Doctor of Philosophy / Over the past decade, population-based cancer registry data have been used increasingly worldwide to evaluate and improve the quality of cancer care. The utility of the conclusions from such studies relies heavily on the data quality and the methods used to analyse the data. Interpretation of comparative survival from such data, examining either temporal trends or geographical differences, is generally not easy. The observed differences could be due to methodological and statistical approaches or to real effects. For example, geographical differences in cancer survival could be due to a number of real factors, including access to primary health care, the availability of diagnostic and treatment facilities and the treatment actually given, or to artefact, such as lead-time bias, stage migration, sampling error or measurement error. Likewise, a temporal increase in survival could be the result of earlier diagnosis and improved treatment of cancer; it could also be due to artefact after the introduction of screening programs (adding lead time), changes in the definition of cancer, stage migration or several of these factors, producing both real and artefactual trends. In this thesis, I report methods that I modified and applied, some technical issues in the use of such data, and an analysis of data from the State of New South Wales (NSW), Australia, illustrating their use in evaluating and potentially improving the quality of cancer care, showing how data quality might affect the conclusions of such analyses. This thesis describes studies of comparative survival based on population-based cancer registry data, with three published papers and one accepted manuscript (subject to minor revision). In the first paper, I describe a modified method for estimating spatial variation in cancer survival using empirical Bayes methods (which was published in Cancer Causes and Control 2004). I demonstrate in this paper that the empirical Bayes method is preferable to standard approaches and show how it can be used to identify cancer types where a focus on reducing area differentials in survival might lead to important gains in survival. In the second paper (published in the European Journal of Cancer 2005), I apply this method to a more complete analysis of spatial variation in survival from colorectal cancer in NSW and show that estimates of spatial variation in colorectal cancer can help to identify subgroups of patients for whom better application of treatment guidelines could improve outcome. I also show how estimates of the numbers of lives that could be extended might assist in setting priorities for treatment improvement. In the third paper, I examine time trends in survival from 28 cancers in NSW between 1980 and 1996 (published in the International Journal of Cancer 2006) and conclude that for many cancers, falls in excess deaths in NSW from 1980 to 1996 are unlikely to be attributable to earlier diagnosis or stage migration; thus, advances in cancer treatment have probably contributed to them. In the accepted manuscript, I described an extension of the work reported in the second paper, investigating the accuracy of staging information recorded in the registry database and assessing the impact of error in its measurement on estimates of spatial variation in survival from colorectal cancer. The results indicate that misclassified registry stage can have an important impact on estimates of spatial variation in stage-specific survival from colorectal cancer. Thus, if cancer registry data are to be used effectively in evaluating and improving cancer care, the quality of stage data might have to be improved. Taken together, the four papers show that creative, informed use of population-based cancer registry data, with appropriate statistical methods and acknowledgement of the limitations of the data, can be a valuable tool for evaluating and possibly improving cancer care. Use of these findings to stimulate evaluation of the quality of cancer care should enhance the value of the investment in cancer registries. They should also stimulate improvement in the quality of cancer registry data, particularly that on stage at diagnosis. The methods developed in this thesis may also be used to improve estimation of geographical variation in other count-based health measures when the available data are sparse.

Doctor of Philosophy / Over the past decade, population-based cancer registry data have been used increasingly worldwide to evaluate and improve the quality of cancer care. The utility of the conclusions from such studies relies heavily on the data quality and the methods used to analyse the data. Interpretation of comparative survival from such data, examining either temporal trends or geographical differences, is generally not easy. The observed differences could be due to methodological and statistical approaches or to real effects. For example, geographical differences in cancer survival could be due to a number of real factors, including access to primary health care, the availability of diagnostic and treatment facilities and the treatment actually given, or to artefact, such as lead-time bias, stage migration, sampling error or measurement error. Likewise, a temporal increase in survival could be the result of earlier diagnosis and improved treatment of cancer; it could also be due to artefact after the introduction of screening programs (adding lead time), changes in the definition of cancer, stage migration or several of these factors, producing both real and artefactual trends. In this thesis, I report methods that I modified and applied, some technical issues in the use of such data, and an analysis of data from the State of New South Wales (NSW), Australia, illustrating their use in evaluating and potentially improving the quality of cancer care, showing how data quality might affect the conclusions of such analyses. This thesis describes studies of comparative survival based on population-based cancer registry data, with three published papers and one accepted manuscript (subject to minor revision). In the first paper, I describe a modified method for estimating spatial variation in cancer survival using empirical Bayes methods (which was published in Cancer Causes and Control 2004). I demonstrate in this paper that the empirical Bayes method is preferable to standard approaches and show how it can be used to identify cancer types where a focus on reducing area differentials in survival might lead to important gains in survival. In the second paper (published in the European Journal of Cancer 2005), I apply this method to a more complete analysis of spatial variation in survival from colorectal cancer in NSW and show that estimates of spatial variation in colorectal cancer can help to identify subgroups of patients for whom better application of treatment guidelines could improve outcome. I also show how estimates of the numbers of lives that could be extended might assist in setting priorities for treatment improvement. In the third paper, I examine time trends in survival from 28 cancers in NSW between 1980 and 1996 (published in the International Journal of Cancer 2006) and conclude that for many cancers, falls in excess deaths in NSW from 1980 to 1996 are unlikely to be attributable to earlier diagnosis or stage migration; thus, advances in cancer treatment have probably contributed to them. In the accepted manuscript, I described an extension of the work reported in the second paper, investigating the accuracy of staging information recorded in the registry database and assessing the impact of error in its measurement on estimates of spatial variation in survival from colorectal cancer. The results indicate that misclassified registry stage can have an important impact on estimates of spatial variation in stage-specific survival from colorectal cancer. Thus, if cancer registry data are to be used effectively in evaluating and improving cancer care, the quality of stage data might have to be improved. Taken together, the four papers show that creative, informed use of population-based cancer registry data, with appropriate statistical methods and acknowledgement of the limitations of the data, can be a valuable tool for evaluating and possibly improving cancer care. Use of these findings to stimulate evaluation of the quality of cancer care should enhance the value of the investment in cancer registries. They should also stimulate improvement in the quality of cancer registry data, particularly that on stage at diagnosis. The methods developed in this thesis may also be used to improve estimation of geographical variation in other count-based health measures when the available data are sparse.

A disponibilidade de grandes bases de dados informatizadas em saúde tornou a técnica de relacionamento de fontes de dados, também conhecida como linkage, uma alternativa para diferentes tipos de estudos. Esta técnica proporciona a geração de uma base de dados mais completa e de baixo custo operacional. Objetivo- Investigar a possibilidade de completar/aperfeiçoar as informações da base de dados do RCBP-SP, no período de 1997 a 2005, utilizando o processo de linkage com três outras bases, a saber: Programa de Aprimoramento de Mortalidade (PRO-AIM), Autorização e Procedimentos de Alta Complexidade (APAC-SIA/SUS) e Fundação Sistema Estadual de Análise de Dados (FSeade). Métodos- Neste estudo foi utilizada a base de dados do RCBP-SP, composta por 343.306 com casos incidentes de câncer do município de São Paulo, registrados no período de 1997 a 2005, com idades que variaram de menos de um a 106 anos, de ambos os sexos. Para a completitude das informações do RCBP-SP foram utilizadas as bases de dados, a saber: PRO-AIM, APAC-SIA/SUS e FSeade. Foram utilizadas as técnicas de linkage probabilística e determinística. O linkage probabilístico foi realizado pelo programa Reclink III versão 3.1.6. Quanto ao linkage determinístico as rotinas foram realizadas em Visual Basic, com as bases hospedadas em SQL Server. Foram calculados os coeficientes brutos de incidência (CBI) e mortalidade (CBM) antes e após o linkage. A análise de sobrevida global foi realizada pela técnica de Kaplan-Meier e para na comparação entre as curvas, utilizou-se o teste de log rank. Foram calculados os valores da área sob a curva, sensibilidade e especificidade para determinar o ponto de corte do escore de maior precisão na identificação dos pares verdadeiros. Resultados- Após o linkage, verificou-se um ganho de 101,5 por cento para a variável endereço e 31,5 por cento para a data do óbito e 80,0 por cento para a data da última informação. Quanto à variável nome da mãe, na base de dados do RCBP-SP antes do linkage esta informação representava somente 0,5 por cento , tendo sido complementada, no geral, em 76.332 registros. A análise de sobrevida global mostrou que antes do processo de linkage havia uma subestimação na probabilidade de estar vivo em todos os períodos analisados. No geral, para a análise de sobrevida truncada em sete anos, a probabilidade de estar vivo no primeiro ano de seguimento antes do linkage foi menor quando comparada a probabilidade de estar vivo ao primeiro ano de seguimento após o linkage (48,8 por cento x 61,1 por cento ; p< 0,001). Conclusão- A técnica de linkage tanto probabilística quanto determinística foi efetiva para completar/aperfeiçoar as informações da base de dados do RCBP-SP. Além do mais, o CBI apresentou um ganho de 3,4 por cento . Quanto ao CBM houve um ganho de 25,8 por cento . Após o uso da técnica de linkage, foi verificado que os valores para a sobrevida global estavam subestimados para ambos os sexos, faixas etárias e para as topografias de câncer / The availability of large computerized databases on health has enabled the record linkage technique, an alternative for different study designs. This technique provides the generation of a more complete database, at low operational cost. Objective to investigate the possibility of completing/improving information from the database of the RCBP-SP, in the period between 1997 and 2005, using the record linkage technique with other three databases, namely: Mortality Improvement Program (PRO-AIM), Authorization of Highly Complex Procedures (APAC-SIA/SUS) and State System of Data Analysis (FSeade), comparing different strategies. Methods In this study we used the database of the RCBP-SP composed of 343,306 incident cancer cases in the Municipality of São Paulo registered in the period between 1997 and 2005 with ages raging from under one to 106 years, from both sexes. To complete the database of the RCBP-SP three databases were used, namely: PRO-AIM, APAC-SIA/SUS and FSeade. Both probabilistic and deterministic record linkage were used. Probabilistic linkage was performed using the Reclink III software, version 3.1.6. As for the the deterministic record linkage, the routines were run in the Visual Basic and databases hosted on a SQL Server. Before and after record linkage, crude incidence (CIR) and mortality rates (CMR) were calculated. The overall survival analysis was performed using the Kaplan-Meier technique and for the comparison between curves, the log rank test was employed. In order to determine the most precise cut-off scores in identifying true matches, we calculated the area under the curve, as well as, sensitivity and specificity. Results After record linkage, it was verified a gain of 101.5 per cent for the variable address, 31.5 per cent for death date and 80,0 per cent for the date of latest information. As for the variable mother´s name, in the database of the RCBP-SP before record linkage, this information represented only 0.5 per cent , having been completed, in general, in 76,332 registries. The overall survival analysis showed that before the record linkage there was an underestimation of the probability of being alive for all periods assessed. In general, for the truncated survival at seven years, the probability of being alive at the first year of follow up before record linkage was lower when compared to the probability of being alive at the first year of follow up after record linkage (48.8 per cent x 61.1 per cent ; p< 0.001). Conclusion Both the probabilistic and deterministic record linkage were effective to complete/improve information from the database of the RCBP-SP. Moreover, the CIR had a gain of de 3.4 per cent . As for the CMR, there was a gain of 25.8 per cent . After using the record linkage technique, it was verified that values for overall survival were underestimated for both sexes, all age groups, and cancer sites

Introdução: A associação entre a infecção pelo vírus da imunodeficiência humana (HIV) e o câncer tem sido documentada desde os primórdios da epidemia da síndrome da imunodeficiência adquirida (Aids). A introdução da highly active antirretroviral therapy (HAART) alterou, profundamente, o curso da epidemia da Aids, reduzindo, drasticamente, a incidência de manifestações definidoras da síndrome, incluindo cânceres. No entanto, existem informações limitadas sobre a incidência de câncer em crianças e adolescentes com Aids vivendo em países em desenvolvimento. Objetivo: Descrever a epidemiologia do câncer em crianças e adolescentes com Aids no Município de São Paulo, no período de 1997 a 2012. Métodos: Trata-se de um estudo de base populacional, utilizando as bases de dados do Registro de Câncer de Base Populacional do Município de São Paulo e do Sistema de Informações de Agravos de Notificação (SINAN). As crianças e adolescentes (< 20 anos) com Aids e câncer foram identificadas por meio de um processo de linkage probabilístico entre as bases de dados supracitadas. Foram calculadas as taxas de incidência brutas e ajustadas por milhão de habitantes. Para comparar a incidência de câncer na população com Aids e a população geral foi calculada a razão de incidência padronizada (RIP) e respectivos intervalos de confiança de 95 por cento (IC 95 por cento ). A análise de tendência foi feita por meio do cálculo do annual percent change (APC) e IC 95 por cento correspondentes. A análise da sobrevida global de cinco anos após o câncer entre pacientes com Aids e na população geral foi calculada por meio do estimador produto limite de Kaplan-Meier e modelos univariados de riscos proporcionais de Cox. Mapas coropléticos em escalas monocromáticas foram gerados para descrever a distribuição de casos no Município. Resultados: Foram identificados 24 casos de câncer em pacientes com Aids menores de 20 anos, sendo 62,5 por cento cânceres definidores de Aids. Os cânceres mais incidentes foram o linfoma não Hodgkin, incluindo o linfoma de Burkitt (12; 50,0 por cento ), o linfoma de Hodgkin (6; 25,0 por cento ) e o sarcoma de Kaposi (3; 12,5 por cento ). A taxa bruta de incidência foi de 1.461,3 casos/milhão. A análise de tendência revelou redução significativa da incidência para todos os cânceres (APC= -14,5), influenciada pela queda nos cânceres definidores de Aids (APC= -17,0). O risco para câncer se mostrou aumentado (RIP= 3,9), sobretudo para o linfoma não Hodgkin, excluindo linfoma de Burkitt (RIP= 22,5), linfoma de Burkitt (RIP= 29,7) e linfoma de Hodgkin (RIP= 18,7). A probabilidade acumulada de sobrevida aos cinco anos foi de 56,3 por cento em crianças e adolescentes com Aids versus 87,5 por cento na população geral. A hazard ratio para óbito foi 5,2 (IC 95 por cento = 2,0; 13,6). O mapa da distribuição geográfica mostrou concentração dos casos nas áreas de classes sociais mais baixas do Município. Conclusão: Houve redução acentuada da incidência de cânceres definidores de Aids, como provável resultado da introdução da HAART. No entanto, crianças e adolescentes com Aids permanecem sob risco aumentado para o desenvolvimento de câncer quando comparadas à população geral. Para aquelas que desenvolveram câncer, o risco para óbito também se mostrou substancialmente elevado / Introduction: The association between human immunodeficiency virus (HIV) infection and cancer has been documented since the beginning of the epidemic of the acquired immunodeficiency syndrome (AIDS). The introduction of the highly active antiretroviral therapy (HAART) has profoundly altered the course of the AIDS epidemic, drastically reducing the incidence of AIDS-defining manifestations, including cancers. Nevertheless, there is limited information on the incidence of cancer in children and adolescents with AIDS living in developing countries. Objective: To describe the cancer epidemiology in children and adolescents with AIDS in the Municipality of São Paulo from 1997 to 2012. Methods: It is a population-based study, using the databases of the Population-based Cancer Registry of São Paulo and the Notifiable Diseases Information System (SINAN). Children and adolescents (< 20 years) with AIDS and cancer have been identified by means of a probabilistic record linkage process between the aforementioned databases. Crude and age-standardized incidence rates per million inhabitants were calculated. To compare the incidence of cancer in people with AIDS and that of the general population, standardized incidence ratio (SIR) and respective 95 per cent confidence intervals (95 per cent CI) were calculated. We examined trends by calculating the annual percent change (APC) and corresponding 95 per cent CI. The analyses of the overall five-year survival after cancer diagnosis among children and adolescents with AIDS and that of the general population were based on the Kaplan-Meier product limit estimator and univariate Cox proportional hazards models. Choropleth maps on monochromatic scales were generated to describe the distribution of cases across the Municipality. Results: We identified 24 cases of cancer in patients with AIDS aged 20 years and younger, of which, 62.5 per cent were AIDS-defining malignancies. The most incident cancers were non-Hodgkin\'s lymphoma, including Burkitt\'s lymphoma (12; 50.0 per cent ), Hodgkin\'s lymphoma (6; 25.0 per cent ) and Kaposi sarcoma (3; 12.5 per cent ). The age-standardized incidence rate was 1,461.3 cases/million. The trend analyses revealed a significant reduction in the incidence of all cancers (APC= -14.5), driven by the decrease in AIDS-defining cancers (APC= -17.0). The overall risk for cancer was significantly increased (SIR= 3.9), especially for non-Hodgkin lymphoma, excluding Burkitts lymphoma (SIR= 22.5), Burkitt\'s lymphoma (SIR= 29.7) and Hodgkin\'s lymphoma (SIR= 18.7). The overall probability of survival at five years after cancer was 56.3 per cent in children and adolescents with AIDS versus 87.5 per cent in the general population. The hazard ratio for death was 5.2 (95 per cent CI= 2.0, 13.6). The map of the geographical distribution showed a concentration of cases in the low-income areas of the Municipality. Conclusion: There was a marked reduction in the incidence of AIDS-defining cancers, likely to be a result of the introduction of HAART. However, children and adolescents with AIDS remain at increased risk for the development of cancer when compared to the general population. For those who developed cancer, the risk of death was also significantly higher

A disponibilidade de grandes bases de dados informatizadas em saúde tornou a técnica de relacionamento de fontes de dados, também conhecida como linkage, uma alternativa para diferentes tipos de estudos. Esta técnica proporciona a geração de uma base de dados mais completa e de baixo custo operacional. Objetivo- Investigar a possibilidade de completar/aperfeiçoar as informações da base de dados do RCBP-SP, no período de 1997 a 2005, utilizando o processo de linkage com três outras bases, a saber: Programa de Aprimoramento de Mortalidade (PRO-AIM), Autorização e Procedimentos de Alta Complexidade (APAC-SIA/SUS) e Fundação Sistema Estadual de Análise de Dados (FSeade). Métodos- Neste estudo foi utilizada a base de dados do RCBP-SP, composta por 343.306 com casos incidentes de câncer do município de São Paulo, registrados no período de 1997 a 2005, com idades que variaram de menos de um a 106 anos, de ambos os sexos. Para a completitude das informações do RCBP-SP foram utilizadas as bases de dados, a saber: PRO-AIM, APAC-SIA/SUS e FSeade. Foram utilizadas as técnicas de linkage probabilística e determinística. O linkage probabilístico foi realizado pelo programa Reclink III versão 3.1.6. Quanto ao linkage determinístico as rotinas foram realizadas em Visual Basic, com as bases hospedadas em SQL Server. Foram calculados os coeficientes brutos de incidência (CBI) e mortalidade (CBM) antes e após o linkage. A análise de sobrevida global foi realizada pela técnica de Kaplan-Meier e para na comparação entre as curvas, utilizou-se o teste de log rank. Foram calculados os valores da área sob a curva, sensibilidade e especificidade para determinar o ponto de corte do escore de maior precisão na identificação dos pares verdadeiros. Resultados- Após o linkage, verificou-se um ganho de 101,5 por cento para a variável endereço e 31,5 por cento para a data do óbito e 80,0 por cento para a data da última informação. Quanto à variável nome da mãe, na base de dados do RCBP-SP antes do linkage esta informação representava somente 0,5 por cento , tendo sido complementada, no geral, em 76.332 registros. A análise de sobrevida global mostrou que antes do processo de linkage havia uma subestimação na probabilidade de estar vivo em todos os períodos analisados. No geral, para a análise de sobrevida truncada em sete anos, a probabilidade de estar vivo no primeiro ano de seguimento antes do linkage foi menor quando comparada a probabilidade de estar vivo ao primeiro ano de seguimento após o linkage (48,8 por cento x 61,1 por cento ; p< 0,001). Conclusão- A técnica de linkage tanto probabilística quanto determinística foi efetiva para completar/aperfeiçoar as informações da base de dados do RCBP-SP. Além do mais, o CBI apresentou um ganho de 3,4 por cento . Quanto ao CBM houve um ganho de 25,8 por cento . Após o uso da técnica de linkage, foi verificado que os valores para a sobrevida global estavam subestimados para ambos os sexos, faixas etárias e para as topografias de câncer / The availability of large computerized databases on health has enabled the record linkage technique, an alternative for different study designs. This technique provides the generation of a more complete database, at low operational cost. Objective to investigate the possibility of completing/improving information from the database of the RCBP-SP, in the period between 1997 and 2005, using the record linkage technique with other three databases, namely: Mortality Improvement Program (PRO-AIM), Authorization of Highly Complex Procedures (APAC-SIA/SUS) and State System of Data Analysis (FSeade), comparing different strategies. Methods In this study we used the database of the RCBP-SP composed of 343,306 incident cancer cases in the Municipality of São Paulo registered in the period between 1997 and 2005 with ages raging from under one to 106 years, from both sexes. To complete the database of the RCBP-SP three databases were used, namely: PRO-AIM, APAC-SIA/SUS and FSeade. Both probabilistic and deterministic record linkage were used. Probabilistic linkage was performed using the Reclink III software, version 3.1.6. As for the the deterministic record linkage, the routines were run in the Visual Basic and databases hosted on a SQL Server. Before and after record linkage, crude incidence (CIR) and mortality rates (CMR) were calculated. The overall survival analysis was performed using the Kaplan-Meier technique and for the comparison between curves, the log rank test was employed. In order to determine the most precise cut-off scores in identifying true matches, we calculated the area under the curve, as well as, sensitivity and specificity. Results After record linkage, it was verified a gain of 101.5 per cent for the variable address, 31.5 per cent for death date and 80,0 per cent for the date of latest information. As for the variable mother´s name, in the database of the RCBP-SP before record linkage, this information represented only 0.5 per cent , having been completed, in general, in 76,332 registries. The overall survival analysis showed that before the record linkage there was an underestimation of the probability of being alive for all periods assessed. In general, for the truncated survival at seven years, the probability of being alive at the first year of follow up before record linkage was lower when compared to the probability of being alive at the first year of follow up after record linkage (48.8 per cent x 61.1 per cent ; p< 0.001). Conclusion Both the probabilistic and deterministic record linkage were effective to complete/improve information from the database of the RCBP-SP. Moreover, the CIR had a gain of de 3.4 per cent . As for the CMR, there was a gain of 25.8 per cent . After using the record linkage technique, it was verified that values for overall survival were underestimated for both sexes, all age groups, and cancer sites

Introdução: A associação entre a infecção pelo vírus da imunodeficiência humana (HIV) e o câncer tem sido documentada desde os primórdios da epidemia da síndrome da imunodeficiência adquirida (Aids). A introdução da highly active antirretroviral therapy (HAART) alterou, profundamente, o curso da epidemia da Aids, reduzindo, drasticamente, a incidência de manifestações definidoras da síndrome, incluindo cânceres. No entanto, existem informações limitadas sobre a incidência de câncer em crianças e adolescentes com Aids vivendo em países em desenvolvimento. Objetivo: Descrever a epidemiologia do câncer em crianças e adolescentes com Aids no Município de São Paulo, no período de 1997 a 2012. Métodos: Trata-se de um estudo de base populacional, utilizando as bases de dados do Registro de Câncer de Base Populacional do Município de São Paulo e do Sistema de Informações de Agravos de Notificação (SINAN). As crianças e adolescentes (< 20 anos) com Aids e câncer foram identificadas por meio de um processo de linkage probabilístico entre as bases de dados supracitadas. Foram calculadas as taxas de incidência brutas e ajustadas por milhão de habitantes. Para comparar a incidência de câncer na população com Aids e a população geral foi calculada a razão de incidência padronizada (RIP) e respectivos intervalos de confiança de 95 por cento (IC 95 por cento ). A análise de tendência foi feita por meio do cálculo do annual percent change (APC) e IC 95 por cento correspondentes. A análise da sobrevida global de cinco anos após o câncer entre pacientes com Aids e na população geral foi calculada por meio do estimador produto limite de Kaplan-Meier e modelos univariados de riscos proporcionais de Cox. Mapas coropléticos em escalas monocromáticas foram gerados para descrever a distribuição de casos no Município. Resultados: Foram identificados 24 casos de câncer em pacientes com Aids menores de 20 anos, sendo 62,5 por cento cânceres definidores de Aids. Os cânceres mais incidentes foram o linfoma não Hodgkin, incluindo o linfoma de Burkitt (12; 50,0 por cento ), o linfoma de Hodgkin (6; 25,0 por cento ) e o sarcoma de Kaposi (3; 12,5 por cento ). A taxa bruta de incidência foi de 1.461,3 casos/milhão. A análise de tendência revelou redução significativa da incidência para todos os cânceres (APC= -14,5), influenciada pela queda nos cânceres definidores de Aids (APC= -17,0). O risco para câncer se mostrou aumentado (RIP= 3,9), sobretudo para o linfoma não Hodgkin, excluindo linfoma de Burkitt (RIP= 22,5), linfoma de Burkitt (RIP= 29,7) e linfoma de Hodgkin (RIP= 18,7). A probabilidade acumulada de sobrevida aos cinco anos foi de 56,3 por cento em crianças e adolescentes com Aids versus 87,5 por cento na população geral. A hazard ratio para óbito foi 5,2 (IC 95 por cento = 2,0; 13,6). O mapa da distribuição geográfica mostrou concentração dos casos nas áreas de classes sociais mais baixas do Município. Conclusão: Houve redução acentuada da incidência de cânceres definidores de Aids, como provável resultado da introdução da HAART. No entanto, crianças e adolescentes com Aids permanecem sob risco aumentado para o desenvolvimento de câncer quando comparadas à população geral. Para aquelas que desenvolveram câncer, o risco para óbito também se mostrou substancialmente elevado / Introduction: The association between human immunodeficiency virus (HIV) infection and cancer has been documented since the beginning of the epidemic of the acquired immunodeficiency syndrome (AIDS). The introduction of the highly active antiretroviral therapy (HAART) has profoundly altered the course of the AIDS epidemic, drastically reducing the incidence of AIDS-defining manifestations, including cancers. Nevertheless, there is limited information on the incidence of cancer in children and adolescents with AIDS living in developing countries. Objective: To describe the cancer epidemiology in children and adolescents with AIDS in the Municipality of São Paulo from 1997 to 2012. Methods: It is a population-based study, using the databases of the Population-based Cancer Registry of São Paulo and the Notifiable Diseases Information System (SINAN). Children and adolescents (< 20 years) with AIDS and cancer have been identified by means of a probabilistic record linkage process between the aforementioned databases. Crude and age-standardized incidence rates per million inhabitants were calculated. To compare the incidence of cancer in people with AIDS and that of the general population, standardized incidence ratio (SIR) and respective 95 per cent confidence intervals (95 per cent CI) were calculated. We examined trends by calculating the annual percent change (APC) and corresponding 95 per cent CI. The analyses of the overall five-year survival after cancer diagnosis among children and adolescents with AIDS and that of the general population were based on the Kaplan-Meier product limit estimator and univariate Cox proportional hazards models. Choropleth maps on monochromatic scales were generated to describe the distribution of cases across the Municipality. Results: We identified 24 cases of cancer in patients with AIDS aged 20 years and younger, of which, 62.5 per cent were AIDS-defining malignancies. The most incident cancers were non-Hodgkin\'s lymphoma, including Burkitt\'s lymphoma (12; 50.0 per cent ), Hodgkin\'s lymphoma (6; 25.0 per cent ) and Kaposi sarcoma (3; 12.5 per cent ). The age-standardized incidence rate was 1,461.3 cases/million. The trend analyses revealed a significant reduction in the incidence of all cancers (APC= -14.5), driven by the decrease in AIDS-defining cancers (APC= -17.0). The overall risk for cancer was significantly increased (SIR= 3.9), especially for non-Hodgkin lymphoma, excluding Burkitts lymphoma (SIR= 22.5), Burkitt\'s lymphoma (SIR= 29.7) and Hodgkin\'s lymphoma (SIR= 18.7). The overall probability of survival at five years after cancer was 56.3 per cent in children and adolescents with AIDS versus 87.5 per cent in the general population. The hazard ratio for death was 5.2 (95 per cent CI= 2.0, 13.6). The map of the geographical distribution showed a concentration of cases in the low-income areas of the Municipality. Conclusion: There was a marked reduction in the incidence of AIDS-defining cancers, likely to be a result of the introduction of HAART. However, children and adolescents with AIDS remain at increased risk for the development of cancer when compared to the general population. For those who developed cancer, the risk of death was also significantly higher