dermatol"},{"issue":"7","key":"6631_CR9","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.27051","volume":"65","author":"F Scorletti","year":"2018","unstructured":"Scorletti F, Hammill A, Patel M, Ricci K, Dasgupta R (2018) Malignant tumors misdiagnosed as benign vascular anomalies. Pediatr Blood Cancer 65(7):e27051","journal-title":"Pediatr Blood Cancer"},{"issue":"4","key":"6631_CR10","doi-asserted-by":"publisher","first-page":"356","DOI":"10.1016\/j.pathol.2017.03.001","volume":"49","author":"J Putra","year":"2017","unstructured":"Putra J, Gupta A (2017) Kaposiform haemangioendothelioma: a review with emphasis on histological differential diagnosis. Pathology 49(4):356\u2013362","journal-title":"Pathology"},{"key":"6631_CR11","doi-asserted-by":"publisher","DOI":"10.1016\/j.anndiagpath.2019.151434","volume":"44","author":"RW Ten Broek","year":"2020","unstructured":"Ten Broek RW, Koelsche C, Eijkelenboom A, Mentzel T, Creytens D, Vokuhl C et al (2020) Kaposiform hemangioendothelioma and tufted angioma - (epi)genetic analysis including genome-wide methylation profiling. Ann Diagn Pathol 44:151434","journal-title":"Ann Diagn Pathol"},{"issue":"4","key":"6631_CR12","doi-asserted-by":"publisher","first-page":"689","DOI":"10.1002\/ijc.35207","volume":"156","author":"T Qiu","year":"2025","unstructured":"Qiu T, Xiang S, Zhou J, Yang M, Lan Y, Zhang X et al (2025) Sirolimus for kaposiform hemangioendothelioma: potential mechanisms of action and resistance. Int J Cancer 156(4):689\u2013699","journal-title":"Int J Cancer"},{"issue":"3","key":"6631_CR13","doi-asserted-by":"publisher","first-page":"440","DOI":"10.1111\/pde.15262","volume":"40","author":"M Maza-Morales","year":"2023","unstructured":"Maza-Morales M, Valdes-Loperena S, Duran-McKinster LC, Garcia-Romero MT (2023) The use of mTOR inhibitors for the treatment of kaposiform hemangioendothelioma. A systematic review. Pediatr Dermatol 40(3):440\u2013445","journal-title":"Pediatr Dermatol"},{"issue":"11","key":"6631_CR14","doi-asserted-by":"publisher","first-page":"1619","DOI":"10.1182\/blood.2021014027","volume":"139","author":"Y Ji","year":"2022","unstructured":"Ji Y, Chen S, Zhou J, Yang K, Zhang X, Xiang B et al (2022) Sirolimus plus prednisolone vs sirolimus monotherapy for kaposiform hemangioendothelioma: a randomized clinical trial. Blood 139(11):1619\u20131630","journal-title":"Blood"},{"issue":"9","key":"6631_CR15","doi-asserted-by":"publisher","first-page":"2345","DOI":"10.1002\/ijc.33406","volume":"148","author":"R Brill","year":"2021","unstructured":"Brill R, Uller W, Huf V, Muller-Wille R, Schmid I, Pohl A et al (2021) Additive value of transarterial embolization to systemic sirolimus treatment in kaposiform hemangioendothelioma. Int J Cancer 148(9):2345\u20132351","journal-title":"Int J Cancer"},{"issue":"6","key":"6631_CR16","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1002\/pbc.20750","volume":"49","author":"J Hauer","year":"2007","unstructured":"Hauer J, Graubner U, Konstantopoulos N, Schmidt S, Pfluger T, Schmid I (2007) Effective treatment of kaposiform hemangioendotheliomas associated with Kasabach-Merritt phenomenon using four-drug regimen. Pediatr Blood Cancer 49(6):852\u2013854","journal-title":"Pediatr Blood Cancer"},{"issue":"10","key":"6631_CR17","doi-asserted-by":"publisher","first-page":"857436","DOI":"10.3389\/fped.2022.857436","volume":"23","author":"A Karastaneva","year":"2022","unstructured":"Karastaneva A, Gasparella P, Tschauner S, Crazzolara R, Kropshofer G, Modl M, Pfleger A, Burmas A, Pocivalnik M, Ulreich R, Zenz W (2022) Indications and limitations of sirolimus in the treatment of vascular anomalies\u2014insights from a retrospective case series. Front Pediat 23(10):857436","journal-title":"Front Pediat"},{"issue":"6","key":"6631_CR18","doi-asserted-by":"publisher","first-page":"2769","DOI":"10.1111\/bcp.15202","volume":"88","author":"VEM Harbers","year":"2022","unstructured":"Harbers VEM, van der Salm N, Pegge SAH, van der Vleuten CJM, Verhoeven BH, Vrancken S et al (2022) Effective low-dose sirolimus regimen for kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon in young infants. Br J Clin Pharmacol 88(6):2769\u20132781","journal-title":"Br J Clin Pharmacol"},{"key":"6631_CR19","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.26470","author":"T Mizuno","year":"2017","unstructured":"Mizuno T, Fukuda T, Emoto C, Mobberley-Schuman PS, Hammill AM, Adams DM et al (2017) Developmental pharmacokinetics of sirolimus: implications for precision dosing in neonates and infants with complicated vascular anomalies. Pediatr Blood Cancer. https:\/\/doi.org\/10.1002\/pbc.26470","journal-title":"Pediatr Blood Cancer"},{"issue":"12","key":"6631_CR20","doi-asserted-by":"publisher","first-page":"1840","DOI":"10.3390\/biom12121840","volume":"12","author":"FG Kapp","year":"2022","unstructured":"Kapp FG, Schneider C, Holm A, Glonnegger H, Niemeyer CM, R\u00f6\u00dfler J, Zieger B (2022) Comprehensive analyses of coagulation parameters in patients with vascular anomalies. Biomolecules 12(12):1840","journal-title":"Biomolecules"},{"issue":"3","key":"6631_CR21","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.30779","volume":"71","author":"AJ Borst","year":"2024","unstructured":"Borst AJ, Eng W, Griffin M, Ricci KW, Engel E, Adams DM et al (2024) Treatment practices and response in kaposiform hemangioendothelioma: a multicenter cohort study. Pediatr Blood Cancer 71(3):e30779","journal-title":"Pediatr Blood Cancer"},{"key":"6631_CR22","doi-asserted-by":"publisher","first-page":"1480213","DOI":"10.3389\/fped.2025.1480213","volume":"13","author":"Y Zhang","year":"2025","unstructured":"Zhang Y, Qiu T, Yang C, Zhou J, Yang M, Gong X et al (2025) Similarities and differences in the clinical features and management of primary lymphedema and kaposiform hemangioendothelioma associated with lymphedema in children. Front Pediatr 13:1480213","journal-title":"Front Pediatr"}],"container-title":["European Journal of Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00431-025-06631-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00431-025-06631-6","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00431-025-06631-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,5]],"date-time":"2026-02-05T02:30:44Z","timestamp":1770258644000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00431-025-06631-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,13]]},"references-count":22,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,1]]}},"alternative-id":["6631"],"URL":"https:\/\/doi.org\/10.1007\/s00431-025-06631-6","relation":{},"ISSN":["1432-1076"],"issn-type":[{"value":"1432-1076","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,13]]},"assertion":[{"value":"4 September 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 November 2025","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 November 2025","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 December 2025","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"The authors declare no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"14"}}