{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T04:46:55Z","timestamp":1760244415189,"version":"build-2065373602"},"reference-count":29,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2022,12,16]],"date-time":"2022-12-16T00:00:00Z","timestamp":1671148800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Seoul National University Hospital Research Fund","award":["0320200110"],"award-info":[{"award-number":["0320200110"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Primary enucleation is a life-saving treatment for advanced intraocular retinoblastoma, particularly in patients with poor visual potential and functional contralateral eyes. This single-center study presents the treatment outcomes of patients with unilateral retinoblastoma who received primary enucleation and adjuvant chemotherapy with cyclophosphamide, vincristine, doxorubicin, and intrathecal methotrexate (CVDM) between 2000 and 2020. Twenty patients were enrolled in the study. The median age at diagnosis was 26 months (range, 1\u201345). Eighteen patients (90%) were in group E and two (10%) were in group D, according to the intraocular classification of retinoblastoma guidelines. Excluding one patient with an inadequate specimen, 19 patients (95%) had optic nerve involvement (ONI) at least up to the lamina cribrosa. Eight patients (40%) had choroidal invasion in addition to ONI. Two patients (10%) were surgical resection margin positive. The overall and event-free survival rates were 100% and 95%, respectively, for a median follow-up duration of 102.24 months (range 24.2\u2013202.9). There were no relapses or deaths due to any cause, but one patient developed secondary rhabdomyosarcoma 99.6 months after chemotherapy. Treatment was well tolerated, with minimal hematotoxicity and hepatotoxicity. CVDM as a post-enucleation chemotherapy for advanced intraocular retinoblastoma has excellent outcomes with tolerable toxicity. However, in line with updated treatment trends, further risk stratification and lowering the treatment intensity should be considered. Continued long-term follow-up is required to further determine late effects.<\/jats:p>","DOI":"10.3390\/children9121983","type":"journal-article","created":{"date-parts":[[2022,12,19]],"date-time":"2022-12-19T06:24:44Z","timestamp":1671431084000},"page":"1983","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Twenty-Year Retrospective Study of Post-Enucleation Chemotherapy in High-Risk Patients with Unilateral Retinoblastoma"],"prefix":"10.3390","volume":"9","author":[{"given":"Yoon","family":"Sunwoo","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jung Yoon","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyun Jin","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bo Kyung","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8822-1988","authenticated-orcid":false,"given":"Kyung Taek","family":"Hong","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sang In","family":"Khwarg","sequence":"additional","affiliation":[{"name":"Department of Opthalmology, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jaemoon","family":"Koh","sequence":"additional","affiliation":[{"name":"Department of Pathology, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8681-1597","authenticated-orcid":false,"given":"Sung-Hye","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Pathology, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6320-6829","authenticated-orcid":false,"given":"Dong Hyun","family":"Jo","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeong Hun","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Opthalmology, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jung-Eun","family":"Cheon","sequence":"additional","affiliation":[{"name":"Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyoung Jin","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Republic of Korea"},{"name":"Wide River Institute of Immunology, Hongcheon 25159, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,12,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1514","DOI":"10.1001\/archopht.1990.01070130016001","article-title":"Retinoblastoma incidence in the United States","volume":"108","author":"Abramson","year":"1990","journal-title":"Arch. 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