{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T10:37:07Z","timestamp":1788863827605,"version":"build-2803163510"},"reference-count":18,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,1,30]],"date-time":"2023-01-30T00:00:00Z","timestamp":1675036800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Dermatology"],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Kaposiform hemangioendothelioma (KHE) is a locally aggressive and potentially lethal vascular tumor of infancy. Current consensus recommendations include the use of vincristine and\/or systemic steroids as first\u2010line treatment. Mammalian target of rapamycin (mTOR) inhibitors represent a promising therapy for patients with KHE. The goal of our study is to critically assess the existing literature on outcomes of patients with KHE treated with mTOR inhibitors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a literature search from 1 January 2000, to 30 April 2022. Articles reporting outcomes of patients treated with mTOR inhibitors for KHE were included. Descriptive statistics were used to describe and summarize the outcomes of the treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We included 327 patients with a mean age at diagnosis of 9.1\u00a0months (SD\u2009\u00b1\u20099). Patients were treated with an mTOR inhibitor for a mean of 15.2\u00a0months (SD\u2009\u00b1\u20094.1). A total of 315 (96.3%) patients had positive outcomes including improvement of the tumor size, symptoms and\/or laboratory parameters in 227 (85%) and complete remission in 38 (12%). Seven (2%) patients did not respond to treatment and seven (2%) died of sepsis (4), Kasabach\u2013Merritt phenomenon complications (1), cardiac and liver failure due to ductus arteriosus (1), or metastatic disease (1).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This systematic review supports the efficacy and safety of mTOR inhibitors for KHE. Their use resulted in positive outcomes in terms of decreased symptoms, reduction in tumor size and improvement in biochemical parameters with a mortality rate of 2%. According to these findings, we suggest revised consensus treatment guidelines for KHE with mTOR inhibitors potentially considered first\u2010line therapy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pde.15262","type":"journal-article","created":{"date-parts":[[2023,1,30]],"date-time":"2023-01-30T23:59:23Z","timestamp":1675123163000},"page":"440-445","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["The use of mTOR inhibitors for the treatment of kaposiform hemangioendothelioma. A systematic review"],"prefix":"10.1111","volume":"40","author":[{"given":"Mariana","family":"Maza\u2010Morales","sequence":"first","affiliation":[{"name":"Dermatology Department National Institute of Pediatrics  Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5035-2181","authenticated-orcid":false,"given":"Sof\u00eda","family":"Vald\u00e9s\u2010Loperena","sequence":"additional","affiliation":[{"name":"Dermatology Department National Institute of Pediatrics  Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4977-4958","authenticated-orcid":false,"given":"Lourdes Carola","family":"Dur\u00e1n\u2010McKinster","sequence":"additional","affiliation":[{"name":"Dermatology Department National Institute of Pediatrics  Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1408-8109","authenticated-orcid":false,"given":"Mar\u00eda Teresa","family":"Garc\u00eda\u2010Romero","sequence":"additional","affiliation":[{"name":"Dermatology Department National Institute of Pediatrics  Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,1,30]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010020\u20101320\u20101"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2013.03.080"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015\u20103257"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.n160"},{"key":"e_1_2_9_6_1","unstructured":"Oxford Centre for Evidence\u2010based medicine: Levels of Evidence(2009).Centre for Evidence\u2010Based Medicine (CEBM) University of Oxford. Published October 1 2020. Accessed August 30 2022.https:\/\/www.cebm.ox.ac.uk\/resources\/levels\u2010of\u2010evidence\/oxford\u2010centre\u2010for\u2010evidence\u2010based\u2010medicine\u2010levels\u2010of\u2010evidence\u2010march\u20102009."},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.16601"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000003431"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.30216"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.15767"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2016\u20102919"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25296"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e3181e001a9"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2017\u20101044"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-200208000-00010"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24543"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/jpc.14370"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.arcped.2019.06.003"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.30775"}],"container-title":["Pediatric Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.15262","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pde.15262","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.15262","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,12]],"date-time":"2023-10-12T04:52:16Z","timestamp":1697086336000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pde.15262"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1,30]]},"references-count":18,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,5]]}},"alternative-id":["10.1111\/pde.15262"],"URL":"https:\/\/doi.org\/10.1111\/pde.15262","archive":["Portico"],"relation":{},"ISSN":["0736-8046","1525-1470"],"issn-type":[{"value":"0736-8046","type":"print"},{"value":"1525-1470","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,1,30]]},"assertion":[{"value":"2022-09-13","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-01-06","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-01-30","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}