Retinoblastoma Survival Following Primary Enucleation by AJCC Staging
Primary enucleation of the eye with retinoblastoma is a widely accessible, life-saving treatment for retinoblastoma. This study evaluated the survival of patients following primary enucleation based on AJCC 8th edition staging. Included were 700 consecutive patients (700 eyes) treated with primary e...
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MDPI AG
2021-12-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/13/24/6240 |
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| author | Junyang Zhao Zhaoxun Feng Gareth Leung Brenda L. Gallie |
| author_facet | Junyang Zhao Zhaoxun Feng Gareth Leung Brenda L. Gallie |
| author_sort | Junyang Zhao |
| collection | DOAJ |
| description | Primary enucleation of the eye with retinoblastoma is a widely accessible, life-saving treatment for retinoblastoma. This study evaluated the survival of patients following primary enucleation based on AJCC 8th edition staging. Included were 700 consecutive patients (700 eyes) treated with primary enucleation at 29 Chinese treatment centers between 2006 and 2015. Excluded were patients with less than one year follow-up, bilateral retinoblastoma, clinical evidence of extraocular disease at diagnosis, or prior focal or systemic therapy. The 5-year overall survival was 95.5%, and 5-year disease-specific survival (DSS) was 95.7%. Survival was better when enucleation was <26 days from diagnosis than delayed >26 days (96.1% vs. 86.9%; <i>p</i> = 0.017). Patients with eyes presenting with raised intraocular pressure with neovascularization and/or buphthalmos (cT3c) had worse 5-year DSS (87.1%) than those without (cT2b, 99.1%; cT3b, 98.7%; cT3d, 97.2%) (<i>p</i> < 0.05). The 5-year DSS based on pathological staging was pT1 (99.5%), pT2a (95.5%), pT3a (100%), pT3b (93.0%), pT3c/d (92.3%), and pT4 (40.9%). Patients with pT3 pathology who received six cycles of adjuvant chemotherapy had better 5-year DSS (97.7%) than those with no chemotherapy (88.1%; <i>p</i> = 0.06) and those who underwent 1–3 cycles (86.9%, <i>p</i> = 0.02) or 4–5 cycles (89.3%, <i>p</i> = 0.06). Patients with pT4 pathology who received six cycles of chemotherapy had better 5-year DSS than those with 0–5 cycles (63.6% vs. 16.7%; <i>p</i> = 0.02). Prompt primary enucleation yielded high long-term survival for children with retinoblastoma. The AJCC 8th edition staging is predictive of survival. |
| first_indexed | 2024-03-10T04:31:36Z |
| format | Article |
| id | doaj.art-3a12121e12c0436897494ea5524f6405 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-10T04:31:36Z |
| publishDate | 2021-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-3a12121e12c0436897494ea5524f64052023-11-23T04:05:46ZengMDPI AGCancers2072-66942021-12-011324624010.3390/cancers13246240Retinoblastoma Survival Following Primary Enucleation by AJCC StagingJunyang Zhao0Zhaoxun Feng1Gareth Leung2Brenda L. Gallie3Department of Pediatric Oncology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing 100045, ChinaDepartment of Ophthalmology, University of Ottawa, Ottawa, ON K1L 8L6, CanadaDepartment of Ophthalmology, University of Ottawa, Ottawa, ON K1L 8L6, CanadaDepartment of Ophthalmology and Vision Science, Hospital for Sick Children, Toronto, ON M5G 1X8, CanadaPrimary enucleation of the eye with retinoblastoma is a widely accessible, life-saving treatment for retinoblastoma. This study evaluated the survival of patients following primary enucleation based on AJCC 8th edition staging. Included were 700 consecutive patients (700 eyes) treated with primary enucleation at 29 Chinese treatment centers between 2006 and 2015. Excluded were patients with less than one year follow-up, bilateral retinoblastoma, clinical evidence of extraocular disease at diagnosis, or prior focal or systemic therapy. The 5-year overall survival was 95.5%, and 5-year disease-specific survival (DSS) was 95.7%. Survival was better when enucleation was <26 days from diagnosis than delayed >26 days (96.1% vs. 86.9%; <i>p</i> = 0.017). Patients with eyes presenting with raised intraocular pressure with neovascularization and/or buphthalmos (cT3c) had worse 5-year DSS (87.1%) than those without (cT2b, 99.1%; cT3b, 98.7%; cT3d, 97.2%) (<i>p</i> < 0.05). The 5-year DSS based on pathological staging was pT1 (99.5%), pT2a (95.5%), pT3a (100%), pT3b (93.0%), pT3c/d (92.3%), and pT4 (40.9%). Patients with pT3 pathology who received six cycles of adjuvant chemotherapy had better 5-year DSS (97.7%) than those with no chemotherapy (88.1%; <i>p</i> = 0.06) and those who underwent 1–3 cycles (86.9%, <i>p</i> = 0.02) or 4–5 cycles (89.3%, <i>p</i> = 0.06). Patients with pT4 pathology who received six cycles of chemotherapy had better 5-year DSS than those with 0–5 cycles (63.6% vs. 16.7%; <i>p</i> = 0.02). Prompt primary enucleation yielded high long-term survival for children with retinoblastoma. The AJCC 8th edition staging is predictive of survival.https://www.mdpi.com/2072-6694/13/24/6240retinoblastomaenucleationsurvivalAJCCclassificationstaging |
| spellingShingle | Junyang Zhao Zhaoxun Feng Gareth Leung Brenda L. Gallie Retinoblastoma Survival Following Primary Enucleation by AJCC Staging Cancers retinoblastoma enucleation survival AJCC classification staging |
| title | Retinoblastoma Survival Following Primary Enucleation by AJCC Staging |
| title_full | Retinoblastoma Survival Following Primary Enucleation by AJCC Staging |
| title_fullStr | Retinoblastoma Survival Following Primary Enucleation by AJCC Staging |
| title_full_unstemmed | Retinoblastoma Survival Following Primary Enucleation by AJCC Staging |
| title_short | Retinoblastoma Survival Following Primary Enucleation by AJCC Staging |
| title_sort | retinoblastoma survival following primary enucleation by ajcc staging |
| topic | retinoblastoma enucleation survival AJCC classification staging |
| url | https://www.mdpi.com/2072-6694/13/24/6240 |
| work_keys_str_mv | AT junyangzhao retinoblastomasurvivalfollowingprimaryenucleationbyajccstaging AT zhaoxunfeng retinoblastomasurvivalfollowingprimaryenucleationbyajccstaging AT garethleung retinoblastomasurvivalfollowingprimaryenucleationbyajccstaging AT brendalgallie retinoblastomasurvivalfollowingprimaryenucleationbyajccstaging |