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.

Is autologous bone marrow transplant (ABMT) and high-dose chemotherapy an approach that can rescue some children with advanced cancer disease?

The object was to determine the role ABMT in children with advanced cancer Those included had failed to respond to conventional treatment with 4 different ablative chemotherapy regimens. Bone marrow stem cells were identified with CD34. Cellular viability was determined after the bone marrow extraction and before the infusion. Fifteen patients were included, whose ages ranged from 1 to 13 years old with a median of 7. Six had acute leukemia, 6 with primitive neuroectodermic tumors, and 3 with other tumors. The median disease-free survival for the whole group was of 2 months, range of 1 to 29 months and SD of 10.1. A total of 6 children are alive (40%) and without evidence of tumor activity from 1 to 29 months. The disease-free survival rate for these group was of 19.1 months, with an SD of 7.9 months.

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.

B-lineage acute lymphoblastic leukemia of childhood. An institutional experience.

A total of 119 children (1990-95) with acute lymphoblastic leukemia (ALL) B-lineage either CD10+ or CD10- were registered into a single non-randomized chemotherapy protocol. Only untreated patients with standard risk were included in the study. Their ages ranged from 1.8-10 years with a mean of 5.1 years. There were 82 (68%) children with early pre B-All, 35 (29%) with pre B-ALL and 2(1.6%) with transitional pre B-ALL (p < 0.00001). The patients were divided according to CD10 reactivity, either + (94 children) or -(25 patients). The event-free survival (EFS) at 60 months for the CD10+ children was of 78% (alive 73/94), while for the CD10- was 71% (alive 18/25) (p = 0.6) and 74% for both groups. The factors that influenced favorably the survival in the CD10+ group were the age between 3 to 5.99 years (p < 0.00001), sex (either male or female), leukocyte count between 10-24.9 x 10(9)/l (p < 0.00001), LDH under 300 U/I (p < 0.00001) and L1 bone marrow cytomorphology (p < 0.00001). In the CD10- patients, the EFS was favorably influenced by the female sex (p = 0.04), leukocyte count under 10 x 10(9)/l (p = 0.05) and LDH < 300 U/l (p = 0.02). CNS infiltration was documented in 4.2% (5/119). Mortality secondary to chemotherapy was seen in 7%. In conclusion, this is the first large series in Mexican children with B-lineage ALL published. Because of the relatively small number of patients in each group (pre B and transitional pre B), all the patients in the current series were treated alike. When the 119 patients were divided only on the basis of CD10 reactivity, the EFS for both groups (CD10+ and-) was similar; therefore, the reactivity to CD10 has no prognostic value in this type of ALL.

[Lymphoblastic lymphoma in children. Poor response in advanced disease with chemotherapy for non-Hodgkin's lymphoma]. / Linfoma linfoblástico en niños. Respuesta pobre en enfermedad avanzada con quimioterapia de linfoma no Hodgkin.

Fifty three pediatric patients with the histopathological diagnosis of lymphoblastic lymphoma (LL) were studied in a retrospective analysis during a 14 year period. Their age ranged from 1 to 16 years with a median of 7 years. Clinical staging was performed according to Murphy's system. There was one child in stage I (2%), 11 in stage II (21%), 14 stage III (26%) and 27 stage IV (51%). Patients in stage IV, 21 (78%) had initial bone marrow involvement, 4 (15%) central nervous system (CNS) infiltration and 2 (7%) simultaneous infiltration to the bone marrow and the CNS. The chemotherapy program consisted of induction, consolidation and maintenance with CNS prophylaxis. The whole program lasted 36 months. Out of 53 patients there were only 45 evaluable for treatment analysis response. A total of 14 (31%) are alive and in a continuous complete remission, with a median duration of remission of 66 months, 8 (18%) children abandoned treatment with a median duration of remission of 10 months. Twenty three patients (51%) are dead. The actuarial survival at 11 year is of 39% +/- 11% with a median remission rate for the whole group of 11.8 months. No patient in complete remission for more than 24 months has relapsed. We conclude that our chemotherapy program is more than adequate for early stages, but for advanced disease it has been a failure. There is a need to modify the chemotherapy program using a very similar protocol as the one used in high risk childhood acute lymphoblastic leukemia.

Leiomiosarcoma hepático como segunda neoplasia: informe sobre un niño con leucemia aguda linfoblástica y tumor hepático / Hepatic leiomyosarcoma as a second malignancy: report in a child with acute lymphoblastic leukemia and hepatic mass

Los pacientes pediátricos con leucemia aguda linfoblástica (LAL) presentan alto índice de curación, pero con mayor incidencia de segundas neoplasias condicionadas por la radioterapia, por la quimioterapia con agentes alquilantes o las epipodofilotoxinas. Se presenta un paciente con LAL en el Instituto Nacional de Pediatría, quien durante el tratamiento de LAL sin presentar alteración citogénica demostrable, desarrolla un leiomiosarcoma hepático de focos primarios múltiples, no existieron antecedentes de uso de manera importante de agentes alquilantes, epipodofilotoxinas ni radiaciones ionizantes. Consideramos la posibilidad de una susceptibilidad genética, que no podemos demostrar actualmente, como condicionante para el desarrollo de esta segunda neoplasia con patrón de presentación clínica poco usual