All children with malignant rhabdoid tumors should be treated initially with intensive chemotherapy.

Malignant rhabdoid tumors (MRT) of soft tissues are aggressive tumors, which can be detected in almost any part of the body. MRT are rare, and very few cases have been reported in the literature. Prognosis of these tumors is extremely poor despite intensive therapy. Some risk factors such as young age or disseminated disease are associated with an aggressive and almost always lethal clinical course. Some clinicians even recommend initial palliative care due to this outcome. We report a case of metastatic MRT in a 6-month-old child with excellent initial response to chemotherapy.

Obesity-dependent metabolic signatures associated with nonalcoholic fatty liver disease progression.

Our understanding of the mechanisms by which nonalcoholic fatty liver disease (NAFLD) progresses from simple steatosis to steatohepatitis (NASH) is still very limited. Despite the growing number of studies linking the disease with altered serum metabolite levels, an obstacle to the development of metabolome-based NAFLD predictors has been the lack of large cohort data from biopsy-proven patients matched for key metabolic features such as obesity. We studied 467 biopsied individuals with normal liver histology (n=90) or diagnosed with NAFLD (steatosis, n=246; NASH, n=131), randomly divided into estimation (80% of all patients) and validation (20% of all patients) groups. Qualitative determinations of 540 serum metabolite variables were performed using ultraperformance liquid chromatography coupled to mass spectrometry (UPLC-MS). The metabolic profile was dependent on patient body-mass index (BMI), suggesting that the NAFLD pathogenesis mechanism may be quite different depending on an individual's level of obesity. A BMI-stratified multivariate model based on the NAFLD serum metabolic profile was used to separate patients with and without NASH. The area under the receiver operating characteristic curve was 0.87 in the estimation and 0.85 in the validation group. The cutoff (0.54) corresponding to maximum average diagnostic accuracy (0.82) predicted NASH with a sensitivity of 0.71 and a specificity of 0.92 (negative/positive predictive values=0.82/0.84). The present data, indicating that a BMI-dependent serum metabolic profile may be able to reliably distinguish NASH from steatosis patients, have significant implications for the development of NASH biomarkers and potential novel targets for therapeutic intervention.

[Low-grade gliomas: review of 10 years]. / Gliomas de bajo grado: revisión de 10 años.

INTRODUCTION: Central nervous system (CNS) tumors are the most common solid tumors in children. Among these, the low-grade gliomas are the most common type, accounting for up to 30-50% of them. PATIENTS AND METHODS: A retrospective analysis was carried out on the epidemiology, clinical characteristics, tumor location, histology, treatment, outcome and long-term sequelae of 111 patients diagnosed with low-grade glioma in the Niño Jesús Children's Hospital of Madrid from January 2002 to December 2011. RESULTS: Of the 111 patients, there were 57 boys and 54 girls. The mean age was 7.26 years (range, 2 months - 19 years). The most common symptoms of presentation were headache (27%) and vomiting (19%). The most common locations were the cerebral hemispheres (38%), followed by the brainstem (27.4%), and cerebellum (18.5%). Histological examination was performed in 89 patients (80.18%). Pilocytic astrocytoma was the most common histological type. Diagnostic biopsy was performed in 20 patients (22.5%), partial resection in 38 patients (42.7%), and total resection in 31 patients (34.8%). Sixteen patients received chemotherapy (14%), and eighteen patients received radiotherapy (16%). Overall survival was 88.3%. Long term hearing, visual and endocrine sequelae were note in 1, 5, and 4 patients, respectively. CONCLUSIONS: The most common histological type is pilocytic astrocytoma. Overall survival was 88.3%. Only 9% of patients had some kind or auditory, visual or endocrine sequelae.

[Hepatosplenic candidiasis in paediatric haematology-oncology patients]. / Candidiasis hepatoesplénica en pacientes hemato-oncológicos pediátricos.

INTRODUCTION: In the last few decades, the survival of children with haematology-oncological malignancies has increased due to more aggressive chemotherapy regimens. This has led to an increase of fungal infections causing significant morbidity and mortality in these patients. Hepatosplenic candidiasis is a disseminated candida infection that affects most commonly the liver and spleen, although other organs may be involved. PATIENTS AND METHODS: We performed a retrospective study of 13 paediatric patients diagnosed with hepatosplenic candidiasis from January 2002 to February 2010 in our paediatric haematology-oncology department following the criteria proposed by the EORTC/MSG (European Organization for Research and Treatment of Cancer and Mycoses study group) updated in 2008. We analysed the clinical characteristics, diagnostic methods, treatment and outcome. RESULTS: The most common symptom of presentation of hepatosplenic candidiasis in our series was persistent fever, up 84.6% of cases, almost all associated with other symptoms. Risk factors for development are non-specific and were present in 92.3% of our patients. The diagnosis of proven infection, which is made by histology or culture, is usually difficult to obtain and in our series it was obtained only in 23.1% of cases. As in our series, diagnosis is often presumptive. Laboratory tests are not useful in the paediatric population and only in one patient we observed an increase in alkaline phosphatase at diagnosis. This result differs from the behaviour of this biological marker in adults. Although prolonged treatment is often necessary for the resolution of the lesions, survival is high with appropriate treatment, 100% in our series. CONCLUSIONS: The mortality of hepatosplenic candidiasis is low with early and adequate treatment.

Gliomas de bajo grado: revisión de 10 años / Low-grade gliomas: Review of 10 years

INTRODUCCIÓN: Los tumores del SNC son los tumores sólidos más frecuentes en la edad pediátrica. Dentro de ellos los gliomas de bajo grado constituyen el tipo más común de tumor del SNC en niños, representando hasta el 30-50% de los mismos. PACIENTES Y MÉTODOS: Análisis retrospectivo de las características epidemiológicas, manifestaciones clínicas, localización del tumor, histología, tipo de tratamiento si lo ha recibido, evolución y secuelas a largo plazo de 111 pacientes diagnosticados de glioma de bajo grado en el Hospital Infantil Universitario Niño Jesús de Madrid entre enero de 2002 y diciembre de 2011. RESULTADOS: De los 111 pacientes 57 eran niños y 54 niñas. La edad media fue de 7,26 años (intervalo 2 meses-19 anos). Los síntomas de presentación más frecuentes fueron la cefalea (27%) y los vómitos (19%). Las localizaciones más frecuentes fueron los hemisferios cerebrales (38%), seguido del tronco cerebral (27,4%) y del cerebelo (18,5%). Se realizó estudio histológico en 89 pacientes (80,18%), siendo el astrocitoma pilocítico el tipo histológico más frecuente. Se realizó biopsia diagnóstica en 20 pacientes (22,5%), resección parcial en 38 pacientes (42,7%) y resección total en 31 pacientes (34,8%). Recibieron quimioterapia 16 pacientes (14%) y radioterapia 18 pacientes (16%). La supervivencia global fue del 88,3%. Un paciente presentó secuelas auditivas, 5 pacientes presentaron secuelas visuales y 4 pacientes secuelas endocrinas. CONCLUSIONES: El tipo histológico más frecuente es el astrocitoma pilocítico. La supervivencia global fue del 88,3%. Solo el 9% de los pacientes presentaron algún tipo de secuela auditiva, visual o endocrinológica

INTRODUCTION: Central nervous system (CNS) tumors are the most common solid tumors in children. Among these, the low-grade gliomas are the most common type, accounting for up to 30-50% of them. PATIENTS AND METHODS: A retrospective analysis was carried out on the epidemiology, clinical characteristics, tumor location, histology, treatment, outcome and long-term sequelae of 111 patients diagnosed with low-grade glioma in the Nino˜ Jesús Children's Hospital of Madrid from January 2002 to December 2011. RESULTS: Of the 111 patients, there were 57 boys and 54 girls. The mean age was 7.26 years (range, 2 months - 19 years). The most common symptoms of presentation were headache (27%) and vomiting (19%). The most common locations were the cerebral hemispheres (38%), followed by the brainstem (27.4%), and cerebellum (18.5%). Histological examination was performed in 89 patients (80.18%). Pilocytic astrocytoma was the most common histological type. Diagnostic biopsy was performed in 20 patients (22.5%), partial resection in 38 patients (42.7%), and total resection in 31 patients (34.8%). Sixteen patients received chemotherapy (14%), and eighteen patients received radiotherapy (16%). Overall survival was 88.3%. Long term hearing, visual and endocrine sequelae were note in 1, 5, and 4 patients, respectively. CONCLUSIONS: The most common histological type is pilocytic astrocytoma. Overall survival was 88.3%. Only 9% of patients had some kind or auditory, visual or endocrine sequelae

Candidiasis hepatoesplénica en pacientes hemato-oncológicos pediátricos / Hepatosplenic candidiasis in paediatric haematology-oncology patients

Introducción: En las últimas décadas, la supervivencia en las enfermedades hemato-oncológicas infantiles ha aumentado gracias a la mejora de los tratamientos, con regímenes quimioterápicos más agresivos. Esto ha conducido a un aumento en la incidencia de infecciones graves, con la consecuente morbimortalidad. La candidiasis hepatoesplénica es una infección diseminada por Candida que suele afectar más frecuentemente al hígado y bazo, aunque pueden verse involucrados otros órganos. Pacientes y métodos: Se ha realizado un estudio retrospectivo de 13 pacientes pediátricos diagnosticados de candidiasis hepatoesplénica desde enero de 2002 a febrero de 2010 en el Servicio de Hemato-Oncología pediátrica del Hospital Niño Jesús de Madrid siguiendo los criterios propuestos por la EORTC/MSG (European Organization for Research and Treatment of Cancer and Mycoses Study Group) revisados en el 2008. Se analizaron las características clínicas, los métodos diagnósticos, el tratamiento realizado y la evolución posterior. Resultados: El síntoma más frecuente de presentación de la candidiasis hepatoesplénica en nuestra serie fue la fiebre persistente, hasta en un 84,6% de los casos, en casi todos, asociada a algún otro síntoma. Los factores de riesgo para desarrollarla son bastante inespecíficos y estaban presentes hasta en un 92,3% de nuestros pacientes. El diagnóstico de infección probada, que se realiza mediante histología o cultivos, normalmente es difícil de obtener y sólo se logró obtener en nuestro caso en un 23,1%. La mayoría de las veces, como en nuestra serie, sólo se llega a un diagnóstico de sospecha. Las pruebas de laboratorio no tienen utilidad en la población pediátrica y tan sólo en un paciente se apreció aumento de la fosfatasa alcalina al diagnóstico. Este resultado difiere del comportamiento de este marcador biológico en los adultos. Aunque suelen ser necesarios tratamientos prolongados para la resolución de las lesiones, la supervivencia es muy alta, en nuestra serie un 100%, con un tratamiento adecuado. Conclusión: La mortalidad de la candidiasis hepatoesplénica es baja si se realiza un tratamiento precoz y adecuado (AU)

Introduction: In the last few decades, the survival of children with haematology-oncological malignancies has increased due to more aggressive chemotherapy regimens. This has led to an increase of fungal infections causing significant morbidity and mortality in these patients. Hepatosplenic candidiasis is a disseminated candida infection that affects most commonly the liver and spleen, although other organs may be involved. Patients and methods: We performed a retrospective study of 13 paediatric patients diagnosed with hepatosplenic candidiasis from January 2002 to February 2010 in our paediatric haematology-oncology department following the criteria proposed by the EORTC/MSG (European Organization for Research and Treatment of Cancer and Mycoses study group) updated in2008. We analysed the clinical characteristics, diagnostic methods, treatment and outcome. Results: The most common symptom of presentation of hepatosplenic candidiasis in our series was persistent fever, up 84.6% of cases, almost all associated with other symptoms. Risk factors for development are non-specific and were present in 92.3% of our patients. The diagnosis of proven infection, which is made by histology or culture, is usually difficult to obtain and in our series it was obtained only in 23.1% of cases. As in our series, diagnosis is often presumptive. Laboratory tests are not useful in the paediatric population and only in one patient we observed an increase in alkaline phosphatase at diagnosis. This result differs from the behaviour of this biological marker in adults. Although prolonged treatment is often necessary for the resolution of the lesions, survival is high with appropriate treatment, 100% in our series. Conclusions: The mortality of hepatosplenic candidiasis is low with early and adequate treatment (AU)