ObjectiveTo evaluate the efficacy and safety of comprehensive treatment for retinoblastoma (RB). MethodsA retrospective clinical study. From January to December in 2019, 157 cases (203 eyes) of RB who were diagnosed by the Department of Ophthalmology of Xinhua Hospital and received comprehensive treatment were included in this study. Of cases, 76 were male, and 81 were female; 111 were unilateral, and 46 were bilateral. The medium of age at diagnosis was 20.1 months. All patients received treatment for the first time. Patients with intraocular tumors were divided into A-E stages, extraocular stage and distant metastasis according to international intraocular RB classification standard. The median follow-up time was 37.4 months. Clinical features, treatment, prognosis and ocular complications of all cases were recorded. ResultsAmong 157 cases (203 eyes), 137 cases (180 eyes) were in intraocular stage; 6, 14, 10, 98, and 52 of eyes were in A-E stages, respectively. Twelve cases (12 eyes) were in extraocular stage; 8 cases (11 eyes) were in distant metastasis stag; 8 cases died due to distant metastasis; 149 cases (94.9%, 149/157) survived; 48 eyes were enucleated, 34 of which underwent initial enucleation, and 14 eyes underwent enucleation after eye-preserving treatment. The overall global salvage rate was 155 eyes (76.4%,155/203), and that after eye-preserving treatment was 91.7% (155/169). Severer eye for bilateral cases was taken into account for statistic; 120 cases (120 eyes) received initial eye-preserving treatment. Among them, 36 and 84 eyes underwent initial intravenous chemotherapy (IVC) and initial intra-arterial chemotherapy (IAC), respectively. The enucleation of the two groups was 7(19.4%, 7/36), 7(8.3%, 7/84); 33 (91.7%, 33/36) and 33 (39.3%, 33/84) eyes received the second treatment, respectively. There was no significant difference in the rate of enucleation between the two treatments (χ2=2.037, P=0.154). There was significant difference in the percentage of secondary treatment (χ2=27.937, P＜0.001). Fifty-four eyes (45.0%, 54/120) stabilized after initial treatment, and 66 eyes (55.0%, 66/120) underwent secondary treatments due to poor response or tumor recurrence. For 66 eyes receiving secondary treatments, enucleation, IAC, intravitreous chemotherapy (IVitC), IAC combined with IVitC, and laser and/or cryotherapy was performed in 6, 18, 12, 13, and 17 eyes, respectively. The number of eyes of enucleation among the IAC, IVitC, and IAC combined with IVitC group was 5 (27.8%, 5/18), 1 (9.3%, 1/12), and 2 (15.4%, 2/13) eyes, which was no significantly different (χ2=2.001, P=0.368). Until the last follow-up, visual acuity outcomes were acquired in 148 eyes (72.9%, 148/203). Among them, 41, 53, 16 and 38 eyes had no light perception, light perception to finger counting, 20/400, and ≥20/200, respectively. In total, among 203 eyes, 121 eyes received IAC, of which 2, 4, and 1 eyes had optic disc atrophy, vitreous hemorrhage, and severe retinal-choroidal atrophy, respectively; 60 eyes received IVitC, of which one and one eye had vitreous hemorrhage and macular hemorrhagic necrosis, respectively. ConclusionsIn this study, the overall survival rate was 94.9% after comprehensive treatment and the rate of global salvage after eye-preserving treatment was 91.7%. The comprehensive treatment of retinoblastoma had a relatively high efficacy and safety.
Objective To observe the high-risk histopathological feature (HRF) and their correlation with prognosis in children with intraocular retinoblastoma (RB) in the intraocular stage after failed eye-preserving treatment and enucleation surgery. MethodsA single-center retrospective case study. From August 2018 to January 2023, 64 children (64 eyes) with advanced intraocular RB who were diagnosed in the ophthalmology department of Beijing Children's Hospital and underwent enucleation surgery after failed eye-preserving treatment were included in the study. The median follow-up time was 51 months. The gender of the children patients, the age of visit and enucleation, International Intraocular Retinoblastoma Classification (IIRC), the initial chemotherapy regimen (hereinafter referred to as "chemotherapy"), the time of enucleation surgery, pathological results, post-enucleation treatment methods and prognosis were collected. The Mann-Whitney U test was used for comparison between groups. Survival analysis was performed using the Kaplan-Meier method, and the log-rank test was used for comparison between groups. ResultsAmong 64 cases and 64 eyes, 370 were male and 27 were female. The age of seeking medical treatment was 20 (11-31) months. The age at which the surgery was performed was 29 (16-40) months. The number of eyes in IIRC stage D and E was 16 and 48 respectively. The initial chemotherapy regimens simply applied (hereinafter referred to as "alone") intravenous systemic chemotherapy (IVC) and ophthalmic artery infusion chemotherapy (IAC) in 40 cases and 11 cases, 13 cases of IVC+IAC. All patients with positive HRF received systemic adjuvant chemotherapy after surgery. There were 37 eyes (57.8%, 37/64) positive for HRF. There was no statistically significant difference in the positive rate of HRF between children in IIRC stage D and stage E (χ2=0.021, P=0.884). Among the 37 eyes with HRF, the numbers of eyes with extensive choroidal invasion, posterior lamina cribrosa optic nerve invasion, scleral invasion and scleral invasion were 17 (45.9%, 17/37), 16 (43.2%, 16/37), 3 (8.1%, 3/37) and 3 (8.1%, 3/37), respectively. During the follow-up period, there were 5 cases (7.8%, 5/64) of extraocular metastasis of the tumor and death, all of which were stage E and had HRF. Among them, the initial treatment plan was IAC for 4 cases, one case of IVC. The survival rates of children among the IVC, IAC or IVC+IAC regimens were 97.5% (39/40), 63.6% (7/11), and 100.0% (13/13) eyes, respectively. The comparison of survival rates among different chemotherapy regimens showed statistically significant differences (χ2=14.233, P＜0.001). The results of survival analysis showed that the cumulative survival rate of those with extensive choroidal invasion, posterior lamina cribrosa optic nerve infiltration, and those who received IAC was significantly lower than that of those without extensive choroidal invasion, posterior lamina cribrosa optic nerve infiltration, and those who received IVC+IAC and IVC (P＜0.05). ConclusionsEye-preserving treatment for children with advanced intraocular RB may increase the positive rate of HRF and the risk of extraocular metastasis. The IVC+IAC eye-protecting treatment plan can improve the survival rate of children.