Tylectomy Safety in Salvage of Eyes with Retinoblastoma.
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SUBMITTER: Zhao J
PROVIDER: S-EPMC8616183 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:The impact of chemotherapy, focal therapies, radiation and co-existing ocular morbidities on histology of eyes with retinoblastoma enucleated following chemoreduction is not well known.Twenty-five eyes (23 patients) with retinoblastoma enucleated after failing eye-salvage therapy were evaluated. Reasons for enucleation (tumor progression, subretinal or vitreous seeds) and co-morbid conditions (neovascular glaucoma, cataract, vitreous hemorrhage and retinal detachment) were documented. All specimens were reviewed for evidence of ciliary body, choroidal, optic nerve, and scleral invasion.The median age at diagnosis was 14 months (range, 1-37 months). Twenty eyes were classified as Reese-Ellsworth Group IV-V at diagnosis. Twenty-four eyes had recurrent disease at enucleation; one eye was enucleated for neovascular glaucoma and vitreous hemorrhage. Co-existing ocular morbidities at enucleation included vitreous hemorrhage (n = 6), retinal detachment (n = 9), neovascular glaucoma (n = 9) and cataracts (n = 3). Histologic findings included choroidal invasion (n = 7), ciliary body invasion (n = 4), optic nerve invasion (n = 6) and scleral invasion (n = 3). The median time from diagnosis to enucleation was 11 months. Co-existing retinal detachment and vitreous hemorrhage significantly increased the likelihood of optic nerve invasion (P = 0.014 and P = 0.011, respectively). Prolonged time to enucleation was significantly associated with the likelihood of choroidal (P = 0.010) and ciliary body (P = 0.021) invasion as well as invasion of multiple sites.In eyes with retinoblastoma enucleated after chemoreduction, co-existing ocular morbidities and time to enucleation are predictive of extra-retinal extension.
| S-EPMC4643656 | biostudies-literature
Project description:PurposeTo examine the outcome of salvage intra-arterial chemotherapy (IAC) for patients with recurrent retinoblastoma after the initial course of IAC and determine the factors influencing clinical outcome.MethodsA total of 73 eyes of 71 patients with recurrent retinoblastoma undergoing salvage IAC after initial successfully IAC between May 2014 and May 2019 were retrospectively reviewed for clinical outcomes. Ocular survival and progression-free survival were used to examine the efficacy of salvage IAC. The factors influencing clinical outcomes were determined using univariate and multivariate analyses.ResultsThe salvage IAC was delivered at mean 9.4 months (median 7, range 2.1-38.3 months) following the last cycle of initial IAC. 86.5% (64/73) eyes relapsed 16 months after the initial IAC. After the salvage IAC, 57 eyes (78.1%) were salvaged, and no further-line therapies were required for 36 eyes (49.3%). The 2-year Kaplan-Meier ocular survival and progression-free survival estimates after salvage IAC were 66.4% (95% CI, 31.5-42.1%) and 38.2% (95% CI, 17.8-28.8%), respectively. Univariate and multivariate analyses showed that the ocular survival and progression-free survival after salvage IAC were significantly associated with the history of vitreous seeds (p = 0.02 and p = 0.03, respectively).ConclusionSalvage IAC is effective for the management of recurrent retinoblastoma after the initial successful IAC. Eyes with a history of vitreous seeds in the course of the disease are more likely to relapse and with worse ocular survival. A close follow-up strategy is imperative to treat the recurrent tumour after salvage IAC.
| S-EPMC9581931 | biostudies-literature
Project description:Tumor-derived cell-free DNA (cfDNA) has biomarker potential; therefore, this study aimed to identify cfDNA in the aqueous humor (AH) of retinoblastoma eyes and correlate somatic chromosomal copy-number alterations (SCNA) with clinical outcomes, specifically eye salvage. AH was extracted via paracentesis during intravitreal injection of chemotherapy or enucleation. Shallow whole-genome sequencing was performed using isolated cfDNA to assess for highly recurrent SCNAs in retinoblastoma including gain of 1q, 2p, 6p, loss of 13q, 16q, and focal MYCN amplification. Sixty-three clinical specimens of AH from 29 eyes of 26 patients were evaluated; 13 eyes were enucleated and 16 were salvaged (e.g., saved). The presence of detectable SCNAs was 92% in enucleated eyes versus 38% in salvaged eyes (P = 0.006). Gain of chromosome 6p was the most common SCNA found in 77% of enucleated eyes, compared with 25% of salvaged eyes (P = 0.0092), and associated with a 10-fold increased odds of enucleation (OR, 10; 95% CI, 1.8-55.6). The median amplitude of 6p gain was 1.47 in enucleated versus 1.07 in salvaged eyes (P = 0.001). The presence of AH SCNAs was correlated retrospectively with eye salvage. The probability of ocular salvage was higher in eyes without detectable SCNAs in the AH (P = 0.0028), specifically 6p gain. This is the first study to correlate clinical outcomes with SCNAs in the AH from retinoblastoma eyes, as such these findings indicate that 6p gain in the aqueous humor is a potential prognostic biomarker for poor clinical response to therapy.Implications: The correlation of clinical outcomes and SCNAs in the AH identified in the current study requires prospective studies to validate these finding before SCNAs, like 6p gain, can be used to predict clinical outcomes at diagnosis. Mol Cancer Res; 16(11); 1701-12. ©2018 AACR.
| S-EPMC6214755 | biostudies-literature
Project description:This case series highlights the novel use of intravitreal melphalan for nonvitreous retinoblastoma. It assesses the efficacy and toxicity of intravitreal melphalan for nonvitreous retinoblastoma.This observational small case series investigates three patients treated with intravitreal melphalan for nonvitreous retinoblastoma that was refractory to multiple-course ophthalmic artery chemosurgery. Patients' demographics, response to treatment, and toxicity of treatment as clinically evaluated are measured by electroretinogram.Three eyes of three patients received a median of 7 weekly intravitreal melphalan injections (30 ?g/0.07 cc) for persistent retinal or subretinal tumors refractory to treatment with multiple-course ophthalmic artery chemosurgery.Eyes remain tumor free at a median of 14-month follow-up. One eye was enucleated because of a vitreous hemorrhage that obscured fundus details. One eye had extinguished electroretinogram recordings before injections and two eyes had a decrease in electroretinogram responses over the intravitreal treatment course. The eye with subretinal seeding demonstrated marked retinopathy by ophthalmoscopy and fluorescein angiography and one eye was enucleated because of the development of a vitreous hemorrhage.This small case series highlights that nonvitreous disease that is, refractory or persistent despite previous ophthalmic artery chemosurgery can regress with intravitreal melphalan. However, this treatment may result in retinal toxicity.
| S-EPMC4917462 | biostudies-literature
Project description:Purpose To evaluate topotecan-based therapy for advanced intraocular retinoblastoma. Patients and Methods Twenty-seven patients with bilateral retinoblastoma (male patients, n = 14; median age, 8.4 months) received chemotherapy: two courses of topotecan plus vincristine followed by three alternating administrations of carboplatin and vincristine for two courses and topotecan plus vincristine for one course, with optional periocular carboplatin. Focal therapy was applied after cycle 2. Event-free survival was defined as avoidance of external-beam radiotherapy (EBRT) and enucleation. Results Of 54 eyes, 42 were Reese-Ellsworth group IV to V, and 37 were International Classification of Retinoblastoma group C to E. Eleven eyes were enucleated: one at diagnosis, nine with progressive disease including three eyes treated with EBRT, and one that developed neovascular glaucoma after completion of therapy. One patient was removed from protocol with prolonged infection in course 1; 26 patients (51 eyes) were analyzed. At 10 years, cumulative incidence of EBRT was 5.9% (SE ± 3), and event-free survival was 69.2% (SE ± 27.2). Ocular survival for Reese-Ellsworth group IV to V eyes was 77.5% (SE ± 21.2); it was 74.3% (SE ≥ 18.8) for International Classification group C to E eyes. Vision testing (median age, 7 years; range, 5 to 10 years) documented 20/70 vision or better in one eye of 23 patients; 19 (76%) of 25 patients demonstrated 20/40 vision or better in one eye. All patients experienced thrombocytopenia (41 episodes in 275 courses; 15%). There were 29 episodes of febrile neutropenia (10%). Grade 3 diarrhea was present in nine of 27 patients, and one patient had an allergic reaction to carboplatin. All patients are alive at median follow-up 7.4 years (range, 2.7 to 10 years). Conclusion Topotecan combined with vincristine, carboplatin, and aggressive focal therapies is an effective regimen for the treatment of advanced retinoblastoma and results in globe salvage with vision. Toxicities were anticipated and managed with appropriate supportive care.
| S-EPMC5455691 | biostudies-literature
Project description:PurposeThe advances in the treatment of retinoblastoma have enabled salvaging the globe in advanced stages with intra-arterial chemotherapy (IAC). We developed a strategy of alternate application of systemic intravenous chemotherapy (IVC) and IAC (referred to as alternate systemic IVC and IAC; ASIAC) to reduce central nervous metastases during IAC and examined its efficacy and safety in eye globe salvage in this study.Materials and methodsBetween January 2010 and February 2021, 43 eyes of 40 patients received ASIAC treatment for retinoblastoma at the Yonsei Cancer Center, Yonsei University Health System. Their medical records were reviewed retrospectively to evaluate the eye salvage rate (ESR), defined from diagnosis to enucleation. High-risk retinoblastoma was defined as group D or E by the International Classification of Retinoblastoma.ResultsThe study enrolled 38 and five cases of high-risk and low-risk retinoblastoma, respectively. In total, 178 IAC and 410 IVC courses were administered, with a median of 4 (interquartile range [IQR], 3.0 to 5.0) IAC and 9 (IQR, 6.0 to 11) IVC courses per eye, respectively. The 5-year ESR was 60.4%±8.7% for the whole cohort, 100% for low-risk retinoblastoma, and 53.6%±9.8% for high-risk retinoblastoma. Among those diagnosed since 2015, the 5-year ESR for high-risk retinoblastoma was 63.5%±14.0%. Fifteen eyes underwent enucleation; no viable tumor was found in three enucleated eyes. There were no deaths in this cohort.ConclusionPrimary IAC-IVC (i.e., ASIAC) for patients with retinoblastoma was tolerable and effective in salvaging the eye and maintaining survival.
| S-EPMC9873333 | biostudies-literature
Project description:ImportancePredictions of visual outcomes are useful in clinical and family decisions regarding treatment for retinoblastoma. Very little has been published on the association of post-treatment visual acuity with pre-treatment electroretinography (ERG), which can be performed on infants too young to reliably quantify visual acuity.ObjectiveTo report associations of pre-treatment ERG with post-treatment visual acuity in eyes with advanced retinoblastoma treated with ophthalmic artery chemosurgery (OAC).DesignRetrospective case-control study of eyes treated from 2006 through 2017, with mean follow-up of 51 months (range 2.3-150 months).SettingSingle large academic center.ParticipantsGroup D and E eyes treated with OAC at Memorial Sloan Kettering Cancer Center with recorded visual acuity and ERG (30Hz flicker).Main outcome and measureSnellen visual acuity (uncorrected) compared to initial 30Hz flicker ERG.ResultsThis study included 157 Group D and E eyes. Results of the Jonckheere-Terpstra test for trend were statistically significant and indicated that eyes with lower pre-treatment ERG readings tended to have more visual impairment post-treatment. Among eyes with initial ERG 75+ μV, 11 of 32 eyes (34%) had visual acuity 20/40 or better. Among eyes with ERG 0 μV, 44 of 46 (96%) had visual acuity of 20/200 or worse.Conclusions and relevanceEyes with advanced intraocular retinoblastoma treated with OAC can achieve excellent visual acuity, but poor ERG at initial visit is associated with poor visual acuity after treatment in the majority of eyes. Expectations regarding visual potential may influence decisions about treatment.
| S-EPMC6334963 | biostudies-literature
Project description:To report retinal function outcomes after ophthalmic artery chemosurgery (OAC) for advanced retinoblastoma (RB) in eyes with minimal pretreatment retinal function.For 72 advanced RB eyes with baseline electroretinograms (ERGs) indistinguishable from noise ('extinguished') or flicker ERG amplitudes <25?µV ('poor'), ERGs were obtained before OAC and at 3?months, 1?year and 2?years after OAC. Presence of baseline retinal detachments (RDs) and their subsequent resolution or persistence was also noted.At 3?months, 1?year and 2?years post-OAC, 'extinguished' eyes showed 9/15, 4/11 and 2/6 detectable ERGs, respectively, and 'poor' eyes showed 19/55, 14/30 and 8/18 ERGs exceeding 25??V, respectively. Correlations between baseline and post-OAC ERGs were poor; however, good correlation (R2) existed between ERGs post-OAC at 3?months and 1?year (0.749), at 3?months and 2?years (0.773) and at 1?year and 2?years (0.771). Overall, 49/70 eyes presented with RD; 29 RDs resolved 3?months post-OAC, with an average ERG change of +20.6??V. Eyes with persistent RD had an average ERG change of -2.2??V. No eyes underwent ?25??V change without RD resolution.Minimal baseline ERGs do not preclude significant recovery of retinal function after OAC. Good correlation exists between ERG outcomes at 3?months and those at subsequent follow-ups, suggesting that ERG amplitudes at 3-month post-OAC can prognosticate longer term retinal function, and that improvement is durable. For eyes presenting with RD, RD resolution is necessary but not sufficient for significant (?25??V) increases in ERG amplitudes.
| S-EPMC5637273 | biostudies-literature
Project description:AimTo introduce a simple iris hook assisted phacoemulsification (PE) procedure and evaluate the safety and efficacy of it in completely vitrectomized eyes.MethodsA single centre study which included 65 previously completely vitrectomized eyes of 62 patients who underwent cataract surgery. Patients were randomly divided into 3 groups. Patients received PE, and intraocular lens (IOL) implantation with the assistance of iris hook (Synergeties™) as group A (25 eyes); patients who received PE assisted with a 25G pars plana irrigation as group B (20 eyes), and patients who received PE performed without the help of any instrument as group C (20 eyes). Main outcome measures were surgery duration, Ultrasound (U/S) total time, endothelial cell density (ECD), cumulative dissipated energy (CDE) and complications of the procedures.ResultsWith the help of iris hook, the patients in group A had the lowest ECD loss rate (0.07±0.03, 0.09±0.03, and 0.10±0.03, P<0.05), shortest CDE (12.2±4.1, 15.8±6.0, and 16.0±6.0, P<0.05) and U/S total time (36.6±13.0s, 46.3±16.4s, and 47.6±16.1s, P<0.05), and minimal incidence of complications. The longest surgery duration was in group B (19.4±1.6min) and maximum complications rate in group C (20% miosis, 10% posterior capsular tears, 5% zonular dialysis, 5% cystoid macular edema). While best-corrected visual acuity (BCVA), intraocular pressure (IOP) and ECD did not show a significant difference among the three groups.ConclusionWithout prolonged surgery duration, the iris hook assistant method can minimize heat generation during surgery and incidence of complications, which transfer the challenged PE in vitrectomized eyes into a regular surgery. It does not need any change in the hydrodynamic parameters and in the bag PE technique, easy to operate even for junior surgeons.
| S-EPMC8569570 | biostudies-literature
Project description:Advantages of a Next Generation Sequencing targeted approach for the molecular diagnosis of Retinoblastoma.
| PRJEB9050 | ENA