RESUMO
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.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Análise Atuarial , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Linfoma não Hodgkin/tratamento farmacológico , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
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
Assuntos
Humanos , Masculino , Criança , Leiomiossarcoma , Leiomiossarcoma/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Neoplasias Hepáticas/diagnóstico , Segunda Neoplasia Primária , Segunda Neoplasia Primária/patologiaRESUMO
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.
Assuntos
Neoplasias Renais/terapia , Equipe de Assistência ao Paciente , Tumor de Wilms/terapia , Criança , Pré-Escolar , Terapia Combinada , Feminino , Humanos , Lactente , Neoplasias Renais/diagnóstico , Neoplasias Renais/epidemiologia , Neoplasias Renais/mortalidade , Masculino , México/epidemiologia , Indução de Remissão , Estudos Retrospectivos , Tumor de Wilms/diagnóstico , Tumor de Wilms/epidemiologia , Tumor de Wilms/mortalidadeRESUMO
Intracardiac tumor extension from nephroblastoma is a rare event. We report on two cases with this peculiar condition who presented with a different set of signs and symptoms. Both were diagnosed in life but only one could be properly managed on time. Emphasis is made upon the most reliable methodology for early detection and the surgical approach as the only plausible way to solve this particular complication.