This was the first time since the Rb-NET MDT Project was launched in 2020 that we gathered together, all its members for a joint multinational meeting. We heard an enlightening update on global blindness given by Prof. Allen Foster from the LSHTM, and case presentations by leading RB experts:
Presented a case of a 6-year-old girl with suspicious mass in the posterior segment and what seems as hypopyon in the anterior chamber. When asked the audience “what would you do next?”, the majority opted for uveitis workup, whereas Dr. Shields suspected it to be retinoblastoma, and treated the child conservatively with chemotherapy and plaque brachytherapy. At final visit, 3 years from presentation, the eye was salvaged and final visual acuity was 20/30 in the affected eye.
Presented a case of a 7-year-old boy with complaints of decreased vision in the right eye. On examination under anesthesia, he had amelanotic masses in both fundi, occupying the macula OD. When asked the audience “what would you do next?”, the majority opted for intravenous chemotherapy treatment. Dr. Kaliki explained that this was in fact a retinoma – a benign variant of retinoblastoma, and further explained how to differentiate malignant retinoblastoma from benign retinoma, and indicated the follow-up protocol. After 1-year of careful follow-up, with no treatment, the tumors appeared the same, confirming the diagnosis.
Presented a case of a 5-month-old girl who was referred from another facility after enucleation of the right eye. In the left eye there was a small tumor in the posterior pole classified as Group A (ICRB). When asked the audience “what would you do next?”, the majority rightfully opted for focal TTT laser. The tumor, however, relapsed, and again, despite repeated laser, and new vitreous seeds arose from it. In the absence of intra-vitreous chemotherapy (2016), intravenous chemotherapy was given, with no benefit. Eventually, the patient underwent PPV and tumorectomy in China, and now is nearly 10 years old, with no active disease and 6/6 vision.
Presented a case of a 4.5-year-old boy whose mother noticed strabismus in the right eye. On fundus examination, there was a large, presumably intraocular amelanotic lesion with surrounding retinal detachment. When asked the audience “what would you do next?”, the majority opted for intravenous chemotherapy. Dr. Teixeira, however, explained that one must perform systemic investigations, including brain and orbit MR, Lumbar puncture, genetic tests, and bone marrow aspiration. On imaging, surprisingly, the tumor extended posteriorly with thickened optic nerve throughout the orbit giving rise to stage IV b (IRSS). The child was treated by a combination of chemotherapy, enucleation, stem cell rescue, and external beam radiotherapy, and is currently being monitored.
Presented a case of a 2-year-old boy whose left eye was enucleated due to advanced retinoblastoma and right eye treated by means of systemic chemotherapy. Following 4 courses of VEC, the tumors OS were not responsive. When asked the audience “what would you do next?”, the majority opted for intra-vitreous chemotherapy. Dr. Popoola, however, suggested that second-line chemotherapy was her choice with this child. In the absence of vitreous seeds (as in this case), injection of chemotherapy into the vitreous would have little effect. The child, unfortunately, progressed despite attempts of globe salvage, and currently, the parents are being consulted for second eye enucleation.
Thank you to all panelists and attendees from across the world, and stay tuned for the Rb-NET Challenges 2023!