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AimsThe goal of this study is to identify the pathological findings and expression of metabolic markers (GLUT-1, PDK-1 and PGC1α) in the tumour microenvironment of both primary and chemoreduced retinoblastoma (Rb) and to correlate with clinicopathological parameters and patient outcome.Methods81 prospective cases were included, in which 53 cases underwent primary enucleation and 28 cases received chemotherapy before enucleation. Immunohistochemistry, qRT-PCR and western blotting were performed to evaluate the expression pattern of metabolic markers in primary and chemoreduced Rb.ResultsTumour microenvironment and histopathological findings were different for both primary and chemoreduced Rb. Increased immunohistochemical expression of GLUT-1, PDK-1 and PGC1α was found in primary Rb as compared with chemoreduced Rb. mRNA expression was also found to be upregulated in primary Rb compared with chemoreduced. On univariate analysis, the presence of more than one histopathological high-risk factor (HRFs>1) and PDK-1 immunoexpression was statistically significant with overall survival. On prognostication in primary and chemoreduced cases with hypoxia, we found increased HR in cases with retrolaminar ON invasion, presence of more than one HRF, and presence of PDK-1 and PGC-1α immunoexpression.ConclusionsThis is the first of its kind study predicting a relevant role of the metabolic markers in primary and chemoreduced Rb with prognostic significance. Differential expression of these markers in both groups of Rb is a novel finding and might be an interesting and beneficial target for the management of Rb patients.

Purpose Intraocular retinoblastoma treatments often combine chemotherapy and focal treatments. A first prospective protocol of conservative treatments in our institution showed the efficacy of the use of two courses of chemoreduction with etoposide and carboplatin, followed by chemothermotherapy using carboplatin as a single agent and diode laser. In order to decrease the possible long-term toxicity of chemotherapy due to etoposide, a randomized neoadjuvant phase II protocol was conducted using vincristine–carboplatin vs etoposide–carboplatin. Patients and methods The study was proposed when initial tumor characteristics did not allow front-line local treatments. Patients included in this phase II noncomparative randomized study of neoadjuvant chemotherapy received vincristin–carboplatin (new arm) vs etoposide–carboplatin (our reference arm). They were subsequently treated by local treatments and chemothermotherapy. Primary end point was the need for secondary enucleation or external beam radiotherapy (EBRT) not exceeding 40% at 2 years. Results A total of 65 eyes in 55 children were included in the study (May 2004 to August 2009). Of these, 32 eyes (27 children) were treated in the arm etoposide–carboplatin and 33 eyes (28 children) in the arm vincristin–carboplatin. At 2 years after treatment, 23/33 (69.7%) eyes were treated and salvaged without EBRT or enucleation in the arm vincristin–carboplatin and 26/32 (81.2%) in the arm etoposide–carboplatin. Conclusion Even if the two treatment arms could be considered as sufficiently active according to the study decision rules, neoadjuvant chemotherapy by two cycles of vincristine–carboplatin followed by chemothermotherapy appear to offer less optimal local control than the etoposide–carboplatin combination.

Introduction This study presents the outcomes of high‐risk group retinoblastoma (Rb) patients enrolled in the AIEOP RTB 012 Protocol. Methods Patients with intraocular unilateral Rb classified as group C/D according to “International Intraocular Retinoblastoma Classification” (IIRC), as well as those with bilateral Rb with at least one eye group C/D/E‐IIRC, were treated with four cycles of carboplatin/etoposide combined with focal treatments. The primary endpoint was to evaluate ocular event‐free survival (EFS) and overall survival (OS), where events were defined as the need for radiotherapy, eye enucleation, and second‐line treatment. Visual acuity (VA) was assessed in all available cases. Results Between February 2012 and September 2017, 60 patients were enrolled (88 eyes), 32 unilateral Rb, 28 bilateral. Twelve eyes were classified as group A/B, 15 group C, 40 group D, and 21 group E. At a median follow‐up of 8.71 years, 42/88 eyes were preserved. The 2‐ and 5‐year eye EFS rates were 31.1% (95% CI: 24.4–44.0) and 29.5% (95% CI: 20.4–39.2), respectively. The corresponding 2‐ and 5‐year eye OS rates were 63.3% (95% CI: 52.7–72.7) and 48.9% (95% CI: 38.1–58.8). Ocular survival significantly differed across IIRC groups, with 2‐ and 5‐year survival rates of 100% for group A/B, 86.7% and 73.3% for group C, 65% and 42.5% for group D, and 23.8% and 14.3% for group E, respectively (p < 0.05). No severe toxicity was reported. Among 28 bilateral Rb patients, 12 had VA of at least 20/30 according to the Snellen chart. Conclusion The AIEOP RTB 012 protocol has proven to be both safe and effective, ensuring a favorable final VA.

Purpose To evaluate the effectiveness and safety of selective ophthalmic arterial injection (SOAI) for retinoblastoma utilizing a microballoon catheter system with an M chamber. Study design Retrospective analysis. Methods and patients This study was sanctioned by theNational Cancer Center Hospital’ Independent Ethics Committee. The surgeon was a general interventional radiologist. After confirming that the distal internal carotid artery was not delineated by balloon occlusion and the ophthalmic artery was visualized using digital subtraction angiography, melphalan was manually administered. Notably, in cases presenting bilateral retinoblastoma, both eyes received treatment in a singular, low-dose procedure. Between July 2015 and December 2021, 125 patients with retinoblastoma (68 boys and 57 girls) underwent SOAI at our facility. The average age at initial treatment was 19.3 months. The study covered 250 procedures, with patients undergoing an average of 3.7 procedures. Results The success rate of the procedure was 99.2%, with a mean procedure duration of 18.3 min. Two distinct technical failures were recorded: one attributed to an internal carotid artery having a wide lumen and the other due to the ophthalmic artery remaining undetected on angiography post-balloon occlusion of the internal carotid artery. Adverse events were minimal but included bronchospasm post-procedure and severe orbital inflammation in 0.8% and 0.4% of cases, respectively. Conclusion SOAI using the microballoon catheter with the M chamber is a feasible and safe procedure for the treatment of retinoblastoma. The success rate was 99.2%. This system can be recommended as intra-arterial chemotherapy for retinoblastoma.

At present, the few photothermal/chemotherapy studies about retinoblastoma that have been reported are mainly restricted to ectopic models involving subcutaneous implantation. However, eyeball is unique physiological structure, the blood-retina barrier (BRB) hinders the absorption of drug molecules through the systemic route. Moreover, the abundant blood circulation in the fundus accelerates drug metabolism. To uphold the required drug concentration, patients must undergo frequent chemotherapy sessions. To address these challenges above, we need to develop a secure and effective drug delivery system (FA-PEG-PDA-DOX) for the fundus. We offered superior therapeutic efficacy with minimal or no side effects and successfully established orthotopic mouse models. We evaluated cellular uptake performance and targeting efficiency of FA-PEG-PDA-DOX nanosystem and assessed its synergistic antitumor effects in vitro and vivo. Biodistribution assessments were performed to determine the retention time and targeting efficiency of the NPs in vivo. Additionally, safety assessments were conducted. Cell endocytosis rates of the FA-PEG-PDA-DOX+Laser group became 5.23 times that of the DOX group and 2.28 times that of FA-PEG-PDA-DOX group without irradiation. The fluorescence signal of FA-PEG-PDA-DOX persisted for more than 120 hours at the tumor site. The number of tumor cells (17.2%) in the proliferative cycle decreased by 61.6% in the photothermal-chemotherapy group, in contrast to that of the saline control group (78.8%). FA-PEG-PDA-DOX nanoparticles(NPs) exhibited favorable biosafety and high biocompatibility. The dual functional targeted nanosystem, with the effects of DOX and mild-temperature elevation by irradiation, resulted in precise chemo/photothermal therapy in nude mice model.

This study aimed to investigate the benefits of using three-dimensional (3D)-printed hip joint fixators after intra-arterial chemotherapy (IAC) by inguinal femoral artery puncture in children with retinoblastoma. Overall, 79 cases of retinoblastoma who had undergone IAC through the femoral artery were selected and divided into an observation group of 50 cases and an intervention group of 29 cases according to the hemostasis method employed. The patients in the observation group were treated with sandbags for hemostasis, while those in the intervention group were given 3D-printed hip joint fixators to help immobilize the hips and sandbags. We used the Face, Legs, Activity, Cry and Consolability scale (FLACC), the Wong–Baker Facial Expression Pain Scale, and self-made questionnaires to evaluate demographics, clinical characteristics, pain, complications, satisfaction, and other indicators of the two groups. There were no significant differences in general data, such as age, gender, height, weight, manual compression time, diseased eye, tumor stage, platelet count, puncture times, pain distribution, and total score, between the groups. There was a positive correlation between FLACC pain and the total Wong–Baker pain score ( r  = 0.599, p  < 0.001). During the 2 h of sandbag compression, sandbags were dislodged in the observation group as many as ten times, which was significantly higher than that in the intervention group (up to four times; p  < 0.001). This was correlated with a very high score of satisfaction (92.34 ± 19.96 out of 100). The 3D-printed hip fixator is easy to operate, has a low incidence of complications, and saves time and effort. It effectively reduces the incidence of sandbags falling off after IAC in children with retinoblastoma and does not increase the patient’s pain. It is a method that could improve hemostasis in young children undergoing IAC by inguinal femoral artery.

PurposeTo assess the effectiveness of intra-arterial chemotherapy (IAC), intravenous chemotherapy (IVC), and their combination in the management of group D retinoblastoma.MethodsThis was a retrospective, interventional, comparative, and non-randomized clinical study, including all eyes with primarily treated group D retinoblastoma managed in a single institution from February 2010 to July 2016. Patient demographics, treatment modality (primary enucleation, intravenous chemotherapy, intra-arterial chemotherapy alone or intravenous, and intra-arterial chemotherapy), additional need for consolidation treatments or intravitreal melphalan (IVM) injections, and follow-up time were recorded. The main outcome measure was ocular survival rate after various treatment modalities.ResultsOf 87 eyes of 83 consecutive cases, 9 eyes (10.3%) were primarily enucleated, 37 (42.6%) eyes received IVC, 30 (34.5%) eyes had IAC, and 11 (12.6%) eyes were treated with IVC followed by IAC. After a mean 81.1 ± 75.2 weeks of follow-up, enucleation rates were 56.8% in the IVC group, 23.3% in the IAC group, and 36.6% in the IVC + IAC group (p = 0.021). There was no significant difference of ocular survival estimates among these 3 groups at 2 or 5 years (p = 0.998, p = 0.986). With logistic regression analysis, age; gender; disease laterality; administration of IVM, TTT, or cryotherapy; mean dose of IVM; follow-up time; and number of IAC or IVC cycles were not significantly related to the enucleation rate (p > 0.05 for all variables).ConclusionsOur experience showed that in group D patients, primary IAC achieved ocular survival in 76.7% of eyes, and less need for local consolidation treatments, whereas following primary IVC ocular survival was 43.2%.

Background and purpose Retinoblastoma (RB) is a rare malignancy affecting the pediatric population. Intravenous chemotherapy is the longstanding delivery method, although intra-arterial (IA) chemotherapy is gaining popularity given the reduced side effects compared with systemic chemotherapy administration. Given the sensitivity of the target organ, patient age, and secondary tumor susceptibility, a premium has been placed on minimizing procedural related radiation exposure. Materials and methods To reduce patient x-ray dose during the IA infusion procedure, customized surgical methods and fluoroscopic techniques were employed. The routine fluoroscopic settings were changed from the standard 7.5 pulses/s and dose level to the detector of 36 nGy/pulse, to a pulse rate of 4 pulses/s and detector dose to 23 nGy/pulse. The angiographic dose indicators (reference point air kerma (Ka) and fluoroscopy time) for a cohort of 10 consecutive patients (12 eyes, 30 infusions) were analyzed. An additional four cases (five eyes, five infusions) were analyzed using dosimeters placed at anatomic locations to reflect scalp, eye, and thyroid dose. Results The mean Ka per treated eye was 20.1±11.9 mGy with a mean fluoroscopic time of 8.5±4.6 min. Dosimetric measurements demonstrated minimal dose to the lens (0.18±0.10 mGy). Measured entrance skin doses varied from 0.7 to 7.0 mGy and were 73.4±19.7% less than the indicated Ka value. Conclusions Ophthalmic arterial melphalan infusion is a safe and effective means to treat RB. Modification to contemporary fluoroscopic systems combined with parsimonious fluoroscopy can minimize radiation exposure.

Retinoblastoma management remains complex, requiring individualized treatment based on International Classification of Retinoblastoma (ICRB) staging, germline mutation status, family psychosocial factors and cultural beliefs, and available institutional resources. For this 2020 retinoblastoma review, PubMed was searched for articles dated as early as 1931, with an emphasis on articles from 1990 to the present day, using keywords of retinoblastoma, chemotherapy, intravenous chemotherapy, chemoreduction, intra-arterial chemotherapy, ophthalmic artery chemosurgery, intravitreal chemotherapy, intracameral chemotherapy, cryotherapy, transpupillary thermotherapy, laser, radiation, external beam radiotherapy, plaque radiotherapy, brachytherapy, and enucleation. We discuss current treatment modalities as used in the year 2020, including intravenous chemotherapy (IVC), intra-arterial chemotherapy (IAC), intravitreal chemotherapy (IvitC), intracameral chemotherapy (IcamC), consolidation therapies (cryotherapy and transpupillary thermotherapy [TTT]), radiation-based therapies (external beam radiotherapy [EBRT] and plaque radiotherapy), and enucleation. Additionally, we present a consensus treatment algorithm based on the agreement of three North American retinoblastoma treatment centers, and encourage further collaboration amongst the world's most expert retinoblastoma treatment centers in order to develop consensus management plans and continue advancement in the identification and treatment of this childhood cancer.