Overall survival and cause-specific mortality in a hospital-based cohort of retinoblastoma patients in São Paulo, Brazil.

Studies are lacking on long-term effects among retinoblastoma patients in low- and middle-income countries. Therefore, we examined cause-specific mortality in a retrospective cohort of retinoblastoma patients treated at Antonio Candido de Camargo Cancer Center (ACCCC), São Paulo, Brazil from 1986 to 2003 and followed up through December 31, 2018. Vital status and cause of death were ascertained from medical records and multiple national databases. We estimated overall and cause-specific survival using the Kaplan-Meier survival method, and estimated standardized mortality ratios (SMRs) and absolute excess risk (AER) of death. This cohort study included 465 retinoblastoma patients (42% hereditary, 58% nonhereditary), with most (77%) patients diagnosed at advanced stages (IV or V). Over an 11-year average follow-up, 80 deaths occurred: 70% due to retinoblastoma, 22% due to subsequent malignant neoplasms (SMNs) and 5% to non-cancer causes. The overall 5-year survival rate was 88% consistent across hereditary and nonhereditary patients (p = .67). Hereditary retinoblastoma patients faced an 86-fold higher risk of SMN-related death compared to the general population (N = 16, SMR = 86.1, 95% CI 52.7-140.5), corresponding to 42.4 excess deaths per 10,000 person-years. This risk remained consistent for those treated with radiotherapy and chemotherapy (N = 10, SMR = 90.3, 95% CI 48.6-167.8) and chemotherapy alone (N = 6, SMR = 80.0, 95% CI 35.9-177.9). Nonhereditary patients had only two SMN-related deaths (SMR = 7.2, 95% CI 1.8-28.7). There was no excess risk of non-cancer-related deaths in either retinoblastoma form. Findings from this cohort with a high proportion of advanced-stage patients and extensive chemotherapy use may help guide policy and healthcare planning, emphasizing the need to enhance early diagnosis and treatment access in less developed countries.

Role of non-chromosomal birth defects on the risk of developing childhood Hodgkin lymphoma: A Children's Oncology Group study.

BACKGROUND: Non-chromosomal birth defects are an important risk factor for several childhood cancers. However, these associations are less clear for Hodgkin lymphoma (HL). Therefore, we sought to more fully elucidate the association between non-chromosomal birth defects and HL risk. PROCEDURE: Information on cases (n = 517) diagnosed with HL (ages of 0-14) at Children's Oncology Group Institutions for the period of 1989-2003 was obtained. Control children without a history of cancer (n = 784) were identified using random digit dialing and individually matched to cases on sex, race/ethnicity, age, and geographic location. Parents completed comprehensive interviews and answered questions including whether their child had been born with a non-chromosomal birth defect. To test the association between birth defects and HL risk, conditional logistic regression was applied to generate adjusted odds ratios (aORs) and 95% confidence intervals (CIs). RESULTS: Children born with any non-chromosomal birth defect were not more likely to be diagnosed with HL at 0-14 years of age (aOR: 0.91; 95% CI: 0.69-1.21). No associations were detected between major or minor birth defects and HL (aOR: 1.34; 95% CI: 0.67-2.67 and aOR: 0.88; 95% CI: 0.57-1.34, respectively). Similarly, no association was observed for children born with any birth defect and EBV-positive HL (aOR: 0.57; 95% CI: 0.25-1.26). CONCLUSIONS: Previous assessments of HL in children with non-chromosomal birth defects have been limited. Using data from the largest case-control study of HL in those <15 years of age, we did not observe strong associations between being born with a birth defect and HL risk.

Linfoma de Hodgkin clássico na infância. Experiência de tratamento e fatores prognósticos / Classical Hodgkin?s lymphoma in childhood. Therapeutic experience and prognostic factors

Objetivos: Estimar as taxas de sobrevida global e livre de eventos em portadores de linfoma de Hodgkin (LH), bem como identificar fatores prognósticos. Métodos: Estudo de coorte retrospectivo, incluindo variáveis demográficas, laboratoriais, tipo histológico, estadiamento e tratamento de 107 pacientes menores de 18 anos de idade admitidos no Departamento de Pediatria do Centro de Tratamento e Pesquisa Hospital do Câncer, no período entre 1985 e 1995. Resultados: Dos pacientes, 81 (76%) eram do sexo masculino e 80% da raça branca. A média de idade foi 10 anos (2 a 18 anos). Adenomegalia cervical foi a principal queixa referida (68% dos pacientes) e 55% apresentavam tempo de queixa menor que seis meses. Os subtipos EN e CM foram encontrados em 43% e 41% dos casos, respectivamente. Os estádios clínicos II e III foram os mais frequentes (33% cada um). Os sítios metastáticos mais frequentes nos EC IV foram fígado (42%) e pulmão (38%). As taxas de SG e SLE em 10 anos foram de 82,4% e 82,5%, respectivamente. O estádio clínico se mostrou como fator prognóstico significativo para as SG e SLE. A análise univariada revelou a presença de sintomas B, nível de Hb £ 9,3 g/dl, leucócitos £ 6.100 mm3, plaquetas £ 274.000/mm3 e ocorrência de recaída como fatores de mau prognóstico, enquanto a análise múltipla mostrou como fatores prognósticos independentes a presença de sintomas B e contagem de plaquetas. Conclusões: A identificação de fatores prognósticos é valiosa para a adequada estratificação dos pacientes em grupos de risco, adequando-os a esquemas de tratamento que maximizem as taxas de cura e minimizem os efeitos colaterais tardios.