Desmoplastic small round cell tumor (DSRCT) is an extremely rare, aggressive sarcoma affecting adolescents and young adults with male predominance. Generally, it originates from the serosal surface of the abdominal cavity. The hallmark characteristic of DSRCT is the EWSR1–WT1 gene fusion. This translocation up-regulates the expression of PDGFRα, VEGF and other proteins related to tumor and vascular cell proliferation. Current management of DSRCT includes a combination of chemotherapy, radiation and aggressive cytoreductive surgery plus intra-peritoneal hyperthermic chemotherapy (HIPEC). Despite advances in multimodal therapy, outcomes remain poor since the majority of patients present disease recurrence and die within three years. The dismal survival makes DSRCT an orphan disease with an urgent need for new drugs. The treatment of advanced and recurrent disease with tyrosine kinase inhibitors, such as pazopanib, sunitinib, and mTOR inhibitors was evaluated by small trials. Recent studies using comprehensive molecular profiling of DSRCT identified potential therapeutic targets. In this review, we aim to describe the current studies conducted to better understand DSRCT biology and to explore the new therapeutic strategies under investigation in preclinical models and in early phase clinical trials.
Desmoplastic small round cell tumor (DSRCT) is an extremely rare, aggressive sarcoma affecting adolescents and young adults with male predominance. Generally, it originates from serosal surface of abdominal cavity. The hallmark characteristic of DSRCT is the EWSR1-WT1 gene fusion. This translocation up-regulates the expression of PDGFRα, VEGF and other proteins related to tumor and vascular cell proliferation. Current management of DSRCT includes a combination of chemotherapy, radiation and aggressive cytoreductive surgery plus intra-peritoneal hyperthermic chemotherapy (HIPEC). Despite advances in multimodal therapy, outcomes remain poor since the majority of patients present disease recurrence and die within 3 years. The dismal survival makes DSRCT an orphan disease with urgent need of new drugs. The treatment of advanced and recurrent disease with tyrosine kinase inhibitors, such as pazopanib, sunitinib, and mTOR inhibitors have been evaluated in small studies. Recent works using comprehensive molecular profiling of DSRCT identified potential therapeutic targets. In this review, we aim to describe the current studies conducted to better understand DSRCT biology and to explore the new therapeutic strategies under investigation in preclinical models and in early phase clinical trials.
RESUMO: O fogo é um elemento da mudança global que pode influenciar as comunidades fúngicas, com intuito de compreender como o fogo influência essas comunidades, foi realizado uma revisão sistemática com meta-análise de 30 estudos que abordassem o tema respostas fúngicas ao fogo. Dos estudos avaliados 56,67% obtiveram resultados positivos para o uso do fogo em função da comunidade fúngica e 43,33% apresentaram efeitos negativos, 56,66% das análises dos microrganismos fúngicos foram realizadas através da coleta do solo. As respostas dos microrganismos fúngicos ao fogo diferiram significativamente entre os tipos de vegetação, a biomassa fúngica aumentou após incêndios em Florestas boreais, mas não após incêndios em Florestas temperadas e tropicais, a Floresta tropical não apresentou diferenças entre os incêndios florestais e queima prescrita. Foi verificado nesta meta-análise que os incêndios florestais levam a uma maior redução da comunidade fúngica do que as queimadas prescritas. Os mecanismos ou processos responsáveis pela diversidade de espécies de fungos após o uso do fogo tanto para incêndios como queima prescrita permanecem desconhecidos. Desta forma a padronização das metodologias é necessária para que se possa realizar comparações entre os estudos da mesma tipologia de vegetação. Meta-analysis of studies on the effect of fire on forest biomes in relation to fungal microorganisms ABSTRACT: Fire is an element of global change that can influence fungal communities, thus participating in the dynamics of ecosystems. In order to understand how fire influences these communities, a meta-analysis of 30 fungal responses published to fire was carried out. From the studied studies 56,67% obtained positive results for the use of fire as a function of the fungal community and 43,33% presented negative effects, 56,66% of the analysis of the fungal microorganisms was done through the collection of the soil. The responses of fungal microorganisms to fire differed significantly between vegetation types and fungal biomass increased after fires in boreal forests, but not after fires in temperate and tropical forests, the tropical forest did not present differences between forest fires and prescribed burning. It was verified in this meta-analysis that the forest fires lead to a greater reduction of the fungal community than the prescribed fires. The mechanisms or processes responsible for the diversity of fungal species after the use of fire for both fire and prescribed burning remain unknown. In this way the standardization of methodologies is necessary so that comparisons can be made between the studies of the same typology of vegetation.
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