Introduction Malignant triton tumor (MTT) is an extremely rare variant of the malignant peripheral nerve sheath tumors (MPNSTs) with rhabdomyosarcomatous differentiation, which was first described in 1932 by Mason. MTT affects, in most cases, patients under 35 years of age, and it is usually manifested as a mass that may or not be painful. However, the incidence in pediatric patients is atypical. This tumor presents an aggressive course and limited survival rate, and the prognosis is different between individuals with or without a concomitant diagnosis of neurofibromatosis type 1 (NF1). Currently, the recommended treatment is surgical resection, and adjuvant chemotherapy and radiotherapy, but its efficacy is not yet clear. Presentation of the case A 13-year-old female patient was referred to the pediatric oncology service due to the presence of an abdominal mass and weight loss, initially diagnosed with Wilms' tumor. After extensive investigation, surgical resection, and immunohistopathological evaluation, the diagnosis of malignant triton tumor was confirmed. The patient also underwent cycles of chemotherapy after resection, and is currently awaiting immunotherapy. Discussion and conclusion Malignant triton tumor is extremely rare and difficult to diagnose, especially in children or young people, age groups in which the incidence of the disease is even lower. This may be the reason it is rarely suspected, and it was a great challenge for the clinical care team. It is essential to consider and investigate this possibility of differential diagnosis, as patients diagnosed with this malignant tumor have a low survival rate and poor prognosis.
RESUMO Objetivo: avaliar epidemiologia, características anatômicas, manejo e prognóstico de pacientes críticos com fraturas de esterno. Métodos: análise retrospectiva de pacientes internados em unidade de terapia intensiva (UTI) de emergências cirúrgicas e trauma de um centro de trauma Tipo III em São Paulo, Brasil. Resultados: foram admitidos 1552 pacientes traumatizados no período de janeiro de 2012 a abril de 2016. Desses, 439 apresentavam trauma torácico e 13 apresentavam fratura de esterno, configurando 0,9% das admissões de trauma e 3% dos traumas torácicos. Desses pacientes, três apresentavam tórax instável e dois foram submetidos à conduta cirúrgica para fixação da fratura. A mortalidade de pacientes com fratura de esterno foi de 29% (três pacientes). Em um dos óbitos pôde-se atribuir a fratura do esterno como contribuinte principal para o desfecho. Conclusão: a fratura de esterno foi diagnosticada em 0,9% dos pacientes críticos vítimas de trauma em UTI especializada. Somente 15% dos pacientes necessitaram de conduta cirúrgica específica na fase aguda e a mortalidade foi decorrente das outras lesões na maior parte dos casos.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.