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Retinoblastoma is curable when diagnosed early and treated appropriately; however, the prognosis is dismal when the basic elements in diagnosis and treatment are lacking. In developing countries, poor education, lower socioeconomic conditions, and inefficient health care systems result in delayed diagnosis and suboptimal care. Furthermore, the complexity of multidisciplinary care required is seldom possible. While ocular salvage is a priority in the Western world, death from retinoblastoma is still a major problem in developing countries. To bring the two ends of this spectrum together and provide a forum for discussion, the One World, One Vision symposium was organized, where clinicians and researchers from various cultural, geographic, and socioeconomic backgrounds converged to discuss their experiences. Strategies for early diagnosis in developing countries were discussed. Elements in the development of retinoblastoma centers in developing countries were discussed, and examples of successful programs were highlighted. An important component in this process is twinning between centers in developing countries and mentor institutions in high-income countries. Global initiatives by nongovernmental organizations such as the International Network for Cancer Treatment and Research, Orbis International, and the International Agency for Prevention of Blindness were presented. Treatment of retinoblastoma in developing countries remains a challenge. However, it is possible to coordinate efforts at multiple levels, including public administrations and nonprofit organizations, to improve the diagnosis and treatment of retinoblastoma and to improve the outcome for these children.

Rosai-Dorfman disease, although historically described as benign and self-limiting, may cause significant morbidity and mortality involving multiple organ systems. Available treatment options may not control the disease. Further research and long-term clinical correlation is necessary.

Background: Chronic hypercapnic respiratory failure is associated with high mortality.While prior work has demonstrated a mortality improvement with high intensity noninvasive ventilation in chronic obstructive pulmonary disease, it is unclear whether a partial pressure carbon dioxide (PCO2) reduction strategy is associated with improved outcomes in other populations of chronic hypercapnia Methods: The objective of this study was to investigate the association between PCO2 reduction (using transcutaneous partial pressure of carbon dioxide as an estimate for arterial PCO2) and survival in a broad population of individuals treated with NIV for chronic hypercapnia. We hypothesized that reductions in PCO2, would be associated with improved survival. Therefore, we performed a cohort study of all subjects evaluated from February 2012 to January 2021 for NIV initiation/optimization due to chronic hypercapnia at a home ventilation clinic in an academic center. We used multivariable Cox proportional hazard models with time-varying coefficients and PCO2 as a timevarying covariate to test the association between PCO2 and all-cause mortality adjusting for known cofounders Results:The mean age of 337 subjects was 57 ± 16 years; 37% female and 85% white.In univariate analysis, survival probability increased with reductions in PCO2 to <50 mm Hg after 90 days, and these remained significant after adjusting for age, sex, race, body mass index, diagnosis, Charlson comorbidity index, and baseline PCO2. In the multivariable analysis, patients who had a < 50 mm Hg had a reduced mortality risk of 94% between 90-179 days (HR=0.06; 95% CI: 0.01 -0.50), 69% between 180 -364 days (HR=0.31; 95% CI: 0.12 -0.79), and 73% for 365 -730 days (HR = 0.27; 95% CI: 0.13 -0.56). Conclusion:Reduction in PCO2 from baseline for subjects with chronic hypercapnia on NIV is associated with improved survival. Management strategies should target the greatest attainable reductions in PCO2.

To investigate the clinical outcomes and late effects associated with the use of proton therapy to treat children with head and neck rhabdomyosarcoma (HN-RMS). Materials/Methods: Between 2006 and 2014, 46 children with HN-RMS were treated consecutively at a single institution and enrolled on a prospective registry protocol. 41 (89%) had localized HN-RMS and 5 (11%) had metastatic disease. 5 patients were Intergroup Rhabdomyosarcoma Study (IRS) Group II, 35 were group III, and 5 were group IV. 3 patients were treated for locally recurrent tumors following prior treatment with chemotherapy and surgery alone. The median age was 5.4 years (range, 0.5 e 15.5) at the start of proton therapy. The primary tumor location was parameningeal for 25 patients (54%), orbital for 13 (28%), and other head and neck sites for 8 (17%). 11 patients with parameningeal tumors were found to have intracranial extension (ICE). Histology was embryonal in 32 (70%) and alveolar in 14 (30%). All patients received multi-agent chemotherapy, most commonly (72%) with Vincristine, Dactinomycin, and Cyclophosphamide per COG and EpSSG protocols. All were treated with proton therapy to the primary site with a median dose of 50.4 CGE (range, 36e50.8 CGE). Median follow-up for surviving patients was 4.3 years (range, 0.8e9.1). Results: The 5-year overall survival was 77% in the entire group and was significantly lower in patients with ICE (PZ0.027). 5-year local control rates were 100%, 80%, and 60% in groups I, II, and III respectively. There were 16 failures with a median time to failure of 12.5 months (range, 4e52 months). The initial sites of failure were local only in 4 patients, regional only in 8, and local and regional in 4. Six local failures were in-field and within the high dose region of the radiation treatment. There were 2 marginal failures. Five of the regional only failures were located within the draining lymph nodes not previously treated with radiation therapy. Four patients (36%) with ICE at diagnosis failed in the CNS. Late toxicity included cataracts in 6, bone hypoplasia in 2, hearing loss in 1, and dental complications in 1. No second malignancies have been reported. Conclusion: Proton therapy has been well tolerated by our cohort of patients with outcomes and toxicities that compare favorably with other published reports. Careful review of nodal coverage and the isolated nodal recurrences in this group might help identify risk factors for lymph node recurrence.

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