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.

[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.

[Wilms' tumor. Its multidisciplinary management]. / Tumor de Wilms. Manejo multidisciplinario.

A total of 115 children with a histopathological diagnosis of Wilms' tumor were studied. The average age was three years. An abdominal tumor was the most frequent clinical manifestations, with a predominating clinicopathological stage II. The most important prognostic factors were the clinical stage and histological subvariety. A five year disease free period during the early stages was very favorable. On the other hand, advances stages and unfavorable histopathology established a poor prognosis. In our experience, stages I and II and favorable histology should not receive radiotherapy but instead brief chemotherapy. The global five year survival was 82%. All the patients with an unfavorable histology occupied stages II and IV. a comparison of disease free survival between stages I and II against III and IV showed statistical significance (p 0.01). Statistical significance also appeared upon comparison between unfavorable versus favorable (p 0.01) histology. Emphasis is placed upon multidisciplinary management of this type of malignant neoplasias.

Treatment of non-Hodgkin's lymphoma in Mexican children. The effectiveness of chemotherapy during malnutrition.

The histological diagnosis of non-Hodgkin's lymphoma (Burkitt's lymphoma excluded) in 147 children was reviewed. The most common site of presentation was in the abdomen (32.6%). The most frequent site of metastatic disease at diagnosis was the bone marrow (27.2%). The most common histology was diffuse undifferentiated non-Burkitt type (37.4%). According to the Murphy staging system, 40.1% were stage III and 27.2% were stage IV. In a nonrandomized prospective study, 121 patients were submitted to a treatment regimen (protocol 8001) and compared with 26 historical controls treated with the COP regimen, consisting of cyclophosphamide, vincristine, and prednisone. Of those patients treated with protocol 8001, nine had intestinal perforation at the site of primary disease. All patients in this group were malnourished at the time of perforation. The overall rate of initial complete remission in those patients treated with protocol 8001 was 90.7%. The duration of remission was from 16 to 108 months, with a median of 39 months. The actuarial rate of disease-free survival was 69% at 2 years and 63% at 6 years, compared with 36% at 6 years of the control group (COP) (p less than 0.01). None of the patients have relapsed after 4 years.