RESUMEN
BACKGROUND: Diagnostic delay results in relatively high mortality among children with retinoblastoma in Uganda, where treatment was limited to surgery and, for some, radiotherapy. In order to improve outcomes, a simple programme of neoadjuvant and adjuvant chemotherapy was introduced. Here we report survival before and after this change to medical practice. METHODS: Affordable standard off-patent chemotherapy agents were administered by trained paramedical staff to groups of patients at the same time. Survival before and after the introduction of chemotherapy was monitored. Between 2006 and 2013 a total of 270 patients with retinoblastoma were included, 181 treated prior to chemotherapy and 89 after (beginning in 2009). We had 94% follow-up and 249 had histological verification of diagnosis. RESULTS: Using a proportional hazards model adjusted for age, sex and laterality, children treated after chemotherapy was introduced had a 37% lower risk of dying (HR 0.63, 95% CI 0.41 to 0.99) compared with children treated before. Prior to the introduction of chemotherapy only 15% of children who survived bilateral disease retained vision after treatment compared with 71% after chemotherapy. CONCLUSIONS: The introduction of chemotherapy proved safe and cost-effective in non-specialist hands and was associated with significant improvements in survival and, among bilateral cases, in preserving vision.
Asunto(s)
Neoplasias de la Retina/mortalidad , Retinoblastoma/mortalidad , Antineoplásicos/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Carboplatino/uso terapéutico , Quimioterapia Adyuvante , Niño , Preescolar , Diagnóstico Tardío , Supervivencia sin Enfermedad , Etopósido/uso terapéutico , Femenino , Humanos , Lactante , Masculino , Terapia Neoadyuvante , Modelos de Riesgos Proporcionales , Neoplasias de la Retina/tratamiento farmacológico , Neoplasias de la Retina/patología , Retinoblastoma/tratamiento farmacológico , Retinoblastoma/patología , Tasa de Supervivencia , Uganda/epidemiología , Vincristina/uso terapéuticoRESUMEN
AIMS: To characterise the clinical features, treatment and outcome of children diagnosed with retinoblastoma in Uganda. METHODS: The study comprised a 6-year nationwide enrolment with follow-up. RESULTS: In total, 282 cases were enrolled, 26% (72) were bilateral; 6% were lost to follow-up. Almost all diagnoses in the first affected eye were International Classification of Retinoblastoma group E or worse. Histology was available for 92%; of those, 45%, had extraocular tumour at diagnosis. Enucleation of the first eye was done for 271; 94 received radiotherapy to the socket and in the last 2â years, 70 children received chemotherapy. At close of study, 139 children had died. Survival, as determined in a proportional hazards model adjusted for age, sex, laterality and treatment era (pre or post introduction of chemotherapy), varied by extent of the tumour (p<0.001); children with only intraocular involvement were 80% less likely to die (HR=0.21, 95% CI 0.12 to 0.35) compared with children with extraocular involvement. CONCLUSIONS: Diagnostic delay results in relatively high mortality among children with retinoblastoma in Uganda. There is an urgent need for more effective treatment modalities, particularly chemotherapy, and nationwide efforts to encourage earlier access to medical care.
Asunto(s)
Neoplasias de la Retina/diagnóstico , Neoplasias de la Retina/mortalidad , Retinoblastoma/diagnóstico , Retinoblastoma/mortalidad , Antineoplásicos/uso terapéutico , Niño , Preescolar , Radioisótopos de Cobalto/uso terapéutico , Diagnóstico Tardío , Enucleación del Ojo , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Neoplasias de la Retina/terapia , Retinoblastoma/terapia , Tasa de Supervivencia , Uganda/epidemiologíaRESUMEN
UNLABELLED: BACKGROUND IN ADULTS,: HIV increases the risk of certain cancers known, or thought, to have an underlying infectious etiology; the impact on the risk of cancer in children is less clear. METHODS: Here, we report results of an on-going study at four pediatric oncology centers in South Africa in which children diagnosed with cancer are tested routinely for HIV. Odds ratios (OR) for the prevalence of various malignancies were calculated (with adjustment for age, sex, and center) for the children infected with HIV using all children with cancer and non-malignant conditions, but not infected with HIV, as a comparison group. RESULTS: Of 882 children with cancer, 38 were HIV infected (for 12 the HIV status was unknown). HIV was associated with Kaposi sarcoma (all 10 cases were HIV infected; P < 0.001) and with Burkitt lymphoma (OR = 46.2, 95% confidence interval (CI) 16.4-130.3; 13/33). For non-Burkitt non-Hodgkin lymphoma (NHL), 2/39 were HIV infected (OR = 5.0, 95% CI 0.9-27.0). No other cancer type was significantly associated with HIV, including lymphoid leukemias (OR = 0.4, 95% CI 0.04-2.9; 1/172). CONCLUSIONS: Only Kaposi sarcoma and Burkitt lymphoma were significantly associated with HIV infection although results for non-Burkitt NHL were suggestive. Notably, we did not identify an association between infection with HIV and lymphoid leukemias, for which an underlying infectious etiology has been suggested.