[Solid malignant neoplasms in the first year of life]. / Neoplasias sólidas malignas durante el primer año de vida.

BACKGROUND: Solid malignant tumors occurring during the first year of life are rare. In Mexico, cancer is the tenth leading cause of mortality in this age group. However there is insufficient information about the characteristics of these children. This report aims to present our experience in a single hospital. PATIENTS AND METHODS: A retrospective study was carried out in all patients diagnosed with solid malignant tumors during the first year of life who were treated in our institution during the previous 5 years. The variables analyzed were age at diagnosis, sex, birth weight, congenital malformations, time since onset of presenting features, type of neoplasm, treatment modalities, overall survival and sequelae. RESULTS: We analyzed 45 patients; the mean age at diagnosis was 150 days and the time since onset of presenting features ranged from 1-180 days. The most frequent tumors found were neuroblastoma (31%), nephroblastoma (13%) and retinoblastoma (13%). Two thirds of the patients presented with advanced stages. Complete tumoral resection was achieved in 55% of the patients, chemotherapy was administered in 80% and radiotherapy in only 2%. Complications after treatment were observed in 24% of the patients. The overall survival was 81% at 66 months of follow-up. CONCLUSIONS: Because cancer is rare in this age group and most of the patients had advanced disease at diagnosis, pediatricians should be more aware of these tumors to increase their early detection. In addition, multicenter studies should be performed to develop better treatment protocols that would improve prognosis and quality of life in these patients.

A multicentre report from the Mexican Retinoblastoma Group.

BACKGROUND: Retinoblastoma (RB) is a relatively uncommon tumour in childhood. The incidence of retinoblastoma in Mexico is probably higher than the incidence reported worldwide, however there is not enough information about the characteristics of this illness in Mexico. This report aims to present the results of a multicentre clinical survey of RB in Mexico. METHODS: A retrospective study was carried out on all RB cases treated in 16 institutions during the last six years. The variables analysed were age at diagnosis, sex, affected eyes, treatment modalities, and pathological staging. Overall survival was obtained. RESULTS: The authors analysed 500 cases; age range was 0-182 months. There were 364 unilateral cases (72.8%). Enucleation was performed in 84.9% of the patients. The St Jude's staging was: 7.4% stage I, 52.8% stage II, 18.0% stage III, 11.4% stage IV, 7.2% not evaluated, and 3.2% missing data. Chemotherapy was used in 74.4% of the patients. Disease free survival was 89% at 73 months follow up. CONCLUSIONS: The paper presents a great number of cases and pioneers multicentre studies in paediatric ophthalmology and oncology in this country. Given the great number of patients in advanced stages and the variability on treatment schemes, it is evident that it is mandatory to work in a cooperative group and develop a national early detection programme as well as a treatment protocol which include all specialists involved in the care of patients with RB.

Ewing's sarcoma: prognosis and survival in Mexican children from a single institution.

A retrospective analysis of 55 patients with Ewing's sarcoma from an institution in Mexico was done between 1980 and 1993. The ages ranged between 2 and 16 years (mean 9.78); 39 were male and 16 female. The most frequent primary sites were in the humerus in 13 of 55 patients (23.6%), followed by the pelvis in 10 out of 55 (18%). Sixty percent of the patients had metastasic disease at diagnosis; the lungs and bones were the most frequently affected sites. Patients with localized disease (n = 22) had a disease-free survival (DFS) of 44%, compared with 20% of those with pulmonary metastasic disease (n = 7) and 8% of patients with metastasic disease to the lungs and elsewhere (n = 26) (p = .00061). Patients in regimen 3 had a DFS of 47% at 36 months of follow-up compared to 20 and 25% for patients in regimens 1 and 2, respectively (p = .01). In those with trunk presentation the DFS was of 25% and in those with presentation in the extremities DFS was 50% (p = .01). Patients with pulmonary metastasic disease at diagnosis have a DFS of 20% in comparison to those without (44%) (p = .00061).

Is the ancillary chemotherapy approach of any value in the treatment of infratentorial primitive neuroectodermal tumors with surgery and radiotherapy?

A retrospective historical analysis of patients under 18 years of age with the histopathological diagnosis of infratentorial primitive neuroectodermal tumor (PNET) is presented. The survey embraced two different groups of children. Group 1 was defined as those patients treated from 1972 to 1984 with surgical resection plus neuraxis radiotherapy alone. Group 2 was made up of children treated from 1990 to 1996 with the same approach but with the addition of adjuvant chemotherapy: cisplatin (day 1) and etoposide (days 1-3) every 3 weeks for 6 months. Group 1 embraced 42 children with an age range of 1-16 years (mean 6 years, SD 4.4 years). In group 2 there were 34 children, their ages ranging from 1 to 18 years (mean 7.2, SD 4.6 years). The prevalence of stages T2M0 and T3M0 was similar in both groups, but in group 1 there were 4 patients (9.5%) whose spinal fluid was positive for tumor cells (M1), while in group 2 there were 7 children (20.5%) with positive spinal fluid. There was an unequivocal initial response to treatment in 86% of these children in group 1 and in 79% in group 2. The event-free survival (EFS) was 30% at 252 months in group 1, while for group 2 the EFS was 67.6% at 63 months (P 0.002). Mortality from tumor activity was noted in 26 patients (70%) in group 1, while in group 2 mortality attributable to tumor progression was documented in 11 children (32%). We conclude that the use of adjuvant chemotherapy in these patients improves survival without any significant morbidity.

[Epidemiological aspects of bilateral retinoblastoma]. / Los aspectos epidemiológicos en el retinoblastoma bilateral.

Retinoblastoma (RB) is the most frequent solid tumor in the Instituto Nacional de Pediatría, México (INP). The bilateral presentation occurs in 25% of all patients. We present some epidemiological data of this form of the disease, in learn these epidemiological variables in our series. We reviewed the clinical charts from 1972 to 1994. We analyzed age, sex, timing of presentation of the second affected eye, positive family history, clinical staging, and the development of secondary malignancies. A total of 105 cases were detected from 412 RB total cases (25%). We observed a male predominance 1.6:1.0 p = 0.04 versus females. The clinical staging showed: retinal stage 9/102, ocular stage 58/102, orbital stage 26/102 and non classified 9/102 p < 0.00001. The asynchronous form is a rare presentation in our experience: 16/105 cases p = 0.00001 (15%). Only 16/105 patients had a positive family history of RB (15%). We found 2 of 105 cases with secondary malignancies (0.01%). The asynchronous form constitutes a rare presentation. We cannot explain the etiology for the high prevalence of bilateral disease. The frequency of secondary malignancies is lower than that reported in the literature.