RESUMEN
OBJECTIVES: To estimate the rate of progression of plexiform neurofibroma after surgery and to identify prognostic factors that predict progression. STUDY DESIGN: A retrospective review of the inpatient and outpatient records of 121 patients, who had 302 procedures on 168 tumors over a 20-year period at a single large pediatric referral center. Data on age, location, indication for surgery, and extent of resection was analyzed for prognostic significance. RESULTS: The overall freedom from progression was 54%. Children < 10 years old had a shorter interval of tumor control than older children (p = 0.0004). Tumors of the head/neck/face fared worse than tumors of the extremities (p = 0.0003). Less extensive resection predicted shorter interval to progression (p < 0.0001). Indication for surgery was not of prognostic importance. In multivariable analysis older age and location in the extremities were predictors of a better outcome. CONCLUSIONS: Tumor progression is a serious problem for children with plexiform neurofibroma. Younger children, children with tumors of the head/neck/face, and tumors that cannot be nearly completely removed are at particular risk. These data may be useful in helping clinicians decide which patients and which tumors are most likely to benefit from surgical intervention.
Asunto(s)
Neoplasias de Cabeza y Cuello/cirugía , Neoplasias/cirugía , Neurofibroma Plexiforme/cirugía , Neurofibromatosis 1/cirugía , Neoplasias del Sistema Nervioso Periférico/cirugía , Neoplasias de la Columna Vertebral/cirugía , Adolescente , Adulto , Niño , Preescolar , Femenino , Neoplasias de Cabeza y Cuello/epidemiología , Hospitales Pediátricos , Humanos , Lactante , Recién Nacido , Estudios Longitudinales , Masculino , Neoplasias/epidemiología , Neurofibroma Plexiforme/epidemiología , Neurofibromatosis 1/epidemiología , Pennsylvania , Neoplasias del Sistema Nervioso Periférico/epidemiología , Complicaciones Posoperatorias/epidemiología , Pronóstico , Estudios Retrospectivos , Neoplasias de la Columna Vertebral/epidemiología , Factores de TiempoRESUMEN
Twenty-four children, aged 1.5-20 yr at diagnosis, with noncortical brain tumors, primarily medulloblastoma, have been followed for 3-4 yr for intellectual status. All the children received craniospinal irradiation, and 19 of 24 received chemotherapy as well. For the group as a whole. Full Scale IQ fell from 104 at baseline to 91 at final follow-up. Children younger than 7 yr at diagnosis showed a significant decrease in IQ as early as year 1, and all changes from baseline to years 3 and 4 were significant. In contrast, children older than 7 yr at diagnosis did not show a significant IQ change from baseline to year 3 or 4. The Spearman correlation coefficient between IQ change and age at diagnosis from baseline to year 4 was 0.57 (P = 0.003). This study supports the hypothesis that children treated with whole brain radiation at a younger age have more severe cognitive impairment than those treated at a later age. Limitations in sample size and duration of observations do not permit us to identify whether a true plateau occurs 2-4 yr after irradiation versus a continued progressive decline in intellectual performance. Moreover, we cannot at this time distinguish between a true dementing process versus failure to acquire new cognitive skills at a rate comparable to age-matched peers.
Asunto(s)
Neoplasias Encefálicas/radioterapia , Trastornos del Conocimiento/etiología , Irradiación Craneana/efectos adversos , Meduloblastoma/radioterapia , Traumatismos por Radiación/psicología , Adolescente , Adulto , Factores de Edad , Edad de Inicio , Neoplasias Encefálicas/psicología , Neoplasias Cerebelosas/psicología , Neoplasias Cerebelosas/radioterapia , Niño , Preescolar , Trastornos del Conocimiento/psicología , Humanos , Lactante , Pruebas de Inteligencia , Meduloblastoma/psicología , Pruebas NeuropsicológicasAsunto(s)
Neoplasias Encefálicas/patología , Antígenos de Neoplasias , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/terapia , Preescolar , Terapia Combinada , Medios de Contraste , Femenino , Gadolinio DTPA , Humanos , Técnicas para Inmunoenzimas , Cariotipificación , Imagen por Resonancia Magnética , Glicoproteínas de Membrana/análisis , Mucina-1 , Compuestos Organometálicos , Ácido Pentético , Tomografía Computarizada por Rayos X , Vimentina/análisisRESUMEN
The authors found that the course and treatment of narcotic withdrawal in two neonates was complicated by prenatal exposure to high doses of diazepam, or Valium (Roche Laboratories, Nutley, NJ). Both of the mothers were on methadone maintenance for narcotic dependency prior to the diagnosis of pregnancy. The authors documented maternal intake of diazepam in the range of 40-60 mg/day for a duration of 4-27 weeks prior to delivery. Both infants initially responded well to medical therapy for narcotic withdrawal, but at 7-14 days of age, withdrawal symptoms intensified, requiring an increase in the dosages of Paregoric (UDL Laboratories, Rockford, IL) and opium tincture in both infants and the addition of phenobarbital therapy in one infant. Both infants continued on medical therapy until they reached 1 month of age. Diazepam use by pregnant women can be associated with a later presentation of withdrawal symptoms in the neonate than that induced by the use of other drugs. Close follow-up during the first month of life is warranted for infants exposed to diazepam prenatally.
Asunto(s)
Diazepam , Enfermedades del Recién Nacido/inducido químicamente , Metadona/administración & dosificación , Complicaciones del Embarazo/inducido químicamente , Síndrome de Abstinencia a Sustancias/etiología , Trastornos Relacionados con Sustancias/complicaciones , Adulto , Diazepam/efectos adversos , Quimioterapia Combinada , Femenino , Humanos , Recién Nacido , Enfermedades del Recién Nacido/tratamiento farmacológico , Masculino , Metadona/efectos adversos , Opio/uso terapéutico , Parasimpatolíticos/uso terapéutico , Fenobarbital/uso terapéutico , Embarazo , Síndrome de Abstinencia a Sustancias/tratamiento farmacológico , Trastornos Relacionados con Sustancias/rehabilitación , Factores de TiempoRESUMEN
Between 1975 and 1989, 45 children with newly diagnosed intracranial ependymomas were treated at the authors' institution. Patients were managed with aggressive surgical resection, followed by postoperative CT or MRI scans to evaluate the extent of resection. Most patients received involved-field radiation therapy, however 10 were treated with craniospinal axis irradiation for disseminated disease, or malignant histology. Beginning in 1983, all patients were also treated with adjuvant chemotherapy with 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), vincristine, and cisplatinum. Four patients died in the immediate postoperative period for an operative mortality of 8.8%. The 5-year progression-free survival for the remaining 41 patients was 36%. Tumor location had little effect on survival, as the 5-year PFS for supratentorial tumors was 26%, compared with 40% for the posterior fossa lesions (ns). Neither histologic degree of malignancy, nor use of adjuvant chemotherapy impacted on survival. The extent of surgical resection, as determined by operative reports and postoperative imaging studies, was a major determinant of outcome, as patients with total or near-total resections experienced a 5-year PFS of 60%, compared with 21% for children with partial resection or biopsy (p less than 0.01). It is concluded that the use of adjuvant chemotherapy with CCNU, vincristine, and cisplatinum does not improve progression-free survival in childhood ependymoma, and that the extent of surgical resection is a major determinant of outcome in this disease.
Asunto(s)
Neoplasias Encefálicas/cirugía , Ependimoma/cirugía , Complicaciones Posoperatorias/mortalidad , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Encefálicas/tratamiento farmacológico , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/radioterapia , Quimioterapia Adyuvante , Niño , Preescolar , Cisplatino/administración & dosificación , Terapia Combinada , Ependimoma/tratamiento farmacológico , Ependimoma/mortalidad , Ependimoma/radioterapia , Femenino , Humanos , Lactante , Lomustina/administración & dosificación , Imagen por Resonancia Magnética , Masculino , Pronóstico , Tasa de Supervivencia , Tomografía Computarizada por Rayos X , Vincristina/administración & dosificaciónRESUMEN
The association between neurofibromatosis and visual pathway gliomas is well documented. The introduction of computed tomography and magnetic resonance imaging has heralded a new era in the understanding of visual pathway gliomas. Both of these noninvasive neuroinvestigative techniques have demonstrated extensive abnormalities throughout the visual pathway in children with visual pathway gliomas, especially in those with neurofibromatosis. The clinical significance of these abnormal areas of brain, especially in asymptomatic patients, is unknown. In an attempt to clarify the incidence, natural history, and clinical course of patients with neurofibromatosis and visual pathway lesions, we reviewed our experience with 24 patients managed consecutively at Children's Hospital of Philadelphia over the past 12 years. The patients in this series were compared to 29 children with visual pathway gliomas without neurofibromatosis who were evaluated at our institution over the same period of time. Visual pathway gliomas in children with neurofibromatosis differ from those in children without neurofibromatosis. In general, lesions tended to be more extensive in patients with neurofibromatosis and the clinical course of these patients is more variable. Twelve of the 24 patients with neurofibromatosis in our series had symptoms of progressive disease at the time of diagnosis and underwent treatment with variable results. Twelve children with neurofibromatosis and visual pathway lesions had static lesions at the time of diagnosis and, to date, 3 have developed progressive disease. From our review we can make some recommendations concerning the management of children with neurofibromatosis and visual pathway gliomas, but many questions remain unanswered. Sequential follow-up of a large cohort of both asymptomatic and symptomatic children with neurofibromatosis and visual pathway lesions is needed to more definitively outline the best management approach for these patients.
Asunto(s)
Neoplasias de los Nervios Craneales/complicaciones , Glioma/complicaciones , Neurofibromatosis 1/complicaciones , Quiasma Óptico/patología , Enfermedades del Nervio Óptico/complicaciones , Adolescente , Niño , Preescolar , Neoplasias de los Nervios Craneales/diagnóstico , Neoplasias de los Nervios Craneales/radioterapia , Femenino , Glioma/diagnóstico , Glioma/radioterapia , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Enfermedades del Nervio Óptico/diagnóstico , Enfermedades del Nervio Óptico/radioterapia , Vías Visuales/patologíaRESUMEN
Vocal cord paralysis with inspiratory stridor during infancy is a well-recognized complication of the Arnold-Chiari malformation associated with myelomeningocele. Management of these symptoms, however, remains controversial. The outcome in 19 infants with symptoms managed by us during 1978-1984 was therefore reviewed. Ten infants had stridor alone at presentation (clinical grade 1), four infants also had apnea (grade 2), and five infants also had associated cyanotic spells and dysphagia (grade 3). Of 14 ventricular shunt revisions performed, seven were associated with resolution: in five of eight infants with grade 1, two of four with grade 2, and none of two with grade 3 symptoms. Of the 10 posterior fossa decompressions performed, two were associated with clinical resolution: in one of four infants with grade 1, one of two with grade 2, and none of four with grade 3 symptoms. Mortality over 6 months following onset of symptoms was absent among infants with grade 1, one with grade 2, and three with grade 3 symptoms. We propose that differences in outcome among the three groups of infants reflect differences in pathologic processes within the brain stem. We speculate that infants with grade 2 or 3 symptoms have more extensive brain stem damage, such as hemorrhage, infarction, and necrosis. Use of our classification system may be helpful in further studies on intervention modalities in these infants.
Asunto(s)
Malformación de Arnold-Chiari/complicaciones , Derivaciones del Líquido Cefalorraquídeo , Meningomielocele/complicaciones , Ruidos Respiratorios/etiología , Parálisis de los Pliegues Vocales/terapia , Fosa Craneal Posterior/cirugía , Humanos , Lactante , Recién Nacido , Reoperación , Estudios Retrospectivos , Traqueotomía , Parálisis de los Pliegues Vocales/etiologíaRESUMEN
Astrocytomas are the most frequently encountered brain neoplasms in the pediatric age group. Current management strategies consist of surgery, radiation therapy, chemotherapy and immunotherapy, although the optimal treatment is yet to be defined. It is hoped that new treatment modalities, and reassessment of current regimens will improve outcome, better in terms of survival and quality of life.