Nephron sparing surgery and surgical management of intravascular extension of Wilms tumor.

Although general treatment approaches for Wilms tumor differ between Children's Oncology Group and Société Internationale d'Oncologie Pédiatrique-Renal Tumors Study Group protocols, complex tumors that may be candidates for nephron sparing surgery (NSS) and those with intravascular tumor extension represent a management challenge. In both of these scenarios, anatomic considerations are important in guiding management, making these areas of significant similarities in management between the international groups. This paper aims to explore the current approaches to NSS and intravascular tumor extension by both international groups, with attention to the evidence supporting these approaches and current knowledge gaps.

Insights Into the Somatic Mutation Burden of Hepatoblastomas From Brazilian Patients.

Hepatoblastoma is a very rare embryonal liver cancer supposed to arise from the impairment of hepatocyte differentiation during embryogenesis. In this study, we investigated by exome sequencing the burden of somatic mutations in a cohort of 10 hepatoblastomas, including a congenital case. Our data disclosed a low mutational background and pointed out to a novel set of candidate genes for hepatoblastoma biology, which were shown to impact gene expression levels. Only three recurrently mutated genes were detected: CTNNB1 and two novel candidates, CX3CL1 and CEP164. A relevant finding was the identification of a recurrent mutation (A235G) in two hepatoblastomas at the CX3CL1 gene; evaluation of RNA and protein expression revealed upregulation of CX3CL1 in tumors. The analysis was replicated in two independents cohorts, substantiating that an activation of the CX3CL1/CX3CR1 pathway occurs in hepatoblastomas. In inflammatory regions of hepatoblastomas, CX3CL1/CX3CR1 were not detected in the infiltrated lymphocytes, in which they should be expressed in normal conditions, whereas necrotic regions exhibited negative labeling in tumor cells, but strongly positive infiltrated lymphocytes. Altogether, these data suggested that CX3CL1/CX3CR1 upregulation may be a common feature of hepatoblastomas, potentially related to chemotherapy response and progression. In addition, three mutational signatures were identified in hepatoblastomas, two of them with predominance of either the COSMIC signatures 1 and 6, found in all cancer types, or the COSMIC signature 29, mostly related to tobacco chewing habit; a third novel mutational signature presented an unspecific pattern with an increase of C>A mutations. Overall, we present here novel candidate genes for hepatoblastoma, with evidence that CX3CL1/CX3CR1 chemokine signaling pathway is likely involved with progression, besides reporting specific mutational signatures.

Posicionamento cirúrgico em cirurgia robótica pediátrica: relato de experiência / Surgical Positioning in Pediatric Robotic Surgery: experience report / Posicionamiento quirúrgico en cirugía robótica pediátrica: relato de experiencia

Objetivo: Relatar a experiência dos primeiros seis casos de cirurgia robótica pediátrica e a atuação dos enfermeiros especialistas em cirurgia robótica em uma instituição oncológica. Método: Trata-se de um relato de experiência vivenciada na prática clínica que fomentou a discussão acerca dos primeiros seis casos de cirurgia robótica pediátrica, ocorridos entre 2017 e 2019. Resultados: O processo de implantação da cirurgia robótica pediátrica começou em 2017, com dois casos iniciais (adrenalectomia e nefrectomia). Todos os casos foram acompanhados e executados na presença do cirurgião urologista proctor para auxiliar a cirurgiã oncológica pediátrica. O posicionamento cirúrgico depende do local a ser abordado e do procedimento robótico que será realizado. Conclusão: Identificou-se a necessidade de uma simulação realística com a participação de todos os membros da equipe para melhoria dos processos assistenciais e da educação permanente na formação da equipe.

Objective: To report the experience of the first six cases of pediatric robotic surgery and the role of nurses who are specialists in robotic surgery in an oncology institution. Method: This is an account of experience report in clinical practice which encouraged the discussion about the first six cases of pediatric robotic surgery, which occurred between 2017 and 2019. Results: The process of implantation of pediatric robotic surgery started in 2017, with two initial cases (adrenalectomy and nephrectomy). All cases were followed up and performed in the presence of the proctor urologist surgeon to assist the pediatric oncology surgeon. Surgical positioning depends on the location to be approached and the robotic procedure that will be performed. Conclusion: The need for a realistic simulations with the participation of all team members to improve the care processes and permanent education in team formation was identified.

Objetivo: informar la experiencia de los primeros seis casos de cirugía robótica pediátrica y el papel de las enfermeras especialistas en cirugía robótica en una institución de oncología. Método: Este es un relato de la experiencia en la práctica clínica, que fomentó la discusión sobre los primeros seis casos de cirugía robótica pediátrica, que ocurrieron entre 2017 y 2019. Resultados: El proceso de implantación de cirugía robótica pediátrica comenzó en 2017, con dos casos iniciales (adrenalectomía y nefrectomía). Todos los casos fueron seguidos y realizados en presencia del cirujano urólogo supervisor para ayudar al cirujano oncólogo pediátrico. El posicionamiento quirúrgico depende de la ubicación a abordar y del procedimiento robótico que se realizará. Conclusión: Se identificó la necesidad de una simulación realista con la participación de todos los miembros del equipo para mejorar los procesos de atención y la educación permanente en la formación del equipo.