[Survival and clinical features of retinoblastoma]. / Supervivencia y aspectos clínicos del retinoblastoma.

INTRODUCTION: Retinoblastoma (RB) is the most frequent intraocular tumor in the pediatric age group, representing 3 % of all childhood neoplasms. In Mexico, the annual incidence varies according to the geographical area studied, ranging from 4-24 cases/million in patients younger than 15 years. Ninety-five percent of cases occur in infants and pre-school children. PATIENTS AND METHOD: An observational, retrospective, descriptive, cross-sectional study of all children with RB admitted to the Oncology Department of the Pediatric Hospital of the National Medical Center in a 10-year period was performed. RESULTS: Fifty-six patients were included. The median age was 24 months. Forty-two children presented unilateral RB. Symptoms at diagnosis were leukokoria, strabismus and reduced visual sharpness. Distribution according to Prats' classification was the following: stage I, 5 patients; stage II, 32; stage III, 14; stage IV, 5. Histological analysis revealed 24 patients with poorly differentiated RB, 16 with well-differentiated RB, 13 with moderately differentiated RB and 7 with undifferentiated RB. In eight patients histological type was not identified. In 12 of the 56 patients cytogenetic and molecular studies were performed. All patients underwent surgery. Patients in the early stages were treated with chemotherapy. A total of 26.7 % of the group presented relapse. The disease-free survival at 10 years was 87.5 %. CONCLUSIONS: As with other childhood neoplasms, early diagnosis and multidisciplinary management of RB has improved prognosis and survival. The symptoms, treatment and survival reported in this pediatric cohort are similar to those of other published series.

Supervivencia y aspectos clínicos del retinoblastoma / Survival and clinical features of retinoblastoma

Introducción El retinoblastoma es el tumor intraocular maligno más frecuente en pediatría. Representa el 3 per cent de las neoplasias de la infancia. En México, la incidencia anual varía de acuerdo con la zona geográfica estudiada en un rango de 4 a 24 casos por millón en menores de 15 años y el 95 per cent de los casos se presenta en la etapa de lactante y preescolar. Pacientes y método Estudio observacional, retrospectivo, descriptivo y transversal. Se incluyeron todos los niños con retinoblastoma atendidos en 10 años en el Servicio de Oncología del Hospital de Pediatría del Centro Médico Nacional IMSS. Resultados Se incluyeron 56 pacientes. La mediana de edad fue de 24 meses. Presentaron retinoblastoma unilateral 42 casos. Los síntomas presentes al diagnóstico fueron: leucocoria, estrabismo y disminución de la agudeza visual. La distribución de acuerdo con la clasificación de Pratt fue: etapa I, 5; etapa II, 32; etapa III, 14; etapa IV, 5. Por histología: 24 pacientes con retinoblastoma pobremente diferenciado, 16 bien diferenciados y 13 moderadamente diferenciados, 7 casos no diferenciados y en 8 casos la variedad histológica no se identificó. En el grupo de pacientes estudiados 12 de los 56 se incluyeron en un protocolo de estudio citogenético y molecular. Todos los pacientes se sometieron a cirugía. La quimioterapia se indicó en los estadios tempranos. El 26,7 per cent presentaron recidivas. La supervivencia libre de enfermedad fue del 87,5 per cent a 10 años. Conclusiones El retinoblastoma al igual que en otras neoplasias en pediatría, el diagnóstico oportuno y el manejo multidisciplinario ha mejorado el pronóstico y la supervivencia. Los síntomas, el tratamiento y la supervivencia de la presente cohorte son similares a lo comunicado en la literatura (AU)

[Neurological and psychological sequelae in children with acute lymphoblastic leukemia who had received radiotherapy and intrathecal methotrexate]. / Secuelas neurológicas y psicológicas que deja la radioterapia o el metotrexate intratecal en niños con leucemia linfoblástica aguda.

UNLABELLED: Our objective was to compare the neurologic and psychological sequelae of children with acute lymphoblastic leukemia (ALL) after three or more years without antineoplasic treatment who underwent cranial irradiation plus intrathecal methotrexate (Group 1) or just intrathecal methotrexate (Group 2). In both groups, a neurologic evaluation, electroencephalogram (EEG) and cranial computed tomography (CCT) were performed. Intellectual quotient and the Bender test were done for the psychological evaluation. Investigators did not know the kind of treatment of each patient. STATISTICS: Fisher's exact test and Mann-Whitney U. There were fourteen patients in group 1 and eight patients in group 2. Intellectual quotient was statistically lower in the first group (median 83.5) than in the second (90.5). Neurologic impairments were found in one patient of each group, alterations of the EEG were found in 6 and 4 in group 1 and 2, respectively, and in the CCT of two patients in group 2 without statistical difference. Children with ALL after cranial irradiation have a greater alteration of intellectual performance than children with intrathecal methotrexate. Neurologic alterations were seen in both groups.

Survival of patients with medulloblastoma treated with carboplatin and etoposide before and after radiotherapy.

BACKGROUND: Medulloblastoma represents 20% of all tumors of the central nervous system. Patients with partial resection of the tumor and those with extension into the neuraxis at diagnosis have been identified as high-risk patients. The objective of our study was to determine tumor response, survival rates and toxicity with a new scheme of treatment with carboplatin, etoposide and radiotherapy. METHODS: All patients received chemotherapy with carboplatin and etoposide every 4 weeks for four courses, hyperfractionated radiotherapy, and another four courses of the above chemotherapy scheme. Tumor response was classified, and global and disease-free survival rates were calculated according to the actuarial survival method. RESULTS: A total of 26 patients were included, with a median age of 6.9 years. Nineteen achieved complete response after the first four courses of chemotherapy, and two more had a complete response after radiotherapy. A total of seven children have died, three of whom did not respond to initial treatment. Global and disease-free survival rates were 69% and 64%, respectively, at 60 months of follow-up. There was no renal or auditory toxicity. Hematological toxicity was transitory and reversible. CONCLUSIONS: This scheme of treatment is effective and can be safely used for pediatric patients with high-risk medulloblastomas. Toxicity was not significant, and survival is similar to other reports.

[Astrocytomas in pediatrics. The prognostic factors and survival]. / Astrocitomas en pediatría. Factores pronósticos y sobrevida.

Tumors of the central nervous system are the second most frequent malignancy in children under the age of 15 the majority of which are astrocytomas. The purpose of this paper is to analyze the effect of some factors on the survival of patients with astrocytomas. We reviewed the clinical charts of patients with a diagnosis of astrocytoma from 1984 to 1995 and we analyzed the age, sex, location, malignant grade and survival according to the actuarial method and log rank tests. A total of 39 patients were detected, 43% were supratentorial, 13% cerebellar and 43% from the brain stem. There was a better prognosis for those located supratentorial or cerebellar with respect to those in the brain stem, with statistical significance. The treatment consisted in surgical resection in 26 patients, 17 of which were found to have a high grade of malignancy and 9 with low grade, with statistically better prognosis for low grade tumors. There were 17 deaths during the period, with 8 of these patients having a high grade tumor a 10, with brain stem tumors. Forty-one percent of the patients that died did so in the first year and 94% during the first two years after diagnosis. Patients with cerebellar astrocytoma and patients with low-grade astrocytomas had the best prognosis.

[Instrument to measure quality of life based on daily performance in pediatric patients with leukemia]. / Un instrumento para medir la calidad de vida por medio del desempeño diario en pacientes pediátricos con leucemia.

The objective of this study was to determine the validity of a questionnaire to measure daily performance as an evaluation of quality of life in pediatric leukemia patients. A questionnaire of the daily activities of a child according to Lickert's criteria was done. A pilot survey was performed in patients without a chronic or disabling disease in order to assess its validity. The impression of the medical attendance of each pediatric patient with leukemia, of their daily performance, was compared with the results of the instrument. A trained nurse applied the questionnaire to 60 mothers of patients (29 female/31 male) as outpatients during the clinical review. Fifteen questionnaires were applied twice to evaluate its consistency. According to the results, 77% of the patients were in remission. 15% in relapse, and 8% during induction to remission. The mean age of the patients was 9 = 3.6 years, ranging from 2-16 years. One hundred percent of the patients obtained a satisfactory grade greater than 35 points. In conclusion, the questionnaire identified adequately the level of the daily performance in addition to the specific affected areas of the patients with leukemia. Our findings are that their level of performance is satisfactory and is not affected because of the disease or the phase of the treatment.