Sacrococcygeal teratoma: evaluation of its approach, treatment and follow-up in two reference children cancer centers in Brazil / Rio de Janeiro.

INTRODUCTION: sacrococcygeal teratoma (TSC) is the most common tumor of the neonatal period. Alphafetoprotein is an important tumor marker and is used in the follow-up period as a marker of malignancy. The complete surgical resection of the tumor associated with coccygectomy is the standard treatment and chemotherapy in different stages are necessary. Follow-up consists of serial exam: tumor markers, imaging searching to possible metastasis sites, in addition to a complete physical examination. METHODOLOGY: a descriptive, retrospective, study was carried out by analyzing a chart of 25 patients of two different reference children cancer center; with TSC in the State of Rio de Janeiro from 2004 to 2019. The clinical and epidemiological data collected were described and a comparison was made between these two centers studied. RESULTS: the sociodemographic characteristics found were similar to those described in the medical literature. Data related to treatment and follow-up, such as the use of chemotherapy, use of specific imaging tests, digital rectal examination, and outpatient follow-up, differed between the two centers studied. There was a 25% loss of follow-up. CONCLUSION: the characteristic of being a non-cancer center can interfere with the full application of the current protocol for the treatment of sacrococcygeal teratoma. The knowledge of the data of the studied cases can allow the optimization of the approach of the patients with this pathology and generate discussions about the integral application of the specific therapeutic protocol in the medical centers that are qualified for such treatment.

Sacrococcygeal teratoma: evaluation of its approach, treatment and follow-up in two reference children cancer centers in Brazil / Rio de Janeiro / Teratoma sacrococcígeo: avaliação de abordagem, tratamento e seguimento em dois centros de referência do estado do Rio de Janeiro

ABSTRACT Introduction: sacrococcygeal teratoma (TSC) is the most common tumor of the neonatal period. Alphafetoprotein is an important tumor marker and is used in the follow-up period as a marker of malignancy. The complete surgical resection of the tumor associated with coccygectomy is the standard treatment and chemotherapy in different stages are necessary. Follow-up consists of serial exam: tumor markers, imaging searching to possible metastasis sites, in addition to a complete physical examination. Methodology: a descriptive, retrospective, study was carried out by analyzing a chart of 25 patients of two different reference children cancer center; with TSC in the State of Rio de Janeiro from 2004 to 2019. The clinical and epidemiological data collected were described and a comparison was made between these two centers studied. Results: the sociodemographic characteristics found were similar to those described in the medical literature. Data related to treatment and follow-up, such as the use of chemotherapy, use of specific imaging tests, digital rectal examination, and outpatient follow-up, differed between the two centers studied. There was a 25% loss of follow-up. Conclusion: the characteristic of being a non-cancer center can interfere with the full application of the current protocol for the treatment of sacrococcygeal teratoma. The knowledge of the data of the studied cases can allow the optimization of the approach of the patients with this pathology and generate discussions about the integral application of the specific therapeutic protocol in the medical centers that are qualified for such treatment.

RESUMO Introdução: teratoma sacrococcígeo é o tumor neonatal mais comum. Alfafetoproteína é um marcador tumoral importante e é utilizado no período de seguimento como um marcador de malignidade. A ressecção cirúrgica completa do tumor associado a coccigectomia é o tratamento padrão, associado a quimioterapia em determinados estadiamentos. Exames de seguimento consistem em avaliação de marcadores tumorais, investigação de sítios de metástases, além de um completo exame físico. Metodologia: foi realizado um estudo retrospectivo descritivo, através da análise de 25 pacientes em dois centros de referência em tratamento oncológico infantil, com teratoma sacrococcígeo no Estado do Rio de Janeiro, entre 2004 e 2019. Os dados clínicos e epidemiológicos foram descritos, comparando-se os dois centros. Resultados: as características socio-demográficas foram similares ao descrito na literatura médica. Os dados relativos ao tratamento e seguimento, como o uso de quimioterapia, uso de testes de imagem específicos, exame de toque e retal e seguimento ambulatorial, foi diferente entre os dois centros. Tivemos uma perda de seguimento de 25%. Conclusão: a característica de ser um centro não oncológico pode interferir com a aplicação do protocolo de tratamento de teratoma sacrococcígeo. O conhecimento dos dados estudados pode permitir a otimização da abordagem dos pacientes com esta patologia e gerar discussões sobre a aplicação integral dos protocolos terapêuticos em centros médicos que são qualificados para tal tratamento.

Tumor de Wilms Bilateral Sincrônico: Avaliação Cirúrgica e Sobrevida / Synchronous Bilateral Wilms Tumor: Surgical Evaluation and Survival / Tumor de Wilms bilateral Sincrónico: Evaluación quirúrgica y Sobrevida

Introdução: os tumores renais representam cerca de 7% de todas as neoplasias malignas da infância. o tumor de Wilms bilateral sincrônico corresponde a 5-7% de todos os nefroblastomas. o tratamento consiste em quimioterapia pré-operatória, seguida de cirurgia conservadora, quimioterapia pós-operatória e, quando indicada, radioterapia. Objetivo: analisar o tipo de cirurgia e a sobrevida global dos pacientes com tumor de Wilms bilateral sincrônico. Método: este estudo de coorte retrospectivo incluiu 18 pacientes pediátricos do Hospital do câncer i, do instituto nacional de câncer José alencar Gomes da silva, no rio de Janeiro, de janeiro de 2000 a dezembro de 2017. curvas de sobrevida foram calculadas pelo método Kaplan-Meier. Resultados: a idade mediana ao diagnóstico foi de 19 meses, sendo dez casos do sexo feminino. em 62,5% dos rins operados, foi possível realizar cirurgia conservadora e, em 41,2% (7/17) dos pacientes, em ambos os rins. a sobrevida global em cinco anos, segundo o tipo cirurgia, foi de 87,5% para cirurgia conservadora e de 62,8% para nefrectomia total (p=0,0001). a sobrevida global em cinco anos para a coorte inteira foi de 70,8%. Conclusão: a cirurgia preservadora de tecido renal em crianças com o tumor de Wilms bilateral sincrônico é viável e pode ser realizada com segurança em centros de referência.

Introduction: Kidney tumors account for about 7% of all childhood malignancies. synchronous bilateral Wilms tumor corresponds to 5-7% of all nephroblastomas. The treatment consists of preoperative chemotherapy, followed by conservative surgery, postoperative chemotherapy and, when indicated, radiotherapy. Objective: to analyze the type of surgery and the overall survival of patients with synchronous bilateral Wilms tumor. Method: This retrospective cohort study included 18 pediatric patients from the Hospital do cancer i, from the national cancer institute José alencar Gomes da silva, in rio de Janeiro, from January 2000 to december 2017. survival curves were calculated using the Kaplan-Meier method. Results:The median age at diagnosis was 19 months, and 10 patients were female. it was possible to perform conservative surgery in 62.5% of the kidneys, and in 41.2% (7/17) of patients in both kidneys. The 5-year overall survival according to the type of surgery was 87.5% for conservative surgery and 62.8% for radical nephrectomy (p=0.0001). The 5-year overall survival of the entire cohort was 70.8%. Conclusion: conservative surgery in pediatric synchronous bilateral Wilms tumor is feasible and can be performed safely in reference centers.

Introducción:los tumores renales representan alrededor del 7% de todas las neoplasias malignas de la infancia. el tumor de Wilms bilateral sincrónico corresponde al 5-7% de todos los nefroblastomas. el tratamiento consiste en quimioterapia preoperatoria, seguida de cirugía conservadora, quimioterapia postoperatoria y, cuando indicada, la radioterapia. Objetivo: analizar el tipo de cirugía y la supervivencia global de los pacientes con un tumor de Wilms bilateral sincrónico. Método: este estudio de cohorte retrospectivo incluyó 18 pacientes pediátricos del Hospital del cáncer i, del instituto nacional de cáncer José alencar Gomes da silva, nel río de Janeiro, de enero de 2000 a diciembre de 2017. las curvas de sobrevida fueron calculadas por el método Kaplan-Meier. Resultados: la edad media al diagnóstico fue de 19 meses, siendo diez casos del sexo femenino. en el 62,5% de los riñones operados fue posible realizar cirugía conservadora, siendo en el 41,2% (7/17) de los pacientes en los dos riñones. la supervivencia global en cinco años según el tipo de cirugía fue de 87,5% para cirugía conservadora y de 62,8% nefrectomía total (p=0,0001). la supervivencia global en cinco años para la cohorte entera fue del 70,8%. Conclusión: la cirugía conservadora en niños con el tumor de Wilms bilateral sincrónico es viable y puede ser realizada con seguridad en centros de referencia