Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees

<p>Abstract</p> <p>Background</p> <p>Esthesioneuroblastoma (ENB) is a rare, aggressive tumor with no established treatment in children. We analyzed a series of pediatric ENB patients with the aim of improving our knowledge of this disease.</p> <p>Methods</p> <p>9 patients (6 males; age 0.9-18 years, median 9.9) were identified by searching the AIEOP (<it>Italian Association of Pediatric Hematology and Oncology</it>) registry and the national databases of rare tumors, soft tissue sarcomas (STS) and neuroblastomas. The data on the cases included in STS treatment protocols were collected prospectively and histology was centrally reviewed; the data and histology concerning the other children were reviewed for the purpose of this analysis.</p> <p>Results</p> <p>All tumors occurred in the sinonasal region with bone erosion (7 patients) and intracranial (4) or intraorbital (4) extension. Three patients were in Kadish stage B, and 6 in stage C. Complete tumor resection was very difficult to achieve, but adding chemotherapy and radiotherapy enabled tumor control in 8 patients. Response to chemotherapy was evident in 5/7 evaluable cases. Radiotherapy (48.5-60 Gy) was delivered in all children but one, due to early disease progression. With a median follow-up of 13.4 years (range 9.2-22.9), 7 patients are alive in 1^stand one in 2nd complete remission. All surviving patients developed treatment-related sequelae, the most frequent being endocrine dysfunctions (4 patients) and craniofacial growth impairments (4 patients).</p> <p>Conclusions</p> <p>Our findings confirm that ENB in children has an aggressive presentation, but multimodal therapy can cure most patients. Our results are encouraging but future strategies must optimize treatment in terms of survival and related morbidities.</p

The effects of age on substrate depletion and hormonal responses during submaximal exercise in hamsters

Senescent hamsters display a marked reduction in volume of voluntary running. The purpose of this study was to determine whether age differences exist in the pattern of fuel utilization during submaximal exercise, which may account for the reduction in voluntary running. Further, we determined the effects of age on muscle oxidative capacity to assess its relationship to endurance performance in senescent hamsters Depletion of carbohydrate and lipid content of skeletal muscle and liver, and changes in blood concentration of various hormones and substrates during one hour of exercise at 60 percent of VO2 max served to assess age effects on utilization of metabolic substrates. Exercise produced equivalent depletion of muscle glycogen and similar rise in plasma free fatty acids in young and old hamsters. No exercise effects on skeletal muscle triglyceride concentration or on plasma glycerol, glucagon or catecholamine concentrations were noted. With palmitoyl carnitine as substrate (but not with pyruvate) State 3 respiration of cardiac and skeletal muscle homogenates was lower in old compared to young hamsters. Although old hamsters have a reduced capacity to oxidize lipids in vitro, few age differences in fuel use are evident in vivo during submaximal exercise. Thus, these minor age differences in substrate utilization do not likely account for the substantial reduction in the levels of spontaneous running in senescent hamsters.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/26840/1/0000400.pd

A Bienal do Vazio: análise comparativa das estratégias de tomada de posição dos jornais O Estado de S. Paulo e Jornal Semanal da 28ª Bienal de São Paulo

This paper proposes a comparative analysis of cultural critical, articles and reports related to the 28th International Biennial of Art of São Paulo published in the newspapers O Estado de S. Paulo and Jornal Semanal da 28ª Bienal de São Paulo, from October to December 2008. Specific aims of analysis are how an institution devoted, like Biennial, creates mechanisms to defend their choices and hegemonic position within the arts field and also to examine how this dedication can be demystified by critics from diffusion field not linked to the institution. For the study will be used the concepts of field, hierarchy of legitimacy and position of an agent developed by Pierre Bourdieu, as well as historical notions of Cultural Journalism and journalistic criticismEste trabalho propõe uma análise comparativa das críticas, artigos e reportagens culturais relacionadas à 28ª Bienal Internacional de Arte de São Paulo publicadas nos jornais O Estado de S. Paulo e Jornal Semanal da 28ª Bienal de São Paulo, no período de outubro a dezembro de 2008. São objetivos específicos da pesquisa analisar como uma instituição consagrada, como a Bienal, cria mecanismos para defender suas escolhas e sua posição hegemônica dentro do campo das artes e, também, analisar de que forma essa consagração pode ser desmistificada por críticos do campo da difusão não vinculados à instituição. Para a realização deste estudo serão utilizados os conceitos de campo, hierarquia das legitimidades e tomadas de posição desenvolvidos por Pierre Bourdieu, assim como as noções históricas de Jornalismo Cultural e crítica jornalístic

Management of unresectable solid papillary cystic tumor of the pancreas. A case report and literature review

Pancreatic solid papillary cystic tumor is a rare neoplasm with an excellent prognosis if surgical excision is complete. We report on a case and review 47 more cases extracted from the published literature to assess the treatment options when solid papillary cystic tumor is considered unresectable. Chemotherapy and radiotherapy were beneficial in a limited number of patients, but therapeutic decisions must be made bearing in mind that patients may be long-term survivors without any treatment because of the tumor's slow growt

Multiple synchronous tumors in a child with Fanconi anemia

Fanconi anemia (FA) is an autosomal recessive inherited syndrome characterized by congenital abnormalities, aplastic anemia, and a high likelihood of developing cancer. We describe a child who presented with 2 synchronous solid tumors (Wilms tumor and neuroblastoma), later found to have FA, who developed severe toxicity and died after a first cycle of chemotherapy. Our experience emphasizes that a predisposing genetic condition should be sought in cases of multiple tumors and that managing FA patients with cancer can be particularly difficul

Self-limited focal epilepsy in a young child with SARS-CoV-2: a case report

Self-limited focal epilepsy in a young child with SARS-CoV-2: a case repor