{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T23:44:36Z","timestamp":1785282276855,"version":"3.55.0"},"reference-count":19,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,10,8]],"date-time":"2017-10-08T00:00:00Z","timestamp":1507420800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2018,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We report the case of a 6\u2010year\u2010old female with xeroderma pigmentosum (XP) who developed a nonoperable scalp tumor, treated with anti\u2010programmed cell death protein 1 (anti\u2010PD\u20101) therapy (nivolumab).\u00a0She presented with a sarcomatoid carcinoma of the scalp with bone lysis as well as vascular and meningeal contact. Nivolumab was initiated because it has emerged as a promising immunotherapy. We observed a dramatic tumor response with excellent tolerance. However, while on nivolumab therapy she developed two large skin melanomas and several squamous cell carcinomas, which have been resected. These results demonstrate that cancer immunotherapy in patients with XP can be impressive but complex and warrants further investigation.<\/jats:p>","DOI":"10.1002\/pbc.26837","type":"journal-article","created":{"date-parts":[[2017,10,8]],"date-time":"2017-10-08T11:52:48Z","timestamp":1507463568000},"source":"Crossref","is-referenced-by-count":23,"title":["Dramatic response to nivolumab in xeroderma pigmentosum skin tumor"],"prefix":"10.1002","volume":"65","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3301-4495","authenticated-orcid":false,"given":"Fanny","family":"Chambon","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology CHU F\u00e9lix Guyon  Saint Denis France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Osdoit","sequence":"additional","affiliation":[{"name":"Department of Dermatology CHU F\u00e9lix Guyon  Saint Denis France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly","family":"Bagny","sequence":"additional","affiliation":[{"name":"Department of Dermatology CHU F\u00e9lix Guyon  Saint Denis France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Moro","sequence":"additional","affiliation":[{"name":"Department of Plastic Pediatric Surgery CHU F\u00e9lix Guyon  Saint Denis France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics CH Mayotte  Mamoudzou France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"R\u00e9guerre","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology CHU F\u00e9lix Guyon  Saint Denis France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,10,8]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2015.61.1509"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2013.53.0105"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.2165\/00003495-200767170-00008"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1586\/era.11.20"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2010.34.1735"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2011.03.005"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2010.083022"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrc3239"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/2051-1426-1-11"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.aad0095"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.4161\/onci.27817"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12916-016-0705-4"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12967-017-1119-6"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2013.08.019"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1602252"},{"issue":"10","key":"e_1_2_8_17_1","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1093\/jnci\/djv295","article-title":"ASCO reveals additional promising results with immunotherapies","volume":"107","author":"Brower V","year":"2015","journal-title":"J Natl Cancer Inst"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(15)70076-8"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(14)60958-2"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-015-0442-6"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.26837","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.26837","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T15:28:02Z","timestamp":1696001282000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.26837"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,10,8]]},"references-count":19,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2018,2]]}},"alternative-id":["10.1002\/pbc.26837"],"URL":"https:\/\/doi.org\/10.1002\/pbc.26837","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,10,8]]},"article-number":"e26837"}}