Twenty-Year Retrospective Study of Post-Enucleation Chemotherapy in High-Risk Patients with Unilateral Retinoblastoma
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 enuc...
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MDPI AG
2022-12-01
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| Online Access: | https://www.mdpi.com/2227-9067/9/12/1983 |
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| author | Yoon Sunwoo Jung Yoon Choi Hyun Jin Park Bo Kyung Kim Kyung Taek Hong Sang In Khwarg Jaemoon Koh Sung-Hye Park Dong Hyun Jo Jeong Hun Kim Jung-Eun Cheon Hyoung Jin Kang |
| author_facet | Yoon Sunwoo Jung Yoon Choi Hyun Jin Park Bo Kyung Kim Kyung Taek Hong Sang In Khwarg Jaemoon Koh Sung-Hye Park Dong Hyun Jo Jeong Hun Kim Jung-Eun Cheon Hyoung Jin Kang |
| author_sort | Yoon Sunwoo |
| collection | DOAJ |
| description | 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, 1–45). 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 24.2–202.9). 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. |
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| series | Children |
| spelling | doaj.art-d8f85ef2dca44e16bcb40f5272d8e1542023-11-24T14:03:04ZengMDPI AGChildren2227-90672022-12-01912198310.3390/children9121983Twenty-Year Retrospective Study of Post-Enucleation Chemotherapy in High-Risk Patients with Unilateral RetinoblastomaYoon Sunwoo0Jung Yoon Choi1Hyun Jin Park2Bo Kyung Kim3Kyung Taek Hong4Sang In Khwarg5Jaemoon Koh6Sung-Hye Park7Dong Hyun Jo8Jeong Hun Kim9Jung-Eun Cheon10Hyoung Jin Kang11Department of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Opthalmology, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Pathology, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Pathology, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Anatomy and Cell Biology, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Opthalmology, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Radiology, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaDepartment of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Republic of KoreaPrimary 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, 1–45). 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 24.2–202.9). 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.https://www.mdpi.com/2227-9067/9/12/1983retinoblastomapost-enucleationadjuvant chemotherapyunilateralhigh risk factors |
| spellingShingle | Yoon Sunwoo Jung Yoon Choi Hyun Jin Park Bo Kyung Kim Kyung Taek Hong Sang In Khwarg Jaemoon Koh Sung-Hye Park Dong Hyun Jo Jeong Hun Kim Jung-Eun Cheon Hyoung Jin Kang Twenty-Year Retrospective Study of Post-Enucleation Chemotherapy in High-Risk Patients with Unilateral Retinoblastoma Children retinoblastoma post-enucleation adjuvant chemotherapy unilateral high risk factors |
| title | Twenty-Year Retrospective Study of Post-Enucleation Chemotherapy in High-Risk Patients with Unilateral Retinoblastoma |
| title_full | Twenty-Year Retrospective Study of Post-Enucleation Chemotherapy in High-Risk Patients with Unilateral Retinoblastoma |
| title_fullStr | Twenty-Year Retrospective Study of Post-Enucleation Chemotherapy in High-Risk Patients with Unilateral Retinoblastoma |
| title_full_unstemmed | Twenty-Year Retrospective Study of Post-Enucleation Chemotherapy in High-Risk Patients with Unilateral Retinoblastoma |
| title_short | Twenty-Year Retrospective Study of Post-Enucleation Chemotherapy in High-Risk Patients with Unilateral Retinoblastoma |
| title_sort | twenty year retrospective study of post enucleation chemotherapy in high risk patients with unilateral retinoblastoma |
| topic | retinoblastoma post-enucleation adjuvant chemotherapy unilateral high risk factors |
| url | https://www.mdpi.com/2227-9067/9/12/1983 |
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