Characteristics of patients with recurrent retinoblastoma: a survival analysis.

BACKGROUND: Management guidelines and corresponding survival data for patients with recurrent retinoblastoma (RB) are lacking. This study aimed to summarize the clinical characteristics of patients with recurrent RB and analyze their survival outcomes. METHODS: We retrospectively analyzed 68 patients with recurrent RB who underwent treatment in our institution from January 2016 to December 2020. Patients were grouped according to location of recurrence: intraocular, orbital, and distant metastasis. RESULTS: The male:female ratio was 1.3:1 and the median age at recurrence was 37.5 months (range, 30.3-62.8). The number of patients in the intraocular recurrence, orbital recurrence, and metastasis groups was 13 (19.1%), 23 (33.8%), and 32 (47.1%), respectively. Thirty patients died, 36 were alive at last follow-up, and two were lost to follow-up. Eye enucleation was performed in 94.1% of patients. Five-year overall survival in patients with intraocular recurrence, orbital recurrence, and metastasis was 84.6%, 69.6%, and 31.3%, respectively (P = 0.001). Most deaths occurred within 2 years of recurrence. Presence of high-risk pathological factors, central nervous system invasion, and absence of combination therapy were independent predictors of worse 5-year overall survival. CONCLUSION: The rate of eye preservation in survivors of recurrent RB was very low. Although 5-year overall survival in patients who underwent treatment for intraocular and orbital recurrence was high, it was low in those with metastasis. RB patients may need lifelong follow-up for recurrence and secondary malignancy.

Strabismus management in retinoblastoma survivors.

PURPOSE: To report the result of strabismus surgery in eye-salvaged retinoblastoma (Rb) patients. METHODS: A retrospective case series including 18 patients with Rb and strabismus who underwent strabismus surgery after completing tumor treatment by a single pediatric ophthalmologist. RESULTS: A total of 18 patients (10 females and 8 males) were included with a mean age of 13.3 ± 3.0 (range, 2-39) months at the time tumor presentation and 6.0 ± 1.5 (range, 4-9) years at the time of strabismus surgery. Ten (56%) patients had unilateral and 8(44%) had bilateral involvement and the most common worse eye tumor's group was D (n = 11), C (n = 4), B (n = 2) and E (n = 1). Macula was involved by the tumors in 12 (67%) patients. The tumors were managed by intravenous chemotherapy (n = 8, 47%), intra-arterial chemotherapy (n = 7, 41%) and both (n = 3, 17%). After complete treatment, the average time to strabismus surgery was 29.9 ± 20.5 (range, 12-84) months. Except for one, visual acuity was equal or less than 1.0 logMAR (≤ 20/200) in the affected eye. Seven (39%) patients had exotropia, 11(61%) had esotropia (P = 0.346) and vertical deviation was found in 8 (48%) cases. The angle of deviation was 42.0 ± 10.4 (range, 30-60) prism diopter (PD) for esotropic and 35.7 ± 7.9 (range, 25-50) PD for exotropic patients (P = 0.32) that after surgery significantly decreased to 8.5 ± 5.3 PD in esotropic cases and 5.9 ± 6.7 PD in exotropic cases (P < 0.001). The mean follow-up after surgery was 15.2 ± 2.0 (range, 10-24) months, in which, 3 (17%) patients needed a second surgery. CONCLUSION: Strabismus surgery in treated Rb is safe and results of the surgeries are acceptable and close to the general population. There was not associated with tumor recurrence or metastasis.

Three-Dimensional Ultrasound Imaging of Retinoblastoma and Its Correlation With Pathology.

BACKGROUND AND OBJECTIVE: Monitoring the response of retinoblastoma to globe-salvaging therapies is based on subjective assessments of changes determined by fundoscopy, ultrasound, and optical coherence tomography. Advances in organ-preserving therapies have increased the need for objective, quantitative estimates of tumor response to treatment. Primary tumor volume is a metric that can be objectively determined as a surrogate measure of treatment response. PATIENTS AND METHODS: We evaluated the correlation of objective, quantitative estimates of tumor volume made with two-dimensional (2D) and three-dimensional (3D) ultrasound with gold standard pathological tumor volumes derived by analysis of enucleation specimens. RESULTS: Twelve eyes in 12 patients undergoing primary enucleation were evaluated by 2D and 3D ultrasound during ophthalmic examination under anesthesia prior to enucleation. 2D- and 3D-ultra-sound measurements of tumor volume were both strongly correlated with pathological estimates of tumor volume (r = 0.69, P = 0.018; and r = 0.66, P = 0.027, respectively). CONCLUSIONS: 2D- and 3D-ultrasound measurements of retinoblastoma primary tumor volume are highly correlated with pathological estimates. 3D measurements are easy to perform with volumetric probes and consider the irregular morphology of the tumor. Further study should be undertaken to evaluate the performance of these metrics as surrogate markers of tumor response to treatment. [Ophthalmic Surg Lasers Imaging Retina 2024;55:136-140.].

Does vitreous haemorrhage and calcification lead to increased risk of enucleation in advanced retinoblastoma?

PURPOSE: To explore whether varying degrees of vitreous haemorrhage (VH) and calcification act as risk factors for enucleation in patients with advanced retinoblastoma (RB). METHODS: Advanced RB was defined by the international classification of RB (Philadelphia version). Basic information for retinoblastoma patients diagnosed as groups D and E in our hospital between January 2017 and June 2022 was reviewed by logistics regression models. Additionally, a correlation analysis was performed, excluding variables with a VIF (variance inflation factor) >10 from the multivariate analysis. RESULTS: A total of 223 eyes diagnosed with RB were included in assessing VH and calcification; of these, 101 (45.3%) eyes experienced VH, and 182 (76.2%) eyes were found to have calcification within the tumour through computed tomography (CT) or B-scan ultrasonography. Ninety-two eyes (41.3%) were enucleated, of which 67 (72.8%) had VH and 68 (73.9%) calcification, both of which were significantly relevant to enucleation (p < 0.001*). Other clinical risk factors, such as corneal edema, anterior chamber haemorrhage, high intraocular pressure during treatment and iris neovascularization, correlated significantly with enucleation (p < 0.001*). Multivariate analysis included IIRC (intraocular international retinoblastoma classification), VH, calcification and high intraocular pressure during treatment as independent risk factors for enucleation. CONCLUSIONS: Despite identifying different potential risk factors for RB, there remains significant controversy concerning which patients require enucleation, and the degree of VH varies. Such eyes need to be evaluated carefully, and management with appropriate adjuvant therapy may improve the outcome of these patients.

Rehabilitation of pediatric retinoblastoma patients with ocular prostheses and their subsequent modifications: A 15-year retrospective study.

PURPOSE: Enucleation is a common treatment modality performed for pediatric retinoblastoma patients, and the resultant defects are reconstructed using an ocular prosthesis. The prostheses are modified or replaced periodically, as the child develops due to orbital growth and patient-error. The purpose of this report is to evaluate the replacement frequency of prostheses in the pediatric oncologic population. METHODS: A retrospective review was completed by the two senior research investigators, of patients that had ocular prostheses fabricated following enucleation of their retinoblastoma from 2005 to 2019 (n = 90). Data collected from the medical records of the patient included the pathology, date of surgery, date of prosthesis delivery, and the replacement schedule of the ocular prosthesis. RESULTS: During the 15-year study period, 78 enucleated observations (ocular prosthesis fabricated) were included for analysis. The median age of the patients at the time of delivery of their first ocular prosthesis was calculated to be 2.6 years (range 0.3-18 years). The median time to the first modification of the prosthesis was calculated to be 6 months. The time to modification of the ocular prosthesis was further stratified by age. CONCLUSION: Pediatric patients require modification of their ocular prostheses throughout their growth and development period. Ocular prostheses are reliable prostheses with predictable outcomes. This data is helpful to set an expectation among the patient, parent, and provider.

Mutant-RB1 circulating tumor DNA in the blood of unilateral retinoblastoma patients: What happens during enucleation surgery: A pilot study.

Cell free DNA (cfDNA) and circulating tumor cell free DNA (ctDNA) from blood (plasma) are increasingly being used in oncology for diagnosis, monitoring response, identifying cancer causing mutations and detecting recurrences. Circulating tumor RB1 DNA (ctDNA) is found in the blood (plasma) of retinoblastoma patients at diagnosis before instituting treatment (naïve). We investigated ctDNA in naïve unilateral patients before enucleation and during enucleation (6 patients/ 8 mutations with specimens collected 5-40 minutes from severing the optic nerve) In our cohort, following transection the optic nerve, ctDNA RB1 VAF was measurably lower than pre-enucleation levels within five minutes, 50% less within 15 minutes and 90% less by 40 minutes.

A comparison of high risk pathological features between primary and secondary enucleation for retinoblastoma.

AIM: To compare the risk and pattern of High-Risk Pathologic Features (HRPF) in retinoblastoma between primary and secondary enucleation. METHODS: A retrospective analysis of 121 eyes from 118 patients who underwent enucleation at the King Hussein Cancer Center (KHCC) Amman, Jordan, between November 2009 and January 2020. Demographic information, tumor stage, time from diagnosis-to-enucleation, results of pathology, metastasis, and mortality were retrieved. RESULTS: Patients in the secondary group (49/121 eyes, 40%) were considerably younger at diagnosis (p = 0.0014), had bilateral disease (p = 0.0001), and had less-progressed disease at presentation (p = 0.016) compared to the primary enucleation. Primarily enucleated eyes were more-likely to have massive choroidal invasion (p = 0.0315) and post-laminar optic nerve invasion (p = 0.027), in spite of the finding that the overall prevalence of HRPF was similar between the two groups (35.5 percent vs. 37.5 percent; p = 0.585). The likelihood of anterior chamber invasion, was considerably higher in secondary enucleated eyes (p = 0.013). We evaluated primary and secondary enucleation for each subgroup (D and E) of the International Intraocular Retinoblastoma Classification (IIRC) and found the prevalence of HRPF was comparable (p = 0.58, 1.0, respectively). The difference in time between diagnosis-to-enucleation in secondary enucleation did not predict HRPF (p = 0.50). There was no discernible difference between primary and secondary enucleated eyes in terms of metastasis or survival (p = 0.156 and 0.44, respectively). CONCLUSION: Systemic chemotherapy has the ability to reduce the extent of tumor expansion that has been pathologically identified. Primary and secondary enucleated eyes are comparable in low metastatic risk only when strict examination and management guidelines are followed.

Polyester 5-0 suture for porous implant placement after retinoblastoma enucleation: analysis of 120 sockets.

BACKGROUND: Techniques used to suture the rectus muscle to the implant can influence the implant-related complications which is still a major problem following retinoblastoma enucleation. The goals of this work were to report the efficacy among patients with retinoblastoma who underwent enucleation followed by porous implant placement with the rectus muscles sutured with 5-0 polyester suture. METHODS: This was a retrospective study of consecutive patients with retinoblastoma who underwent primary enucleation and porous implant placement with the rectus muscles tagged and sutured to the implant with polyester 5-0 suture. All the patients were followed up for a minimum of 2 years. The main outcome measure was implant exposure. The secondary efficacy measures were other implant-related complications. RESULTS: Between May 2016 and December 2018, a total of 120 patients (120 eyes) underwent primary enucleation and porous implant placement were included. Postoperatively, 10/120 (8.3%) eyes developed exposure or conjunctival granuloma. Exposure was the most common postoperative complication (7/10, 70.0%). There were no cases of implant extrusion, migration, or infection. CONCLUSIONS: Polyester 5-0 sutures are successful in patients with retinoblastoma who underwent enucleation followed by porous implant placement. Complications are minimal. Polyester 5-0 sutures were not associated with unacceptable complications in this pediatric population.

The survival analysis and feasibility study of Pars Plana Vitrectomy (PPV) in the treatment of retinoblastoma.

PURPOSE: To analyze the safety and efficacy of Pars Plana Vitrectomy (PPV) as a treatment for retinoblastoma patients and to evaluate the feasibility. METHODS AND PATIENTS: We collected 342 eyes who had PPV after systemic chemotherapy in our retrospective study, then analyze the 5-year overall survival and 5-year event-free survival rate, recurrence rate, and metastasis rate. The above data were used to evaluate the feasibility of PPV in the treatment of retinoblastoma. RESULTS: The mean value of follow-up time was 62.9 months from PPV. Of all 342 eyes, 18% eyes underwent enucleation of the eyeball. Excluding Non-PPV related deaths eyes, the 5-year overall survival rates and event-free survival were 95% and 80%; the tumor recurrence rate and metastasis rate were approximately 26% and 1.2%, respectively; the mortality was 3.9%. And the incidence of high-risk pathological factors of enucleated eyes after PPV was 32%. CONCLUSION: Our results suggest that Pars Plana Vitrectomy as a new approach to preserve the eyeball of RB children is feasible, especially for those patients who cannot be completely controlled by systemic chemotherapy or the tumors with vitreous seeds. Although the outcomes in our study are very optimistic, we also recommend an experienced eye surgeon to perform the operation and strictly control the indications for PPV surgery. And enough systemic chemotherapy is very important before and after surgery. LEVEL OF EVIDENCE: Treatment study (Retrospective comparative study), III.

Quantity and duration of exposure to general anesthesia for pediatric patients with retinoblastoma.

PURPOSE: To quantify the duration of anesthesia required for optimal management of retinoblastoma (Rb), stratified by clinical factors. METHODS: The medical records of Rb patients treated at Phoenix Children's Hospital between January 2011 and January 2022 were reviewed retrospectively. Demographic, tumor, and treatment data were collected. Anesthesia time was recorded for procedures requiring general anesthesia, including intra-arterial chemotherapy (IAC), enucleation, brainstem auditory evoked response testing (BAER), port placement, magnetic resonance imaging (MRI), and examination under anesthesia (EUA). Descriptive statistics were used to summarize patient and clinical characteristics. RESULTS: Total anesthesia time was 48,991 minutes for 610 procedures in 43 patients. The median follow-up time was 36 months (range, 12-114 months). Average anesthetic durations per exposure were 274, 152, 81.8, 62.5, 60.7, and 45 minutes for IAC, enucleation, BAER, port placement, MRI, and EUA, respectively. Patients with bilateral Rb underwent a median of 1,659 minutes of total anesthesia, compared with 397 minutes for those with unilateral disease. In patients with unilateral Rb, median total anesthesia time was 2,651, 1681, 312, 397 minutes for International Classification of Retinoblastoma grades B, C, D, and E tumors, respectively. Patients who received IAC as their primary treatment had the highest median anesthesia duration (2,100 minutes), followed by systemic chemo (654 minutes) and enucleation (289 minutes). CONCLUSIONS: Treatment of Rb requires prolonged and repeated exposure to general anesthesia. Future studies are required to determine the potential effects of these childhood anesthetic exposures.

Clinicopathological Correlations in Enucleated Globes of Late-Stage Coats Disease with a Review of the Literature.

BACKGROUND: Coats disease may cause diagnostic dilemma because of its variable clinical presentation that can be suspicious of retinoblastoma. Late sequelae of the disease are blinding with possible enucleation. We demonstrate the main histopathological findings of Coats enucleated eyes with literature review. METHODS: This was a retrospective study of all enucleated globes diagnosed as Coats disease over 30 years and were reviewed by two pathologists. The corresponding demographic data, clinical presentation, pre-operative clinical impression, and indication for enucleation were collected. Descriptive analysis of our own series data was performed. Our findings were then correlated to published data that were collected from 1983 to 2021 from the PUBMED database in English-written language. Shields classification was used as an inclusion criterion for the published reports to be analyzed. RESULTS: We had seven enucleated globes with Coats disease. Mean age at presentation was 3.2 years (range 3 months to 9 years). Male predominance was observed in 6 and all cases were unilateral. Strabismus was the most common initial presentation (57%, n = 4), followed by leukocoria (43%, n = 3). Indication for enucleation was mostly suspected retinoblastoma (57%, n = 4). Four eyes were classified as stage 4, and 2 had advanced stage 5 changes. Histopathologically, subretinal fluid with lipid-laden macrophages was seen in all cases, the anterior chamber was shallow in 5/7 with angle neovascularization in 2/7. Telangiectatic vessels were clearly observed in 4/7. CONCLUSION: Coats disease is a potentially visually disabling disease that is mostly unilateral in 95%, has male predominance of 81%, and wide age range with a mean of 17 years. In Saudi Arabia, the disease seems to present at younger age, tends to be more advanced, and may be indistinguishable from retinoblastoma at the time of diagnosis. Shields staging of Coats is highly recommended to be followed clinically to unify the pathways for treatment and to correlate the concluded outcomes.

Understanding the Experiences of Living With an Artificial Eye in Children With Retinoblastoma-Perspectives of Children and Their Parents.

Background: Retinoblastoma is a rare form of pediatric eye cancer for which enucleation is a common treatment modality. There is an increasing focus upon the impact of enucleation on children and families. This study aimed to explore the experiences of children and their families following enucleation to consider the barriers that may be encountered when adjusting to living with an artificial eye and identifying the support services and strategies used to address these barriers. Methods: Using a descriptive qualitative approach, interviews were conducted with 12 parents and seven children and thematic analysis was used to identify four themes representing the perspective of parents and children. Results: Parent themes identified were (a) entry into the world of retinoblastoma; (b) the importance of specialist support; (c) a family learning to cope; and (d) navigating school. The perspectives of children were (a) the importance of preparation and play; (b) positive reinforcement and hospital support; (c) support and openness at home; and (d) the importance of good school planning. Two overarching themes related to parent and child adaptation were also identified. Discussion: The study findings reveal that the process of adapting to living with an artificial eye extended to influencing decisions around parenting, learning to advocate for their child, and supporting children through school and peer relationships. For children, the importance of ongoing support and information was vital to enhance understanding, adaptation, and development of independence. Overall, the study suggests that children and families undergoing enucleation need continuing, individualized, and specialist support.

Correlation between clinical presentations, radiological findings and high risk histopathological features of primary enucleated eyes with advanced retinoblastoma at Queen Sirikit National Institute of Child Health: 5 years result.

PURPOSE: To evaluate the correlation between clinical presentations, radiological findings and high risk histopathological features of primary enucleated eyes in patients with advanced retinoblastoma at a tertiary child hospital in Thailand. MATERIALS AND METHODS: We retrospectively reviewed the medical records of patients who were treated with primary enucleation of tumor eyes between 2015-2020. Demographic data, radiological assessment, and histopathological findings were collected. The association between clinical presentations and high-risk histopathological features in the primary enucleated eyes were evaluated. The radiological findings, which showed characteristic of high risk features, were compared with the histopathological findings. RESULTS: Thirty-three enucleated eyes were enrolled in this study. The mean age at diagnosis was 23.12 months. Most patients had unilateral group E retinoblastoma, with no difference in sex and laterality of the eye. Leukocoria was the most common presentation, followed by proptosis and uveitis. Older age at presentation were statistically associated with post laminar cribrosa optic nerve invasion (P-value 0.0027) and high-risk histopathological features in enucleated eyes (P-value 0.0032). Clinical presentations with proptosis were statistically associated with post laminar cribrosa optic nerve invasion, while leukocoria and uveitis were statistically associated with anterior segment invasion. Unifocal intraocular mass with necrosis was the most common histopathological finding. High-risk features were found in 45% of primary enucleated eye. The sensitivity and specificity of magnetic resonance imaging (MRI) for detected optic nerve invasion in retinoblastoma patients were 75% and 54%, respectively. CONCLUSION: Patients with unilateral retinoblastoma who presented with older age related to high-risk features after enucleation. Ophthalmic examination with slitlamp is the best way for detection of anterior segment invasion. Choroidal invasion was unable to predict with clinical presentation. MRI was the better imaging for detection of post laminar cribrosa optic nerve invasion.

Aqueous humor as a surrogate biomarker for retinoblastoma tumor tissue.

PURPOSE: To demonstrate the feasibility of identifying a germline RB1 pathogenic variant in retinoblastoma (RB) from an aqueous humor (AH) sample. METHODS: In this pilot case series, peripheral blood, fresh tumor tissue, and AH were obtained from 3 eyes of 3 RB patients who underwent enucleation at a tertiary eye care institute. After isolation of the cell-free DNA (cfDNA), sequence analysis of the RB1 core promoter and of exons 1 through 27, including nearby flanking intronic regions, was performed using a custom targeted hybridization protocol, followed by high-throughput sequencing. RESULTS: The study cohort included 3 enucleated eyes with advanced RB (group E [n = 2], group D [n = 1]). In case 1, deletion of the RB1 promoter to exon 23 (delP->23) on both alleles was identified from tumor as well as AH samples and absent in the blood sample, indicative of absence of a germline RB1 pathogenic variant. In case 2, two heterozygous RB1 nonsense variants, c.610G>T p.(Glu204Ter) and c.751C>T p.(Arg251Ter), were identified in tumor and AH samples (allele frequency of 49% and 45%, resp.) and were absent in the blood sample, indicative of absence of a germline RB1 pathogenic variant. In case 3, a heterozygous c.2326-14T>A substitution on allele 1 and loss of heterozygosity on allele 2 were identified in the tumor and AH (allele frequency of 97%), with the same heterozygous mutation in the blood sample, indicating presence of a germline RB1 pathogenic variant. CONCLUSIONS: The pathogenic RB1 variant results from AH in all 3 eyes were concordant with direct tumor DNA sampling, suggesting that AH can serve as a surrogate for tumor tissue. Because the AH can be accessed during treatment, specific testing can be performed even in the absence of enucleation.

Pediatric Cataract Surgery Following Treatment for Retinoblastoma: A Case Series and Systematic Review.

PURPOSE: To determine the visual and refractive outcomes and the ocular and systemic complications of cataract surgery in eyes treated for retinoblastoma. DESIGN: Retrospective consecutive case series and systematic review. METHODS: Children <18 years of age with retinoblastoma who underwent surgery for secondary cataract between 2000 and 2020 were reviewed. Medline (OVID), Embase, Web of Science, and the Cochrane database were searched from inception to August 2020. RESULTS: A total of 15 eyes of 15 children were included. The mean age at retinoblastoma diagnosis was 12 months (median, 14; interquartile range [IQR], 4-19). Cataract developed at a mean age of 39 months (median, 31; IQR, 20-52), secondary to multiple treatments (n = 7), pars-plana vitrectomy (n = 3), external-beam radiotherapy (n = 2), laser (n = 2), and retinal detachment (n = 1). The mean preoperative quiescent interval was 44 months (median, 28; IQR, 15-64). Primary intraocular lens implantation was performed in 93%, posterior capsulotomy in 40%, and anterior vitrectomy in 33% of participants. Postoperatively, 100% had improved fundus visibility and 73% had improved vision. Complications included visual axis opacification (11 of 15), capsular phimosis (5 of 15), and zonulopathy (3 of 15). No patient developed intraocular recurrence, extraocular extension, or metastasis at a mean of 76 months (median, 78; IQR, 29-128) follow-up. The systematic review identified 852 studies, with 18 meeting inclusion criteria. Across all studies (n = 220 children), intraocular recurrence occurred in 6%, globe salvage in 91%, and extraocular extension and metastasis in <1%. CONCLUSIONS: Modern retinoblastoma therapies, including intravitreal chemotherapy and vitrectomy, cause secondary cataract. Following cataract surgery, intraocular recurrence risk is low and extraocular spread is rare. Although surgery improves tumor visualization, visual prognosis may be limited by several factors. Challenges include biometry limitations and a high incidence of zonulopathy.

Outcomes of enucleation and porous polyethylene orbital implant insertion in patients with paediatric retinoblastoma: a long-term follow-up study.

BACKGROUND/AIMS: To report the long-term outcomes of enucleation and insertion of porous polyethylene (PP) orbital implant according to the evolving surgical techniques and implant in patients with paediatric retinoblastoma . METHODS: Patients with paediatric retinoblastoma who underwent enucleation and PP implant insertion from December 1998 to December 2014 were retrospectively reviewed and divided into four groups: group A, classic enucleation +PP implant; group B, enucleation +PP implant +anterior closure of the posterior Tenon's (ACPT) capsule; group C, enucleation +PP implant +free orbital fat graft +ACPT and group D, enucleation +smooth surface tunnel PP implant +ACPT. Survival analysis of implant exposure and eyelid malpositions was performed. RESULTS: One hundred and ninety-eight eyes of 196 patients were included. The median follow-up period was 13.0 years (range, 5.0-21.1). A 20 mm implant was inserted for 149 eyes (75.3%). The 10-year exposure-free survival probabilities were 44.6% in group A, 96.4% in group B, 97.4% in group C and 97.7% in group D. ACPT was associated with significant reduction in implant exposure (p<0.001). The most common eyelid malposition was upper eyelid ptosis (24.2%). The eyelid malposition-free survival probability did not differ among the four groups. However, the insertion of a 20 mm implant was associated with significant reduction in upper eyelid ptosis and lower eyelid entropion (p=0.004 and 0.038, respectively). CONCLUSIONS: The long-term postenucleation implant exposure was rare after PP implant insertion and ACPT, even with a 20 mm-diameter implant. A larger implant can be beneficial in long-term prevention of eyelid malposition.

Primary laser therapy as monotherapy for discrete retinoblastoma.

BACKGROUND/AIM: Laser photocoagulation is less invasive than chemotherapy (systemic, intra-arterial or periocular) and brachytherapy. We studied the safety and efficacy of laser as primary monotherapy for discrete retinoblastoma with well-defined borders and attached retina. METHODS: A single-institution retrospective non-comparative review (2004-2018) of discrete retinoblastoma tumours managed with primary laser (532 or 810 nm wavelength, 0.5-1 s duration and power titrated until desired tumour whitening). Efficacy was evaluated by tumour long-term stability avoiding non-laser therapies. Safety was evaluated by frequency of laser-related complications and uncontrollable tumour progression. RESULTS: Eligible were 112 tumours in 55 eyes of 44 patients. Laser monotherapy (median 2 sessions) achieved initial remission in 95/112 (85%) tumour. Initial encircling only laser photocoagulation was associated with tumour progression (9/11, one tumour had vitreous seeding) compared with direct or combined photocoagulation techniques (0/94 and 0/7 tumours, respectively, p<0.001). Direct laser had no vitreous seeding, haemorrhage or injury to vital structures. Tumour recurrences developed in 52/112 (46%) tumour but repeat laser achieved long-term stability, except five tumour recurrences that required invasive therapy. Receiver operating characteristic analysis identified threshold largest basal diameter of 3 disc diameters (DD) for successful laser monotherapy, where 92/106 (87%) of tumours ≤3 DD and 0/6>3 DD achieved long-term stability with laser monotherapy (p<0.001). Overall, 35/55 (64%) eyes and 24/44 (55%) patients achieved long-term stability with laser monotherapy. No eye was enucleated for uncontrollable tumour progression. CONCLUSIONS: Discrete retinoblastoma ≤3 DD can be effectively and safely managed with laser monotherapy, sparing a significant proportion of patients/eyes from more invasive therapies.