Pediatric hematology oncology in Brazil

Abstract

Brazil is the largest and most populous country in South America with an area of ∼8,511,965 km2 and a population of 170,029,560 habitants. It is composed mostly of young people; 2007 estimate reveals that 38% of the population is under 19 years of age 1. For 2009, the Instituto Nacional de Câncer (National Cancer Institute) estimates around 9,890 patients with cancer under 19 years of age. There are currently 20 population-based cancer registries (PBCRs) in Brazil. A recent report has described an analysis of the incidence of childhood cancer based on data from 14 PBCRs corresponding to 15% of the child and adolescent populations in Brazil 2. Data were obtained from registry databases, including information on population coverage and data quality indicators. The International Classification of Childhood Cancer was used. Age-adjusted incidence rates (AAIRs) per 1,000,000 children/adolescents ranged from 95 to 231 among the 14 PBCRs with median of 150 per million. The highest AAIRs were seen in Goiania and São Paulo and the lowest were seen in Salvador, Fortaleza, and Campinas. Leukemia, lymphoma, and CNS tumors were the most frequent malignancies in all registries 2. In Brazil over the past two decades, significant changes in mortality have been seen. For instance, in 1979, infectious diseases were the main cause of death among individuals aged <19 years, and malignant neoplasms represented only 1.1% of all deaths (9th). In 2005, the cancer mortality rate in this age group corresponded to 8% of all death cases and, currently, cancer is therefore the second cause of death (5–18 years old) being external causes the leading factor. Relating these mortality data to the specific organization of health services, especially to child and adolescent healthcare, may bring new challenges for oncology care and the Brazilian Unified Health System. Decrease of mortality rates also differs according to regions and its socioeconomic status. For example, higher decrease in mortality from childhood leukemia was observed in more developed states, possibly reflecting better health care 3. Pediatric Oncology in Brazil started in 1964 at Hospital A.C. Camargo with the work of pediatrician Dr. Alois Bianchi, who invited to support oncologists in dealing with childhood cancer. In 1976, a fellowship program was created at Hospital A.C. Camargo with 56 fellows graduated to date; some of them have started their own Pediatric Oncology Departments all over the country 4. There are several Pediatric Oncology fellowship programs distributed in almost all Brazilian states. In 1996, three societies created a Board Certification Examination for Pediatric Oncology Specialist (Brazilian Oncology Society, Brazilian Medical Association and Federal Council of Medicine). Two hundred seven pediatric oncologists have received the degree. Since its beginning, Pediatric Oncology has significantly developed on regards of research, including multidisciplinary expertise, advanced technologies as well as the creation of Master and PhD degree programs. Partnership between private foundations and the Ministry of Health developed project proposals in order to improve research and clinical care. A network of studies linking clinical, biomarkers, and epidemiological data was created. The amount of biological samples sent for diagnosis increased threefold from 2000 to 2007 (n = 3,618) compared to the period from 1990 to 1999 (n = 1,003). Several multidisciplinary projects have been done, immunophenotyping–genotyping studies of acute childhood leukemia, epidemiology and molecular studies of infant acute leukemia, genetic polymorphism of childhood leukemia, and GATA-1 mutations in Down syndrome children 5. The Cooperative Groups Initiative in Brazil started in 1980, allowing exchange of expertise and proposals of better care. In 1981, a Brazilian Pediatric Oncology Society was formed by Dr. Virgilio Carvalho Pinto, a pediatric surgeon who helped to create the society gathering professionals from different centers in Brazil. Seventy-six centers are represented in the society through its 339 members, including nurses, psychologists, physiotherapists, pathologists, pediatric surgeons, radiotherapists, biologists, and pediatric oncologists. There are seven well-established cooperative groups with ongoing protocols including the participation in international studies. Unfortunately, the enrollment rate of new patients is still low. For example, in the Brazilian Wilms Tumor (WT) Cooperative Group, an important dividend of the first trial was the ability to extend the advantages of one of the best treatment protocols available for WT to several major cancer treatment centers in Brazil although we estimate that the trial accrued ∼25% of all new cases of WT occurring in the entire country during that period 6. In the Brazilian Osteosarcoma (OS) Study Group, the number of patients included in the two studies represents 10% of the expected number of patients with OS in Brazil. Five-year overall survival was 60% among patients with non-metastatic disease 7. The Brazilian Cooperative Group for Treatment of Childhood Acute Lymphocytic Leukemia started clinical trials in 1980. Three consecutive multicenter studies in 994 children with newly diagnosed ALL have been completed. In this population, event-free survival has improved from 50% in the period from 1980 to 1982 to 70% for children treated from 1985 to 1988 8. The Brazilian Germ Cell Tumor (GCTs) Study Group established a network for diagnosis, standardized treatment, and documentation. It has significantly improved the survival through the introduction of a standard protocol for patients with GCTs. For high-risk patients, a two agent regimen showed superior survival rate compared to the five agent regimen (83.3% vs. 58.8%) 9. Another effort of international exchange for improvement of clinical care involves the establishment of twinning programs which could overcome the impact of socioeconomic status, such as the program between the St. Jude Children´s Research Hospital and Instituto Materno Infantil de Pernambuco 10. National Campaign for early diagnosis of retinoblastoma in Brazil was initiated in September 2002 with the support of TUCCA and INCTR. The campaign includes educational material for the population, primary health care workers, and ophthalmologists 11. To host the 41st SIOP meeting in São Paulo, Brazil is the perfect landmark of the progress of pediatric oncology since its beginning to the challenges ahead.