Global Retinoblastoma Study Group IMPORTANCE Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale.OBJECTIVES To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. DESIGN, SETTING, AND PARTICIPANTSA total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. MAIN OUTCOMES AND MEASURESAge at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. RESULTSThe cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low-and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI,, and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI,). CONCLUSIONS AND RELEVANCEThis study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.

Wilms tumour is a relatively common and curable paediatric tumour. Known challenges to cure in low income countries are late presentation with advanced disease, malnutrition, failure to complete treatment and limited facilities. In this article, management recommendations are given for a low income setting where only the minimal requirements for treatment with curative intent are available (setting 1). These include general management, supportive care, social support and registration of patients. Recommendations specific for Wilms tumour care include diagnostic procedures with emphasis on the role of ultrasonography, preoperative chemotherapy with a reduced dosage for malnourished children and postoperative chemotherapy based on surgical staging. Pediatr Blood Cancer 2013; 60: 5–11. © 2012 Wiley Periodicals, Inc.

SummaryCancer is increasingly important in low and middle-income settings where infectious diseases are declining. Childhood cancers treated in well-resourced centres have excellent outcomes with more than 80% survival. This success is not reflected in low-income settings where challenges involve every step on the care pathway. Access to diagnosis, delayed presentation, advanced disease, co-morbidities and underlying malnutrition make treatment difficult. Treatments are costly for impoverished families. Yet, the common haematological malignancies (Burkitt lymphoma, Hodgkin lymphoma, non Hodgkin lymphoma) are relatively easy to diagnose and, when managed with simple chemotherapy protocols, give limited but good results. As funding becomes available for cancer research we must ensure that the care and cure of these children is top of the agenda. There is already evidence of improved outcomes in middle-income countries. For others there is a long journey ahead.

PURPOSE Pediatric sarcomas represent an important group of childhood tumors that require treatment at Muhimbili National Hospital (MNH), the largest pediatric oncology center in Tanzania. Treatment is often adapted from established childhood protocols validated in clinical trials from the United States and the United Kingdom. There are no studies describing the types of pediatric sarcomas most commonly seen in Tanzania to understand similarities and disparities with other countries and which sarcomas to prioritize in adapting treatment protocols. The objective of this study was to establish a baseline of the epidemiologic and clinical features of pediatric sarcomas diagnosed at MNH. METHODS Information was collected on clinical and tumor features of all children seen at MNH pediatric oncology unit between 2011 and 2016 with a confirmed histologic diagnosis of either bone or soft tissue sarcoma (STS). RESULTS A total of 135 cases were analyzed; 89 (66%) were STS and 46 (34%) were bone sarcomas. There was a slight female predominance (n = 69; 51%), and the mean age (SD) of patients was 6.3 (5.1) years. Greater than 90% (n = 123) of the cases presented with a painless swelling. The commonest STS, accounting for almost three-fourths of the cases (n = 66) was rhabdomyosarcoma (RMS), with embryonal subtype being the most common RMS (n = 49; 74%). Osteosarcoma was the most common bone sarcoma, accounting for greater than 80% (n = 40) of the cases. Ewing sarcoma accounted for less than 15% (n = 6). Most of the patients presented with stage IV disease (n = 57; 87%) and lung was the commonest metastatic site. CONCLUSION To our knowledge, this report is the first study documenting the epidemiologic and clinical features of pediatric sarcomas in a modern Tanzanian pediatric hospital. Embryonal RMS and osteosarcomas should be prioritized for adapting treatment protocols from other countries.

BackgroundThe travel distance from home to a treatment centre, which may impact the stage at diagnosis, has not been investigated for retinoblastoma, the most common childhood eye cancer. We aimed to investigate the travel burden and its impact on clinical presentation in a large sample of patients with retinoblastoma from Africa and Europe.MethodsA cross-sectional analysis including 518 treatment-naïve patients with retinoblastoma residing in 40 European countries and 1024 treatment-naïve patients with retinoblastoma residing in 43 African countries.ResultsCapture rate was 42.2% of expected patients from Africa and 108.8% from Europe. African patients were older (95% CI −12.4 to −5.4, p<0.001), had fewer cases of familial retinoblastoma (95% CI 2.0 to 5.3, p<0.001) and presented with more advanced disease (95% CI 6.0 to 9.8, p<0.001); 43.4% and 15.4% of Africans had extraocular retinoblastoma and distant metastasis at the time of diagnosis, respectively, compared to 2.9% and 1.0% of the Europeans. To reach a retinoblastoma centre, European patients travelled 421.8 km compared to Africans who travelled 185.7 km (p<0.001). On regression analysis, lower-national income level, African residence and older age (p<0.001), but not travel distance (p=0.19), were risk factors for advanced disease.ConclusionsFewer than half the expected number of patients with retinoblastoma presented to African referral centres in 2017, suggesting poor awareness or other barriers to access. Despite the relatively shorter distance travelled by African patients, they presented with later-stage disease. Health education about retinoblastoma is needed for carers and health workers in Africa in order to increase capture rate and promote early referral.

SummaryEast Africa was at the forefront of early achievements and discoveries in paediatric oncology thanks to Denis Burkitt’s seminal work. Although these successes have been built upon and continued elsewhere, they were sadly not sustained in sub‐Saharan Africa for a variety of reasons. In recent years however this situation is slowly changing in countries across the continent. Tanzania is one such African country. Until very recently, survival rates of 5–10% for all children’s cancers were expected. However, change has been brought about thanks to the combined efforts and commitments of the Tanzanian Ministry of Health, non‐governmental organizations – such as The International Network for Cancer Treatment and Research and Children in Crossfire – and the participation of the private sector. Services are rapidly developing and outcomes are continuing to improve with 1‐year survival rates of approximately 60% achieved. Efforts to maintain this early progress are concentrated around providing high quality local subspecialty medical training and continued local ownership of the programme.

Purpose: In sub-Saharan Africa, cancer treatment facilities are often scarce and centered in urban areas, which means many patients must travel long distances to reach cancer care. Still, little is known about how this distribution of cancer centers impacts patients' ability to access care. Methods: Records of all pediatric patients (&lt;18yo) treated for cancer in Tanzania in 2019 through the Tanzanian Pediatric Cancer Network were reviewed. This network represents all 9 hospitals currently treating children with cancer in Tanzania. Demographic and diagnostic information was recorded. ArcGIS was used to estimate patients' travel times from home addresses to cancer care. AccessMod was used to calculate 4-hour access for the population. Results: In 2019, a total of 821 pediatric patients were diagnosed with cancer with an incidence of 1.42/100,000 population. The most common diagnoses were Wilms tumor (17.2%, n=134), retinoblastoma (16.8%, n=131), and acute lymphoblastic leukemia (15.9%, n=124). There was regional variation by diagnosis, with the Lake Zone having the highest incidence of Burkitt lymphoma (0.29; average 0.15) and retinoblastoma (0.5; average 0.33). Reported regional incidence inversely correlated to distance to cancer treatment facility. Median travel time to cancer care for patients was 4.55 hours. Currently 74.5% of the population lives within 4 hours of existing cancer care facilities, and it would require adding cancer care capacity to 8 additional facilities to reach 95% coverage. Conclusions: This is the first comprehensive evaluation of the incidence of presenting pediatric cancer patients in Tanzania. Only 18.8% of the estimated pediatric patients with cancer in Tanzania were treated in 2019. The reported incidence was lower in areas further away from treatment centers, suggesting the importance of increased access to cancer care facilities as part of a future national scale-up strategy. Citation Format: Luke Maillie, Alice Mutagonda, Lulu Chirande, Laiti Rehema, Shakilu Jumanne, Elton Meleki, Furaha Serventi, Julius Alloyce, Franco Afyusisye, Sebastian Sanchez, Matthew Sisk, Marie Jose Voeten, Ester Kawira, Heronima Joas, Trish Scanlan, Kristin Schroeder. The Tanzanian Pediatric Cancer Network: A Comprehensive Evaluation of the Incidence of Presenting Patients and Access to Pediatric Cancer Care in Tanzania [abstract]. In: Proceedings of the 9th Annual Symposium on Global Cancer Research; Global Cancer Research and Control: Looking Back and Charting a Path Forward; 2021 Mar 10-11. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2021;30(7 Suppl):Abstract nr 101.

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