{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T09:51:46Z","timestamp":1773827506136,"version":"3.50.1"},"reference-count":40,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2019,10,1]],"date-time":"2019-10-01T00:00:00Z","timestamp":1569888000000},"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 Blood &amp; Cancer"],"published-print":{"date-parts":[[2020,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Retinoblastoma with macroscopic optic nerve (ON) invasion depicted by imaging at diagnosis remains a major problem and carries a poor prognosis. We sought to describe the treatment and outcome of these high\u2010risk patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Retrospective mono\u2010institutional clinical, radiological, and histological review of patients with uni\u2010 or bilateral retinoblastoma with obvious ON invasion, defined by radiological optic nerve enlargement (RONE) depicted by computed tomography scan or magnetic resonance imaging (MRI), was performed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Between 1997 and 2014, among the 936 patients with retinoblastoma treated at Institut Curie, 11 had detectable RONE. Retinoblastoma was unilateral in 10 and bilateral in one. Median age at diagnosis was 28 months (range, 11\u201096). ON enlargement extended to the orbital portion in three patients, to the optic canal in five, to the prechiasmatic portion in two, and to the optic chiasm in one. Nine patients received neoadjuvant chemotherapy and partial response was obtained in all. Enucleation was performed in 10\/11 patients\u2014by an anterior approach in three and by anterior and subfrontal approaches in seven. Three patients had a positive ON resection margin (2\/3 after primary enucleation). All enucleated patients received adjuvant treatment (conventional chemotherapy: 10, high\u2010dose chemotherapy: seven, radiotherapy: five). Leptomeningeal progression occurred in four patients. Seven are in first complete remission (median follow up: 8 years [3.5\u201019.4]).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Neoadjuvant chemotherapy and microscopic complete resection have a pivotal role in the management of retinoblastoma with RONE. MRI is recommended for initial and pre\u2010operative accurate staging. Surgery should be performed by neurosurgeons in case of posterior nerve invasion. Radiotherapy is required in case of incomplete resection.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.27998","type":"journal-article","created":{"date-parts":[[2019,10,1]],"date-time":"2019-10-01T07:19:58Z","timestamp":1569914398000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Management of advanced uni\u2010 or bilateral retinoblastoma with macroscopic optic nerve invasion"],"prefix":"10.1002","volume":"67","author":[{"given":"Marie\u2010Louise","family":"Choucair","sequence":"first","affiliation":[{"name":"Oncology Center SIREDO: Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer Institut Curie  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2794-5875","authenticated-orcid":false,"given":"Herv\u00e9 J.","family":"Brisse","sequence":"additional","affiliation":[{"name":"Radiology Department Institut Curie  Paris France"},{"name":"Paris Sciences et Lettres Research University  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Fr\u00e9neaux","sequence":"additional","affiliation":[{"name":"Paris Sciences et Lettres Research University  Paris France"},{"name":"Tumor Biology Department Institut Curie  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurence","family":"Desjardins","sequence":"additional","affiliation":[{"name":"Paris Sciences et Lettres Research University  Paris France"},{"name":"Ocular Oncology Institut Curie  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Georg","family":"Dorfm\u00fcller","sequence":"additional","affiliation":[{"name":"Pediatric Neurosurgery Department Fondation Rothschild  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"St\u00e9phanie","family":"Puget","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurosurgery Necker Hospital  Paris France"},{"name":"Universit\u00e9 Paris Descartes  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R\u00e9mi","family":"Dendale","sequence":"additional","affiliation":[{"name":"Paris Sciences et Lettres Research University  Paris France"},{"name":"Radiation Oncology Department Institut Curie  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marion","family":"Chevrier","sequence":"additional","affiliation":[{"name":"Paris Sciences et Lettres Research University  Paris France"},{"name":"Biostatistics Department Institut Curie  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fran\u00e7ois","family":"Doz","sequence":"additional","affiliation":[{"name":"Oncology Center SIREDO: Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer Institut Curie  Paris France"},{"name":"Universit\u00e9 Paris Descartes  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Livia","family":"Lumbroso\u2010Le Rouic","sequence":"additional","affiliation":[{"name":"Paris Sciences et Lettres Research University  Paris France"},{"name":"Ocular Oncology Institut Curie  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5213-3353","authenticated-orcid":false,"given":"Isabelle","family":"Aerts","sequence":"additional","affiliation":[{"name":"Oncology Center SIREDO: Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer Institut Curie  Paris France"},{"name":"Paris Sciences et Lettres Research University  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,10]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.22608\/APO.201778"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.11647"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25048"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-011-2201-5"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-014-3514-1"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(20001115)89:10<2117::AID-CNCR12>3.0.CO;2-9"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.87.3.327"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/mpo.10249"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.22504"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.22491"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1080\/02841850410004238"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1995.13.4.902"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1993.11.4.630"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.5858\/133.8.1199"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/mpo.2950130606"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.23317"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0959-8049(97)10019-3"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0360-3016(02)03756-2"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24468"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20606"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.survophthal.2015.01.002"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21067"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0142(19960315)77:6<1206::AID-CNCR30>3.0.CO;2-3"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.120.7.923"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21427"},{"issue":"1","key":"e_1_2_9_27_1","first-page":"41","article-title":"Evaluation of chemoprophylaxis in patients with unilateral retinoblastoma with high\u2010risk features on histopathologic examination","volume":"119","author":"Uusitalo MS","year":"2001","journal-title":"Arch Ophthalmol"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1136\/bjophthalmol-2014-306222"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.26270"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2012.42.3962"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e3181923cc5"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-199909000-00006"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2010.32.5332"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.35.9190"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12094-006-0093-x"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2017.35.15_suppl.10506"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.1097\/IAE.0000000000000253"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.3109\/13816818709028524"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24280"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.3109\/13816810.2011.592173"},{"key":"e_1_2_9_41_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21052"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.27998","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/pbc.27998","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.27998","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,9]],"date-time":"2023-09-09T01:11:21Z","timestamp":1694221881000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.27998"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10]]},"references-count":40,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,1]]}},"alternative-id":["10.1002\/pbc.27998"],"URL":"https:\/\/doi.org\/10.1002\/pbc.27998","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,10]]},"assertion":[{"value":"2019-04-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-09-04","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-10-01","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e27998"}}