Reacción de fototoxicidad por metotrexato / Photoxicity reaction due to methotrexate

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[Neonatal tumors: clinical and therapeutic characteristics. Analysis of 72 patients in La Fe University Children's Hospital in Valencia (Spain)]. / Tumores neonatales: características clínicas y terapéuticas. Análisis de 72 casos del hospital infantil La Fe de Valencia.

BACKGROUND: Neonatal tumors, which represent only 1.5-2% of all pediatric tumors, have distinctive features. Their incidence is imprecise, as selection criteria vary and there are few published studies. OBJECTIVE: To analyze and disseminate findings on the histological, clinical, therapeutic and follow-up characteristics of neonatal tumors. MATERIAL AND METHODS: We performed a retrospective study of the clinical records of patients diagnosed with neonatal tumors in La Fe University Children's Hospital in Valencia (Spain) between January 1990 and December 1999. Hamartomas, flat and cavernous cutaneous hemangiomas, nevi, lipomas, subcutaneous fibroma, lymphangiomas, and epidermal cysts were excluded. A systematic review of the literature published in the previous 25 years was performed using Medline, Cancerlit, Index Citation Science, and Embase. The search profile combined neonatal or congenital and tumor or cancer or neoplasm. The most interesting studies, as well as the most relevant references contained in these studies and published before the search period, were selected. RESULTS: The clinical records of 72 patients with neonatal tumors (40 boys and 32 girls), representing 2.8% of all pediatric tumors, were reviewed. The most frequent tumors were hemangiomas (20.8%, 15 patients), neuroblastomas (16.7%, 12 patients), teratomas (12.5 %, 9 patients), and soft tissue tumors (9.7 %, 7 patients). Eighty-six percent of the patients were symptomatic during the first week of life. Although diagnosis was prenatal in 22.2 % of the patients, the most frequent findings on physical examination were identification of a mass or cutaneous lesion in 24 patients (33.3%) and an abdominal mass or hepatomegaly in 13 patients (18%). An associated disease, malformation or syndrome was found in 15 patients (20.8 %). Treatment included surgery (50% of patients) and drugs as monotherapy or coadjuvant therapy (13.9%). Thirteen patients received irradiation after the neonatal period. Spontaneous complete remission took place in six patients. Twenty patients died (27.8%). Mortality was highest in patients with central nervous system tumors or leukemias (83.3% and 75 % respectively). By contrast, none of the patients with hemangiomas or teratomas died. Mortality was low in patients with neuroblastoma (8.3%). At the time of the study, survival was 73 %, with a median follow-up of 8 years. CONCLUSIONS: Due to their biological features, neonatal tumors represent a distinctive subgroup in pediatric oncohematology. The concept of neonatal tumor should be unified to allow the results of different research groups to be analyzed and compared. Despite the methodological limitations found, the clinical, diagnostic, therapeutic, and follow-up characteristics of our patients are similar to those of other published series. The differences found could be explained by the diverse selection criteria employed.

Tumores neonatales: características clínicas y terapéuticas. Análisis de 72 casos del hospital infantil La Fe de Valencia / Neonatal tumors: clinical and therapeutic characteristics. Analysis of 72 patients in La Fe University Children’s Hospital in Valencia (Spain)

Antecedentes Los tumores neonatales, aunque sólo representan el 1,5-2% de los tumores pediátricos, constituyen un subgrupo con personalidad propia. La incidencia es imprecisa, por los criterios de selección variables, y por los escasos estudios publicados. Objetivo Analizar y divulgar las características histológicas, clínicas, terapéuticas y evolutivas de los tumores neonatales. Material y métodos Estudio retrospectivo de las historias clínicas de los pacientes con tumor neonatal, atendidos en el Hospital Infantil Universitario La Fe de Valencia desde enero de 1990 a diciembre de 1999. Se excluyeron los hamartomas, hemangiomas cutáneos planos y cavernosos, nevos, lipomas, fibromas subcutáneos, linfangiomas y quistes epidérmicos. Revisión bibliográfica sistemática de los últimos 25 años obtenida del Medline, Cancerlit, Index Citation Science y Embase. El perfil de búsqueda utilizado fue la combinación de “neonatal, congenital” y “tumors, cancer, neoplasms”. Se seleccionaron los trabajos más interesantes y, de sus referencias, se han recuperdo las más relevantes publicadas previamente al período de la búsqueda. Resultados Se recogieron 72 pacientes con tumor neonatal (40 varones y 32 mujeres), que representan el 2,8% del total de pacientes diagnosticados con tumores pediátricos de nuestra institución. Los hemangiomas son los tumores neonatales más frecuentes (20,8%, 15 casos), seguidos por los neuroblastomas (16,7%, 12 casos), teratomas (12,5%, 9 casos) y los tumores de partes blandas (9,7 %, 7 casos). En el 86 % de los casos las manifestaciones clínicas aparecieron durante la primera semana de vida. Aunque en 16 casos (22,2%) el diagnóstico fue prenatal, la sintomatología predominante fue la visualización de una tumoración o lesión cutánea en 24 casos (33,3 %) y la presencia de una masa abdominal o hepatomegalia en 13 casos (18 %). En 15 casos (20,8 %) existía alguna malformación, enfermedad o síndrome asociado. De las diferentes modalidades terapéuticas, la cirugía fue la opción más empleada (50%). En el 13,9 % de los casos se administró quimioterapia, en monoterapia o con carácter coadyuvante. Únicamente 3 pacientes recibieron radioterapia fuera del período neonatal. En 6 casos se evidenció la regresión espontánea completa de la tumoración. Fallecieron 20 pacientes (27,8 %). Por grupos histológicos, la mayor mortalidad correspondió al grupo de pacientes con tumores del sistema nervioso central (83,3 %) y al de las leucemias (75 %). Por el contrario, ninguno de los pacientes afectados de hemangiomas y teratomas falleció, destacando así mismo la baja mortalidad de los neuroblastomas (8,3 %). En el momento del análisis la supervivencia en nuestra serie es del 73 %, con una mediana de seguimiento de 8 años. Conclusiones Los tumores neonatales, por sus características biológicas constituyen un subgrupo específico dentro de la oncohematología pediátrica. Es necesario unificar el concepto de tumor neonatal para poder analizar y comparar las diferencias de las distintas series publicadas. A pesar de las limitaciones metodológicas encontradas, las características clínicas, diagnósticas, terapéuticas y evolutivas de nuestra serie son similares a las publicadas por otros autores. Probablemente, las diferencias encontradas puedan explicarse por los diversos criterios de selección de los diferentes estudios

Background Neonatal tumors, which represent only 1.5-2% of all pediatric tumors, have distinctive features. Their incidence is imprecise, as selection criteria vary and there are few published studies. Objective To analyze and disseminate findings on the histological, clinical, therapeutic and follow-up characteristics of neonatal tumors. Material and methods We performed a retrospective study of the clinical records of patients diagnosed with neonatal tumors in La Fe University Children’s Hospital in Valencia (Spain) between January 1990 and December 1999. Hamartomas, flat and cavernous cutaneous hemangiomas, nevi, lipomas, subcutaneous fibroma, lymphangiomas, and epidermal cysts were excluded. A systematic review of the literature published in the previous 25 years was performed using Medline, Cancerlit, Index Citation Science, and Embase. The search profile combined “neonatal or congenital” and “tumor or cancer or neoplasm”. The most interesting studies, as well as the most relevant references contained in these studies and published before the search period, were selected. Results The clinical records of 72 patients with neonatal tumors (40 boys and 32 girls), representing 2.8% of all pediatric tumors, were reviewed. The most frequent tumors were hemangiomas (20.8%, 15 patients), neuroblastomas (16.7%, 12 patients), teratomas (12.5 %, 9 patients), and soft tissue tumors (9.7 %, 7 patients). Eighty-six percent of the patients were symptomatic during the first week of life. Although diagnosis was prenatal in 22.2 % of the patients, the most frequent findings on physical examination were identification of a mass or cutaneous lesion in 24 patients (33.3%) and an abdominal mass or hepatomegaly in 13 patients (18%). An associated disease, malformation or syndrome was found in 15 patients (20.8 %). Treatment included surgery (50% of patients) and drugs as monotherapy or coadjuvant therapy (13.9%). Thirteen patients received irradiation after the neonatal period. Spontaneous complete remission took place in six patients. Twenty patients died (27.8%). Mortality was highest in patients with central nervous system tumors or leukemias (83.3% and 75 % respectively). By contrast, none of the patients with hemangiomas or teratomas died. Mortality was low in patients with neuroblastoma (8.3%). At the time of the study, survival was 73 %, with a median follow-up of 8 years. Conclusions Due to their biological features, neonatal tumors represent a distinctive subgroup in pediatric oncohematology. The concept of neonatal tumor should be unified to allow the results of different research groups to be analyzed and compared. Despite the methodological limitations found, the clinical, diagnostic, therapeutic, and follow-up characteristics of our patients are similar to those of other published series. The differences found could be explained by the diverse selection criteria employed

[Risk factors associated with neonatal tumors. Experience of a pediatric environmental health specialty unit in Valencia (Spain)]. / Factores de riesgo asociados a los tumores neonatales. Experiencia de una unidad de salud medioambiental pediátrica (PEHSU-Valencia).

BACKGROUND: Neonatal tumors (NT) result from a variable combination of constitutional and environmental determinants. Multiple risk factors (RF) are involved in their development, although most are unknown. OBJECTIVE: To document the constitutional, environmental, preconceptional and transplacental RF associated with the development of NT with greater or lesser scientific evidence. PATIENTS AND METHODS: We investigated known RF in NT diagnosed at La Fe University Children's Hospital from January 1990 to December 1999, using a questionnaire completed by parents in a personal interview, either at the Pediatric Environmental Health Specialty Unit (PEHSU-Valencia) or at their home. RF associated with NT and childhood cancer were identified through a literature review of the last 25 years through Medline, Cancerlit, Science Citation Index, and Embase. RESULTS: The questionnaire was completed in 59 of 74 NT diagnosed during the period studied. All patients were Caucasians born in Spain. Constitutional-genetic factors were identified in 13.6 %: dominant hereditary syndromes in 5 (familial retinoblastoma in 1 and tuberous sclerosis in 4), and non-hereditary chromosomal syndromes in 3 (trisomy 21). Notable environmental factors were: a) ionizing radiation: preconceptional exposure in 32.2 % and in utero exposure in 6.8 %; b) non-ionizing radiation; 54.2 % reported preconceptional exposure and in utero exposure at home; c) pregnancy-related problems: infections in 13.6 % (8 patients) and a prior history of miscarriage in 20.3 %; d) drugs/chemicals administered during pregnancy: capillary dye in 42.4 %, daily facial make-up in 45.8 %, antacids in 30.5 %, acetaminophen in 34 %, hormone therapy in 10 %, cocaine and marijuana consumption in 11.7 %; e) tobacco: exposure to active smoking in 93.2 % of patients; f) alcohol: in utero exposure in 6.8 %; g) occupational exposure in parents: paternal in 49 % of cases (hydrocarbons in 15, metal in 3, chemical in 2, timber in 2, and agriculture in 7). Four were exposed to pesticides, 3 to solvents, and 3 to paints; and maternal in 12 cases (20 %): low frequency non-ionizing radiation in 6 (textile in 5, and railway in 1), 5 in the service sector (2 hairdressers, 3 cleaners), and 1 the in chemical industry. In another 6 there was exposure to solvents and paints (footwear industry). CONCLUSIONS: The hypotheses formulated in this observational study, which should be analyzed in future case-control and cohort studies, are: a) to scientifically document genetic-constitutional RF in the etiopathogenesis of NT; b) to determine the frequency and etiology of prior miscarriage and its possible association with NT; c) to determine the importance of active smoking before and during pregnancy as a RF for NT; d) to establish the importance of parental occupational exposure in the etiopathogenesis of NT; and e) to define more precisely the role of other physical and chemical RF in the development of NT. To determine and record the RF involved in the etiopathogenesis of NT, the environmental history should be documented in pediatric cancers. The identification of RF is one of the main objectives of Pediatric Environmental Health Specialty Units.

Factores de riesgo asociados a los tumores neonatales. Experiencia de una unidad de salud medioambiental pediátrica (PEHSU-Valencia) / Risk factors associated with neonatal tumors. Experience of a pediatric environmental health specialty unit in Valencia (Spain)

Antecedentes: Los tumores neonatales son el resultado final de la combinación variable del determinante constitucional y del medioambiental. Cada uno está constituido por multitud de factores de riesgo , mayoritariamente desconocidos. Objetivo: Documentar los factores de riesgo constitucionales y medioambientales preconcepcionales y transplacentarios asociados con mayor o menor evidencia científica al desarrollo de los tumores neonatales. Pacientes y métodos: Descripción de los factores de riesgo presentes en los tumores neonatales diagnosticados en el Hospital Infantil Universitario La Fe de Valencia, desde enero de 1990 a diciembre de 1999, obtenidos mediante un cuestionario a través de una entrevista personal a los padres de los pacientes, en la Unidad de Salud Medioambiental Pediátrica (PEHSU-Valencia) o en el domicilio de los pacientes. Revisión bibliográfica de los últimos 25 años del Medline, Cancerlit, Index Citation Science y Embase de los factores de riesgo asociados a los tumores neonatales y cánceres infantiles en general. Resultados: En 59 de los 74 tumores neonatales registrados se realizó el cuestionario. Todos eran de raza blanca y nacionalidad española. Los factores genéticos-constitucionales se asociaron en el 13,6 %: cinco síndromes hereditarios dominantes (un retinoblastoma familiar y cuatro esclerosis tuberosas) y tres síndromes cromosómicos no hereditarios (trisomía 21). Los factores ambientales más destacables asociados fueron: a) radiación ionizante: 32,2 % exposición preconcepcional y 6,8 % transplacentaria; b) radiación no ionizante: 54,2 % relataban exposición preconcepcional y transplacentaria en el entorno ambiental domiciliario; c) problemas gestacionales: 13,6 % infecciones gestacionales (8 casos) y el 20,3 % (12 casos) presentaban el antecedente de aborto en una gestación anterior; d) fármacos/medicaciones/drogas durante la gestación: 42,4 % tintes capilares, 45,8 % maquillaje facial diario, 30,5 % antiácidos, 34 % paracetamol, 10 % tratamiento hormonal, 11,7 % consumo de marihuana y cocaína; e) tabaco: la exposición al tabaquismo activo estuvo asociada al 93,2 % de los pacientes; f) alcohol: en el 6,8 % exposición transplacentaria; g) exposición ocupacional de los padres: en el 49 % existía exposición ocupacional paterna de riesgo (15 hidrocarburos, tres metales, dos químicas, dos madereras y siete agricultores). En ellos destaca exposición a pesticidas en cuatro, solventes en tres y pinturas en tres. En 12 casos (20 %) existía exposición materna ocupacional de riesgo: seis a radiaciones no ionizantes de baja frecuencia (cinco industria textil y una ferroviaria), cinco en el sector de servicios (dos peluqueras y tres limpiadoras), y una en la industria química. En otras seis existía exposición a solventes y pinturas (sector del calzado). Conclusiones: Las hipótesis que formulamos en el presente trabajo observacional, y que deben analizarse en futuros estudios de casos-controles y cohortes, son las siguientes: a) documentar con mayor solidez científica la presencia de los factores de riesgo constitucionales-genéticos en la etiopatogenia de los tumores neonatales; b) constatar la frecuencia y la etiología del antecedente materno de aborto o pérdida fetal, y su hipotética relación con los tumores neonatales; c) importancia del tabaquismo activo preconcepcional y transplacentario como factores de riesgo en los tumores neonatales; d) averiguar la importancia de los factores de riesgo ocupacionales parentales en la etiopatogenia de los tumores neonatales, y e) detallar con más precisión la contribución del resto de factores de riesgo físicos y químicos en el desarrollo de los tumores neonatales. Así mismo, el conocimiento y obtención de los factores de riesgo implicados en la etiopatogenia de los tumores neonatales, plantean la necesidad de documentar la historia medioambiental en los cánceres pediátricos. Determinar los factores de riesgo constituye uno de los principales objetivos de las Unidades de Salud Medioambiental Pediátrica

Background: Neonatal tumors (NT) result from a variable combination of constitutional and environmental determinants. Multiple risk factors (RF) are involved in their development, although most are unknown. Objective: To document the constitutional, environmental, preconceptional and transplacental RF associated with the development of NT with greater or lesser scientific evidence. Patients and methods: We investigated known RF in NT diagnosed at La Fe University Children's Hospital from January 1990 to December 1999, using a questionnaire completed by parents in a personal interview, either at the Pediatric Environmental Health Specialty Unit (PEHSU-Valencia) or at their home. RF associated with NT and childhood cancer were identified through a literature review of the last 25 years through Medline, Cancerlit, Science Citation Index, and Embase. Results: The questionnaire was completed in 59 of 74 NT diagnosed during the period studied. All patients were Caucasians born in Spain. Constitutional-genetic factors were identified in 13.6 %: dominant hereditary syndromes in 5 (familial retinoblastoma in 1 and tuberous sclerosis in 4), and non-hereditary chromosomal syndromes in 3 (trisomy 21). Notable environmental factors were: a) ionizing radiation: preconceptional exposure in 32.2 % and in utero exposure in 6.8 %; b) non-ionizing radiation; 54.2 % reported preconceptional exposure and in utero exposure at home; c) pregnancy-related problems: infections in 13.6 % (8 patients) and a prior history of miscarriage in 20.3 %; d) drugs/chemicals administered during pregnancy: capillary dye in 42.4 %, daily facial make-up in 45.8 %, antacids in 30.5 %, acetaminophen in 34 %, hormone therapy in 10 %, cocaine and marijuana consumption in 11.7 %; e) tobacco: exposure to active smoking in 93.2 % of patients; f) alcohol: in utero exposure in 6.8 %; g) occupational exposure in parents: paternal in 49 % of cases (hydrocarbons in 15, metal in 3, chemical in 2, timber in 2, and agriculture in 7). Four were exposed to pesticides, 3 to solvents, and 3 to paints; and maternal in 12 cases (20 %): low frequency non-ionizing radiation in 6 (textile in 5, and railway in 1), 5 in the service sector (2 hairdressers, 3 cleaners), and 1 the in chemical industry. In another 6 there was exposure to solvents and paints (footwear industry). Conclusions: The hypotheses formulated in this observational study, which should be analyzed in future case-control and cohort studies, are: a) to scientifically document genetic-constitutional RF in the etiopathogenesis of NT; b) to determine the frequency and etiology of prior miscarriage and its possible association with NT; c) to determine the importance of active smoking before and during pregnancy as a RF for NT; d) to establish the importance of parental occupational exposure in the etiopathogenesis of NT; and e) to define more precisely the role of other physical and chemical RF in the development of NT. To determine and record the RF involved in the etiopathogenesis of NT, the environmental history should be documented in pediatric cancers. The identification of RF is one of the main objectives of Pediatric Environmental Health Specialty Units

Trasplante de progenitores hematopoyéticos de sangre de cordón umbilical en niños / Transplantation of umbilical cord blood hematopoietic progenitor cells in children

OBJETIVO: Estudio retrospectivo de los resultados del trasplante de progenitores hematopoyéticos de sangre de cordón umbilical en España. MÉTODOS: Veintiocho niños con edad media de 6,5 años y peso medio de 25 kg recibieron un trasplante de sangre de cordón umbilical entre julio de 1994 y mayo de 1998 en distintos centros pertenecientes al Grupo Español para el Trasplante de Medula ósea en niños (GETMON). El donante fue en 2 pacientes un hermano HLA idéntico, en otros 2 pacientes un familiar no idéntico y en los 24 restantes un donante no emparentado. Entre éstos, la identidad antigénica HLA (A, B y DR) 6/6 sólo se observaba en 3 pacientes. Los trasplantes se realizaron en su mayoría por leucemia (21pacientes, 75) y en fase avanzada. Los restantes 7 pacientes se trasplantaron por una enfermedad genética, en su mayoría inmunodeficiencia congénita. El tratamiento de acondicionamiento incluyó irradiación corporal total en 10 pacientes y poliquimioterapia en los restantes. La profilaxis de la enfermedad del injerto contra huésped aguda se realizó con ciclosporina en todos los casos añadiendo corticoides o metotrexato en los trasplantes sin identidad HLA. La media de células perfundidas fue de 53,4x106/kg. RESULTADOS: El fallo de implante de la sangre de cordón umbilical se observó en 9 pacientes. Presentaron enfermedad del injerto contra huésped aguda superior al grado II 18 pacientes (64,3 por ciento). Ocho (28,6 por ciento) presentaron EICH grave. La supervivencia actuarial libre de enfermedad (SLE) de la serie global fue del 34,4>=9 por ciento a 3años, con una media de seguimiento de 16,6 meses. Se observó una mejor SLE en las enfermedades congénitas, con una SLE del 71>=17 por ciento y también en los pacientes que recibieron trasplante de sangre de cordón umbilical con una identidad HLA A, B y DR 6/6, en los que la SLE fue del 66>=19 por ciento. CONCLUSIÓN: Los mejores resultados se obtuvieron en las enfermedades genéticas. Se ha observado una correlación inversa entre la SLE y la disparidad antigénica HLA. La incidencia relativamente alta de la enfermedad injerto contra huésped aguda en esta serie, podría relacionarse con la escasa precisión de la tipificación HLA efectuada en algunos pacientes (AU)

[Transplantation of umbilical cord blood hematopoietic progenitor cells in children]. / Trasplante de progenitores hematopoyéticos de sangre de cordón umbilical en niños.

AIM: Retrospective study of the outcome of cord blood transplantation (CBT) in children in Spain. METHODS: Twenty-eight patients (mean age 6.5 years; mean weight 25 kg) received a CBT between July 1994 and May 1998 in several centres of the Spanish Pediatric Bone Marrow Transplant Group. In 2 patients the donor was an identical human leukocyte antigen (HLA)-sibling and in two the donor was a mismatched family donor. In 24 patients the donor was unrelated, and 21 of these received an HLA-mismatched CBT. Twenty-one patients (75 %) received a CBT for leukemia mainly in advanced phase. Seven patients were transplanted for genetic disease. Of these, five had congenital immunodeficiency. The conditioning treatment included total body irradiation in ten patients and combined chemotherapy in the remaining patients. In all patients graft-versus-host disease (GVHD) prophylaxis was performed with cyclosporine, and corticosteroids or methotrexate were added in patients with HLA-mismatched donors. The mean number of nucleated cells infused was 53.4 x 106/kg. RESULTS: Graft failure was observed in nine patients. Eighteen patients (64.3%) developed grade IIIV acute GVHD. Eight patients (28.6%) developed severe GVHD. Actuarial event free survival (EFS) of all the patients was 34.4 +/- 9% at 3 years, with a mean followup of 16.6 months. EFS was more favorable in patients with genetic disease (71>=6 17%) and in those with an HLA (A, B and DR) identical donor (6/6) (66>=6 19%). CONCLUSIONS: The most favorable results were obtained in patients with genetic diseases. We observed an inverse correlation between EFS and patients with HLA identical donors. The high incidence of severe acute GVHD could have been related to a lack of accuracy in the HLA typography of some patients.

[A comparative study of the weight-height development of the long-term survivors of acute leukemia and solid tumors]. / Estudio comparativo del desarrollo pondoestatural de los supervivientes a largo plazo de leucemia aguda y tumores sólidos.

OBJECTIVE: Our objectives were to analyze the final height and nutritional status in survivors of childhood cancer, their evolution since diagnosis and to identify neoplasm- and/or therapy-related differences. PATIENTS AND METHODS: A survey of long-term survivors of childhood cancer (acute leukemia, Wilms' tumor, sympathetic nervous system tumors) diagnosed before 15 years of age and between 1971 and 1985 in a single tertiary care center was performed. Final height, target height and body mass index were measured at evaluation. Height and body mass index, at diagnosis and at the end of treatment, was retrieved from their clinical records. All parameters are expressed as standard deviation scores of the mean of the population reference. Survivors were grouped according to diagnosis and type of treatment. Comparisons between groups and within each group were made at the time of diagnosis, at the end of treatment and at the time of evaluation. RESULTS: Sixty-one survivors of acute leukemia and 62 of solid tumors were included (32 Wilms' tumors, 20 neuroblastomas, 4 ganglioneuroblastomas and 6 ganglioneuromas). Eighty survivors had attained final height at the time of evaluation and their target height was available. Fourteen had at least one relapse. The mean height standard deviation score was positive at diagnosis and negative at the time of evaluation in all groups. Mean height loss ranged from -0.84 for the non-irradiated acute leukemia group to -1.34 for the non-irradiated solid tumor group. Adjusted final height for target height showed stature loss only in irradiated groups. Height loss was equivalent in cranially irradiated survivors (-0.32 after 18 Gy, -0.34 after 24-25.5 Gy). The age at menarche correlated negatively with the dose of cranial radiotherapy (r = -0.6, p = 0.002) and positively with stature loss (r = 0.5, p = 0.006). The mean body mass index standard deviation score was negative at diagnosis and positive at the time of evaluation in all groups. Twenty percent of solid tumor survivors and 12.5% of acute leukemia survivors were malnourished at diagnosis. Nutritional status improved in all groups at the time of evaluation. Obesity was more frequent in those who received cranial radiotherapy (14%) or intensified therapy (21%) compared with those non-cranially irradiated (none) or whose therapy was less intense (9%). CONCLUSIONS: Most survivors of childhood cancer attained their target height. Stature loss was related to cranial radiotherapy in acute leukemia survivors and to spinal irradiation in solid tumor survivors. At diagnosis, malnourishment was more frequent in solid tumor patients, while at the time of evaluation obesity was associated with a more intensified therapy.

[Down's syndrome and leukemia]. / Síndrome de Down y leucemia.

OBJECTIVE: Children with Down's Syndrome (DS) have a high risk for leukemia and need special clinical management. For this reason we have reviewed our experience. PATIENTS AND METHODS: All children with DS diagnosed a having acute leukemia during a 21-year period were reviewed retrospectively. Treatment was administered according to current protocols in our unit at the time of diagnosis without any initial modification. RESULTS: There were 13 children with DS and acute leukemia [6 ALL, 4 AML and 3 transient leukemias (TL)]. No patient presented CNS leukemia at diagnosis. All children with AML and DS were under three years of age and standard treatments did not achieve satisfactory results. TL regressed in two newborns without developing AMKL later. Five out of six patients with DS and ALL achieved complete remission. Currently, 4 of these children are alive and off therapy. Toxicities related to treatment were observed in almost all of the patients. CONCLUSIONS: Children with DS suffer a higher risk of developing leukemia. They should receive standard protocols, but aggressive supportive care might be provided as they have a higher incidence of treatment related toxicities. Prognosis of these children is similar or even better in some cases than children without DS. TL is a true neoplastic process capable of spontaneous remission and it can progress to AMKL.

[Granulocyte recovery and early infection complications in oncologic pediatric patients treated with hematopoietic stem cell transplantation]. / Prendimiento de la serie blanca y complicaciones infecciosas precoces en pacientes oncológicos pediátricos sometidos a transplante de precursores hematopoyéticos.

OBJECTIVE: Infections during the early post-BMT period are a major cause of morbidity in BMT recipients. We report our experience in a single institution. PATIENTS AND METHODS: Clinical reports from all BMT patients between 1989-1995 were reviewed retrospectively. Routine reverse isolation techniques, prophylactic measures and central venous catheter were used in all cases and starting in 1992, G-CSF was also used. Fever in the aplasia period was considered a sign of infection. RESULTS: Sixty-three BMTs were performed between 1989 and 1995 with 30 being ABMT, 17 allogenic and 16 PBSC. The time for granulocyte recovery was shorter in PBSC + G-CSF patients (d + 11). There were 65 infections in the early post-BMT period: 22 were microbiologically confirmed, 18 of them being of bacterial origin and 14 disseminated. Gram positive bacteria were predominant. There were 6 cases of interstitial pneumonitis, with 3 of them being lethal. Infection-related mortality was 6.3%. CONCLUSIONS: 1) BMTs can be safely performed using conventional reverse isolation. 2) The use of PBSC and G-CSF accelerates granulocyte recovery in BMTs. 3) Infections due to Gram positive bacteria were a major cause of morbidity in our series. 4) Pneumonitis and fungal infections are the most frequent lethal infections in our patients.

Renal adenocarcinoma in an 8-year-old child, with a t(X;17)(p11.2;q25).

This report describes a chromosomal abnormality in an 8-year-old boy with renal adenocarcinoma. All tumor cells had the karyotype 46,XY, t(X;17)(p11.2;q25). This karyotype is compared with the cytogenetic descriptions of renal cell adenocarcinoma in two other pediatric patients and in adults.

Scale for assessing quality of life of children survivors of cranial posterior fossa tumors.

BACKGROUND: Evaluation of quality of life of survivors of brain tumors is an important aspect of outcome that must be included in clinical studies. METHODS: We have developed a new scale for assessing quality of life (QL) of pediatric long-term survivors of posterior fossa tumors based on their physical, psychointellectual, and endocrine/growth status. We have studied 39 patients, with a median follow-up of 9 years. Twenty-five had cerebellar astrocytoma (CA), 6 medulloblastoma (MDB), 5 brain-stem glioma (BSG) and 3 ependymoma of IV ventricle (EPD). RESULTS: Sixty-six percent of children showed neurologic and/or visual sequelae. Little or no significant disability (Bloom's levels I-II) were present in 66%. Psychointellectual dysfunction was present in 44%, with an IQ < 90 in 39%. Endocrine and growth disorders were found in 26%, mostly stature anomalies. According to our scale, QL scores were high in 19 patients (49%), intermediate in 8 (20%), and low in the remaining 12 (31%). Unfavourable outcomes were related to age of less than 4 years, tumors other than CA (MDB, BSG, EPD), incomplete tumoral resection, and employment of radiotherapy and chemotherapy. CONCLUSION: Our results are comparable to others previously reported, and this supports the validity of our scale. We consider that this scale is applicable to evaluate QL of children survivors of cranial tumors.

[Non-Hodgkin's lymphoma in childhood: 19-year experience in a pediatric oncology unit]. / Linfomas no Hodgkin en la infancia: 19 años de experiencia en una unidad de oncología pediátrica.

Ninety-one patients with Non-Hodgkin's Lymphoma (NHL) were treated in our Pediatric Oncology Unit during a 19 year period. The median age at diagnosis was 5.8 years and there was a higher incidence in males. All patients were classified according to Murphy's stages and Rappaport's modified classification. Advanced disease and non-lymphoblastic histology were prevailing. Chemotherapy was the preferred treatment. Forty-seven patients (54%) are alive with a median follow-up period of 6.2 years. Actuarial survival rate at 5 years is 0.6. Advances in chemotherapy led to an increase in NHL patient's survival. Twenty patients died because of the disease and 21 because of fatal complications.

[Retinoblastoma: multidisciplinary treatment in a group of 32 patients]. / Retinoblastoma: tratamiento multidisciplinario en una serie de 32 pacientes.

Thirty-two children with retinoblastoma (RTB) were treated in our oncology unit between September 1981 and October 1989. There were 18 unilateral RTB and 14 bilateral. The median age at diagnosis was 24 months. Leukocoria was the most common sign of presentation (66%). All patients were treated with a protocol based on Reese-Ellsworth classification and the anatomo-clinic extension. Thirty patients were enucleated (7 bilaterally), 14 of which needed coadjuvant chemotherapy and 6 orbital radiotherapy. Two patients died, one from each group. The disease-free survival rate is 90.6% with a medium follow-up of 43.5 months. Seven children went blind because of bilateral enucleation and seven present facial deformity caused by radiotherapy. We underline the importance of improving the effectiveness of conservative treatment in order to reduce the number of enucleation.

[Acute lymphoblastic leukemia: clinical data and results of intensive chemotherapy]. / Leucemia no linfoblástica aguda: datos clínicos y resultados de una quimioterapia intensiva.

Twenty-one children, between eight months and twelve years old, diagnosed as having acute nonlymphoblastic leukemia (ANLL), were treated with an intensive chemotherapy based on a modified VAPA protocol. Complete remission rate was 80%, and the 5 year-survival rate is 46%. One chloroma, one central nervous system, and four bone marrow relapses were observed; all except the first having an unfavourable outcome. Significant risk prognostic factors in this series proved to be hepatomegaly (greater than 5 cm) and delay in obtaining remission. No fatal complications were observed.

[Systemic candidiasis in children with cancer]. / Candidiasis sistémicas en niños con cáncer.

Systemic fungal infections have long been recognized in terminally ill patients with cancer. Systemic candidiasis is the most common, the incidence having increased in the last few years. Eight children diagnosed as having systemic candidiasis during a two-year period (1987-1988) are presented. Three had an isolated fungal pneumonitis, two an hepatosplenic candidiasis, one a multisystemic involvement (hepatosplenic, pulmonary and nodular skin lesions) and the remaining two patients had a Candida sepsis with no visceral lesions having been documented. All patients had neutropenia and prolonged fever no responsive to broad spectrum antibiotics. We would like to underline the importance of an early and prolonged antifungal therapy, especially in hepatosplenic candidiasis, in order to obtain the cure.

[Acute lymphoblastic leukemia of standard risk: preliminary results obtained with protocol LL-IV-81]. / Leucemia linfoblástica aguda de riesgo estándar: resultados preliminares del protocolo LL-IV-81.

Forty-five patients with acute lymphoblastic leukemia of standard risk who were treated with the same protocol of therapy are presented. Induction therapy consisted of Vincristine (5 doses), L-asparaginase (10 doses) and prednisone. Complete remission was attained in 43 cases (95%). Two different methods of CNS prophylaxis were used; radiotherapy of the skull (18 Gy) associated with intrathecal methotrexate and intermediate dose intravenous methotrexate (1 g/M, 12 dose) with simultaneously intrathecal methotrexate. With a mean follow-up time of 28 months in group A and 35 Mo in group B, 82% and 75% respectively remain in continuous complete remission (p: NS). One case of meningeal leukemia was detected in each group. There patients in group A (13.6%) and four in group B (20%) suffered a bone marrow relapse. Actuarial survival rate at 36 months were 76 among the patients in group A and 83% in group B. Our results indicate that the two methods of CNS prophylaxis are both effective and in this series we have not observed any decrease in the bone marrow relapse rate when intermediate dose methotrexate was used.