RESUMO
This is a retrospective study of 55 children under the age of 2 years diagnosed with Langerhans cell histiocytosis (LCH). They were classified according to age and organ function and dysfunction following Lahey's criteria. The studied population was divided into four groups by age of diagnosis (0-6, 7-12, 13-18, and 19-24 months). Statistical analysis showed no significant difference in outcome between age groups, although the population under 6 months had a 81.3% fatality rate. The presence of organ dysfunction was a major cause of death in all age groups, being statistically significant in outcome (P > 0.005) compared with patients without organ dysfunction. The presence of thrombocytopenia and/or respiratory dysfunction was also highly associated with a fatal outcome. In the surviving population, no second malignancies have been reported. The late secondary effects of therapy include endocrine, orofacial, and osseous pathologies.
Assuntos
Histiocitose de Células de Langerhans/epidemiologia , Fatores Etários , Causas de Morte , Feminino , Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/mortalidade , Humanos , Lactente , Masculino , México/epidemiologia , Insuficiência de Múltiplos Órgãos/etiologia , Insuficiência de Múltiplos Órgãos/mortalidade , Vigilância da População , Insuficiência Respiratória/etiologia , Estudos Retrospectivos , Trombocitopenia/etiologiaRESUMO
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.
Assuntos
Átrios do Coração/cirurgia , Neoplasias Cardíacas/secundário , Neoplasias Renais/cirurgia , Células Neoplásicas Circulantes , Nefrectomia , Tumor de Wilms/secundário , Criança , Pré-Escolar , Terapia Combinada , Feminino , Átrios do Coração/patologia , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/cirurgia , Humanos , Neoplasias Renais/patologia , Masculino , Necrose , Tumor de Wilms/patologia , Tumor de Wilms/cirurgiaRESUMO
A total of 17 patients with meningeal sarcoma were diagnosed and treated at the National Institute of Pediatrics in Mexico City in a period of 15 years. Among the diagnostic methodology used in this group we found that angiography is still the best to be used so far. On the other hand, the chemotherapy protocol employed did not improve the survival obtained with surgery and radiotherapy. Therefore we suggest that a new chemotherapy protocol has to be designed in order to obtain better results. Of particular interest, we found in this group of patients that the time elapsed between the first sign of disease to the moment of diagnosis varied from 2 months to 10 years without any prognostic significance.
Assuntos
Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Neoplasias Meníngeas/terapia , Meningioma/terapiaRESUMO
We cared for 124 pediatric patients with a histologic diagnosis of Langerhans' cell histiocytosis (histiocytosis X) over a period of 14 years. Clinical, laboratory, and radiographic findings were analyzed. The most frequent manifestations were bone lesions, lymph node involvement, and skin infiltration. Liver disease was noted in 50% of patients and lung disease in 23%; hematologic changes were also frequent. Dysfunction and involvement of these three organ systems, plus age of onset, distinguished the group of patients with the highest mortality. All patients with generalized disease or organ dysfunction were treated with systemic chemotherapy. The actuarial survival curve at 10 years was 63%.
Assuntos
Histiocitose de Células de Langerhans , Adolescente , Fatores Etários , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doenças Ósseas/tratamento farmacológico , Doenças Ósseas/patologia , Criança , Pré-Escolar , Ciclofosfamida/administração & dosagem , Feminino , Histiocitose de Células de Langerhans/tratamento farmacológico , Histiocitose de Células de Langerhans/patologia , Humanos , Lactente , Recém-Nascido , Doenças Linfáticas/tratamento farmacológico , Doenças Linfáticas/patologia , Masculino , México , Prednisona/administração & dosagem , Procarbazina/administração & dosagem , Prognóstico , Dermatopatias/tratamento farmacológico , Dermatopatias/patologia , Vincristina/administração & dosagemRESUMO
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.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma não Hodgkin/tratamento farmacológico , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Asparaginase/administração & dosagem , Criança , Pré-Escolar , Ensaios Clínicos como Assunto , Ciclofosfamida/administração & dosagem , Citarabina/administração & dosagem , Feminino , Humanos , Hidrocortisona/administração & dosagem , Perfuração Intestinal/etiologia , Linfoma não Hodgkin/complicações , Masculino , Mercaptopurina/administração & dosagem , Metotrexato/administração & dosagem , Distúrbios Nutricionais/complicações , Prednisona/administração & dosagem , Vincristina/administração & dosagemRESUMO
Thirty patients with Burkitt's lymphoma sporadic type are presented. All of these cases fulfilled the histologic criteria of Burkitt's tumor. The age ranged from 2 to 12 years, with a median of 4 years. Fever and chills were the most common symptoms. The abdomen was the site of initial presentation in more than half of these cases. Close to one-third of the patients had involvement of the central nervous system. Fifty percent of the patients presented with lactic dehydrogenase elevation. Uric acid was elevated in close to one-fourth of these patients. The sonogram was the most useful technique in those instances of abdominal presentation. All patients were staged according to Ziegler criteria after an initial surgical diagnostic procedure was performed. None of the patients received radiotherapy to the primary tumor. All were submitted to a chemotherapy program for 12 months. Only 26 fitted the criteria of being evaluable. The duration of remission was from 1 to 144 months, with a median of 18.2 months. The actuarial survival was 84% at 3 years. Most of the responders corresponded to Stages A, B, and AR, while Stages C and D had a high mortality rate with elevated lactic dehydrogenase and uric acid levels.
Assuntos
Linfoma de Burkitt/fisiopatologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Aspartato Aminotransferases/sangue , Linfoma de Burkitt/tratamento farmacológico , Linfoma de Burkitt/enzimologia , Linfoma de Burkitt/mortalidade , Criança , Pré-Escolar , Feminino , Humanos , L-Lactato Desidrogenase/sangue , Masculino , Ácido Úrico/metabolismoRESUMO
Se sometieron 38 pacientes pediatricos con diagnostico citologico en medula osea de leucemia aguda linfoblastica tipo L1, de acuerdo a la clasificacion del grupo Franco-Americano-Britanico (FAB) a un protocolo de tratamiento disenado en el Servicio de Oncologia. Previamente al inicio de tratamiento todos los pacientes fueron clasificados en bajo o alto riesto segun parametros ampliamente conocidos. Se efectuo correlacion entre sobrevida y factores pronosticos. El 21% de estos pacientes desarrollaron enfermedad extramedular principalmente al sistema nervioso central a pesar de tratamiento profilactico convencional. Dos pacientes presentaron infiltracion testicular. El 94.7% de los pacientes tuvieron una respuesta completa al esquema de induccion a la remision. Al momento de este reporte 23 pacientes se encuentran vivos y en remision, de estos, 10 ya terminaron el tratamiento de 36 meses encontrandose en vigilancia exclusivamente. Consideramos que aun cuando el numero de pacientes de este estudio es reducido, los resultados de tratamiento son comparables con cualquier otra serie similar