RESUMO
PURPOSE: Alagille syndrome (ALGS) is a multisystem autosomal dominant disorder with highly variable expression. Intracranial arterial and venous anomalies have a reported prevalence of 30-40% and can increase the risk of stroke by 16%. Few reports document the frequency and evolution of cerebrovascular abnormalities (CVAs) in children with ALGS. We aimed to define the spectrum, frequency, and evolution of CVAs in a series of children with ALGS using magnetic resonance angiography (MRA). METHODS: We conducted a single-center, retrospective study in a large tertiary pediatric hospital. CVAs were grouped into 4 categories: 1) Stenosis or narrowing; 2) Aneurysms and ectasias; 3) Tortuosity; and 4) Vascular anomalies and anatomical variants. RESULTS: Thirty-two children met the inclusion criteria. The median age at initial diagnosis was 6 (3.8-10.3) years. Thirteen (40%) had follow-up MRI at a mean of 55 (31.5-66) months. Eighteen (56%) had CVAs; the most frequent fell into group 1 (n = 12, 37.5%). CVAs were stable over time, except for one patient with Moyamoya arteriopathy (MMA). One patient developed a transient ischemic attack secondary to an embolic event. Three (9.3%) had microhemorrhages at the initial diagnosis secondary to Tetralogy of Fallot. Another patient had recurrent subdural hematomas of unknown cause. CONCLUSION: CVAs were stable except in the presence of MMA. Vascular strokes, which are reported in older patients with ALGS, were not a common feature in children under 16 years of age, either at presentation or over the 31.5-66 month follow-up period.
Assuntos
Síndrome de Alagille , Angiografia por Ressonância Magnética , Humanos , Síndrome de Alagille/diagnóstico por imagem , Síndrome de Alagille/complicações , Masculino , Feminino , Criança , Pré-Escolar , Estudos Retrospectivos , Angiografia por Ressonância Magnética/métodos , Transtornos Cerebrovasculares/diagnóstico por imagemRESUMO
PURPOSE: Pediatric spinal cord gliomas (PSGs) are rare in children and few reports detail their imaging features. We tested the association of tumoral grade with imaging features and proposed a novel approach to categorize post-contrast enhancement patterns in PSGs. METHODS: This single-center, retrospective study included patients <21 years of age with preoperative spinal MRI and confirmed pathological diagnosis of PSG from 2000-2022. Tumors were classified using the 5th edition of the WHO CNS Tumors Classification. Two radiologists reviewed multiple imaging features, and classified enhancement patterns using a novel approach. Fisher's exact test determined associations between imaging and histological features. RESULTS: Forty-one PSGs were reviewed. Thirty-four were intramedullary, and seven were extramedullary. Pilocytic astrocytoma was the most common tumor (39.02%). Pain and weakness were the most prevalent symptoms. Seven patients (17.07%) died. Cyst, syringomyelia, and leptomeningeal enhancement were associated with tumor grade. Widening of the spinal canal was observed only in low-grade astrocytomas. There was a significant association between tumor grade and contrast enhancement pattern. Specifically, low-grade PSGs were more likely to exhibit type 1A enhancement (mass-like, with well-defined enhancing margins) and less likely to exhibit type 1B enhancement (mass-like, with ill-defined enhancing margins). CONCLUSION: PSGs display overlapping imaging features, making grade differentiation challenging based solely on imaging. The correlation between tumor grade and contrast enhancement patterns suggests a potential diagnostic avenue, requiring further validation with larger, multicenter studies. Furthermore, Low-grade PSGs display cysts and syringomyelia more frequently, and leptomeningeal enhancement is less common.
Assuntos
Glioma , Imageamento por Ressonância Magnética , Gradação de Tumores , Neoplasias da Medula Espinal , Humanos , Masculino , Feminino , Criança , Estudos Retrospectivos , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/patologia , Glioma/diagnóstico por imagem , Glioma/patologia , Adolescente , Imageamento por Ressonância Magnética/métodos , Pré-Escolar , Meios de Contraste , LactenteRESUMO
PURPOSE: The traditional imaging findings reported in Sturge-Weber syndrome (SWS) include endpoints of cortical injury-cortical atrophy and cortical calcifications-but also what has been termed a "leptomeningeal angiomatosis," the latter recognized and reported as a leptomeningeal enhancement on magnetic resonance imaging (MRI). The objective of this study is to demonstrate through neuropathological correlation that the "leptomeningeal angiomatosis" in patients with Sturge-Weber syndrome (SWS), represents a re-opened primitive venous network in the subarachnoid space that likely acts as an alternative venous drainage pathway, seen separately to abnormal pial enhancement. MATERIALS AND METHODS: Retrospective review of MR imaging and surgical pathology of patients that underwent surgery for epilepsy at a tertiary, children's hospital. A pediatric radiologist with more than 20 years of experience reviewed the MR imaging. Surgically resected brain specimens that had been sectioned and fixed in 10% paraformaldehyde for histologic processing, following processing and paraffin embedding, were cut into 5-µm unstained slides which were subsequently stained with hematoxylin and eosin (H&E). Slides were re-examined by a board-certified pediatric neuropathologist, and histologic features specifically relating to cerebral surface and vascularity were documented for correlation with MR imaging of the resected region performed prior to resection. RESULTS: Five patients were reviewed (3 boys and 2 girls; the median age at the onset of seizures was 12 months (IQR, 7 to 45 months); the median age at surgery was 33 months (IQR, 23.5 to 56.5 months)). Surgical procedures included the following: 4, hemispherotomy (right: 2, left: 2) and 1, hemispherectomy (right). A subarachnoid space varicose network was present on both MRI and histology in 4 patients. Calcifications were seen on both MRI and histology in 3 patients. Abnormal leptomeningeal enhancement was present in 5 patients and seen separately from the subarachnoid vascular network in 4 patients. CONCLUSION: Histopathology confirmed the MRI findings of a subarachnoid space varicose network seen separately from leptomeningeal enhancement and presumed to represent an alternative venous drainage pathway to compensate for maldevelopment of cortical veins, the primary abnormality in SWS. No pial-based angioma was identified.
Assuntos
Imageamento por Ressonância Magnética , Síndrome de Sturge-Weber , Humanos , Síndrome de Sturge-Weber/diagnóstico por imagem , Síndrome de Sturge-Weber/cirurgia , Síndrome de Sturge-Weber/patologia , Masculino , Feminino , Estudos Retrospectivos , Pré-Escolar , Imageamento por Ressonância Magnética/métodos , Criança , Lactente , Pia-Máter/diagnóstico por imagem , Pia-Máter/patologia , Espaço Subaracnóideo/diagnóstico por imagem , Espaço Subaracnóideo/patologia , Espaço Subaracnóideo/cirurgia , Adolescente , Angiomatose/cirurgia , Angiomatose/diagnóstico por imagem , Angiomatose/patologia , Varizes/diagnóstico por imagem , Varizes/cirurgia , Varizes/patologiaRESUMO
Introduction: Burkitt's lymphoma is a non- Hodgkin B-cell lymphoma whose cranial location is extremely rare. Clinical case: The case of a 35-year-old Peruvian man with a progressive parietal left cranial tumor is described. A biopsy with immunohistochemistry was performed for the diagnostic confirmation of Burkitt's lymphoma. First-line outpatient treatment with CODOX-M/IVAC was initiated, however, he stopped attending his treatment for 2 months. He returns, he is shown progression of the disease and is treated with rituximab, ifosfamide carboplatin and etoposide. Does not respond satisfactorily and dies. Conclusions: Burkitt's lymphoma is a very aggressive tumor that, if not treated in a timely manner, has high mortality, as happened with the patient in the case.
Introducción: El linfoma de Burkitt es un linfoma no Hodgkin de células B cuya ubicación craneal es extremadamente rara. Caso clínico: Se describe el caso de un hombre peruano de 35 años con una tumoración craneal parietal izquierda de crecimiento progresivo. Para la confirmación diagnóstica de linfoma de Burkitt se realizó una biopsia con inmunohistoquímica. Se inició tratamiento ambulatorio de primera línea con CODOX-M/IVAC, sin embargo, deja de acudir a su tratamiento por 2 meses. Retorna, se le evidencia progresión de la enfermedad y se le trata con rituximab, ifosfamida carboplatino y etopósido. No responde de forma satisfactoria y fallece. Conclusiones: El Linfoma de Burkitt es un tumor muy agresivo que si no es tratado oportunamente presenta alta mortalidad, tal como ocurrió con el paciente del caso.
Assuntos
Linfoma de Burkitt/diagnóstico , Neoplasias Cranianas/diagnóstico , Adulto , Biópsia , Evolução Fatal , Humanos , Imageamento por Ressonância Magnética , MasculinoRESUMO
Introduction: Solid-pseudopapillary neoplasms (SPN), or Frantz tumor, is a rare, low-grade neoplasm that occurs mainly in young women. It was described in 1959 by Virginia Frantz and constitutes 0.2-2.7 % of all pancreatic tumors. Computed tomography (CT) plays an important role in the diagnosis of this pathology of scarce reporting. The objective of the present study is to describe the epidemiological and tomographic characteristics of the SPN in the Instituto Nacional de Enfermedades Neoplásicas (INEN) of Peru. Methods: Descriptive cross-sectional study performed with medical records of all patients diagnosed with SPN between 2004 and 2014. The variables described were tumor size, location, shape, borders, thickness of the capsule, composition, calcifications and uptake of contrast. The categorical variables were expressed in absolute and relative frequencies; while the numerical variables were described with median and interquartile deviation (ID). Statistical support STATA Version 12.0 was used. Results: Of all pancreatic cystic tumors (PCT), 51.9% corresponded to confirmed cases of TSP. The median age was 23.5 years. The isolated location in the head predominated (33.3%); the most frequent mixed location was body and tail (16.7%); diameter was more frequent between 5.1-10 cm and the content of the majority was predominantly solid. 30.0% of the NSP presented calcifications. Conclusion: Most cases of INP SPN (2004-2014) have similar characteristics to those reported in the international literature.
Introducción: El tumor sólido pseudopilar de páncreas (TSP), o tumor de Frantz, es una neoplasia rara de bajo grado que acontece principalmente en mujeres jóvenes. Fue descrita en 1959 por Virginia Frantz y constituye del 0.2-2.7% de todos los tumores pancreáticos. La tomografía computada (TC) desempeña un papel importante en el diagnóstico de esta patología de escaso reporte. El objetivo del presente estudio es describir las características epidemiológicas y tomográficas del TSP en el Instituto Nacional de Enfermedades Neoplásicas (INEN) del Perú. Métodos: Estudio transversal descriptivo realizado con historias clínicas (HC) de todos los pacientes con diagnóstico de TSP entre los años 2004 y 2014. Las variables descritas fueron tamaño del tumor, ubicación, forma, bordes, grosor de la cápsula, composición, calcificaciones y captación de contraste. Las variables categóricas fueron expresadas en frecuencias absolutas y relativas; mientras que las variables numéricas fueron descritas con mediana y desviación intercuartílica (DIC). Se utilizó como soporte estadístico STATA Versión 12.0. Resultados: De todos los tumores quísticos de páncreas (TQP), el 51.9 % correspondió a casos confirmados de TSP. La mediana de la edad fue 23.5 años. Predominó la ubicación aislada en cabeza (33.3 %); la ubicación mixta más frecuente fue la de cuerpo y cola (16.7 %); fue más frecuente el diámetro entre 5.1-10 cm y el contenido de la mayoría fue a predominio sólido. El 30.0 % de los TSP presentaron calcificaciones. Conclusión: La mayoría casos de TSP del INEN (2004-2014) tienen características similares a lo reportado en la literatura internacional.
Assuntos
Neoplasias Pancreáticas/diagnóstico por imagem , Estudos Transversais , Humanos , Neoplasias Pancreáticas/epidemiologia , Peru/epidemiologia , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
Objetivo: Describir las conductas sexuales en una muestra de adultos jóvenes limeños de nivel socioeconómico C y D. Diseño: Estudio descriptivo transversal. Lugar: EIGER. Participantes: 156 adultos jóvenes. Intervenciones: Se usó un cuestionario semi-estructurado auto-administrado y se realizó el análisis con el Software STATA 9.0. Principales medidas de resultados: Para comparar las variables cuantitativas de acuerdo a variables cualitativas dicotómicas se usó el test T de Student, previo examen de los supuestos de normalidad por el test de Shapiro Wilk y de homogeneidad de varianzas por el test de Levene, de no cumplirse los supuestos se usó el test de suma de Rangos de Wilxocon. Para evaluar la asociación entre dos variables cualitativas se usó el Test exacto de Fisher. Resultados: La edad promedio de inicio sexual fue de 17.23 ±3.51 años. La primera relación sexual se da con la pareja sentimental, el número promedio de parejas sexuales 1.88 ±1.01 en mujeres y 4.35 ±3.57 en hombres. Tener más de una pareja sexual durante la misma época, usar el Internet para buscar parejas sexuales, acudir a los servicios de prostitución y tener RS con parejas ocasionales conocidas o desconocidas son conductas mayoritariamente practicadas por hombres en nuestro estudio. Conclusiones: La población masculina se encuentra en mayor riesgo por presentar conductas y actitudes más riesgosas que la población femenina.
Objective: To describe sexual behavior patterns in a sample of Peruvian young adults of socioeconomic levels C and D. Study Design: Descriptive, cross- sectional study. Participants: 156 young adults Interventions: We used a self-completed questionnaire and analyzed it with the statistical software package STATA. Main outcome measures: The T test of Student was used to compare the quantitative variables according to the dichotomy qualitative variables, with a previous examination of the suppositions of normality by the test of Shapiro Wilk and the homogeneity of variances by the test of Levene. If the suppositions were not completed, we used the test of sum of Ranges of Wilxocon. To evaluate the association between two qualitative variables, the exact Test of Fisher was used. Results: Respondents have on average, started their sexual life by the age of 17.23 ±3.51 years. Mostly, the initial intercourse is with their partner ; the average number of sexual partners is 1.88 ±1.01 in women and 4.35 ±3.57 in men. To have more than one sexual partner at the same time, to use the Internet with a sexual purpose, to attend prostitution services and to have intercourse with occasional well-known or strangers are behavior patterns practiced mainly by men in our study. Conclusions: Male population is more threatened because of high-risk behavior and attitudes than female population.