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The treatment of bilateral Wilms tumors (BWT) involves curing the cancer, preserving long-term renal function, and maintaining a good quality of life. Established methods for achieving these goals include preoperative chemotherapy and nephron-sparing surgery (NSS). This study aimed to evaluate the experience of a single institution in treating patients with BWT. We analyzed cases of BWT treated at the Pediatric Oncology Institute-GRAACC-Federal University of São Paulo over a period of 35 years. Bleeding control was performed with manual compression of the renal parenchyma. Thirty-three patients were included in the study. Thirty cases were synchronous tumors. The mean age at diagnosis was 30.4 months (±22 m) and 66.7% were girls. The median follow-up period was 83 months. Neoadjuvant chemotherapy was the primary approach in most patients (87.9%), with a simultaneous upfront surgical approach performed in 84.8%. Most patients underwent bilateral NSS (70.4%). There were no early complications in this series, but 39.4% had clinical complications. The five-year survival rate was 76%. Therefore, it is clear that the surgical approach to BWT plays a crucial role in achieving good outcomes. However, it is difficult to standardize surgical techniques and technology may have the potential to enhance safety.

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El nefroma mesoblastico congénito es un tumor renal infrecuente, pero común en el periodo neonatal. De comportamiento benigno y pronóstico excelente en su variedad clásica pero en el subtipo celular puede presentar recurrencia local, invasión retroperitoneal y metástasis pulmonares y cerebrales. El tratamiento es la nefrectomía radical. Los factores pronósticos son la edad menor a tres meses y la resección quirúrgica completa. Paciente de 17 días nacida a las 35 semanas por polihidramnios, con diagnóstico prenatal de masa abdominal. Al examen físico masa en flanco derecho e hipertensión arterial (121/79 mm Hg, TAM 77mm Hg). Ecografía abdominal con riñón derecho aumentado de tamaño y voluminosa formación sólida, de 4,4 x 4,3 x 4,8 cm, heterogénea y vascularizada al Doppler en polo inferior. Tomografía de abdomen con formación nodular heterogénea de 5,0 x 4,1 x 5,1 cm en polo inferior del riñón derecho. Se realizó nefrectomía radical derecha sin complicaciones. Evolución en los últimos 20 meses satisfactoria y libre de enfermedad.

Congenital mesoblastic nephroma is an uncommon renal tumor, but frequent in neonates. Classical variety usually benign with excellent prognosis. Cellular cases may be recurrent with retroperitoneal invasion, brain and lung metastasis. Radical nephrectomy is the treatment of choice. Relevant prognostic factors are diagnosis before three months of age and complete resection. A 17 day old patient, borned at 35 weeks due to polyhydramnios, with prenatal diagnosis of abdominal mass. On examination, right flank abdominal mass and arterial hypertension (121/79 mm Hg, MAP 77 mm Hg). Abdominal ultrasound showed an enlarged right kidney with a voluminous solid heterogeneous mass, 4.4 x 4.3 x 4.8 cm in the lower pole, vascularized on Doppler scan. Computed tomography showed a big 5.0 x 4.1 x 5.1 cm, nodular, heterogeneous mass in the lower pole of the right kidney. Right radical nephrectomy was performed with an uneventful recovery. Follow up for the last 20 months satisfactory and free of disease.

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We herein report the challenging evaluation and planning process involved in performing the first successful surgical resection of a renal tumor with extensive inferior vena cava tumor thrombosis reaching the right atrium in a pediatric patient within the Central American region. In November 2018, the Oncology Department of the National Children's Hospital in Costa Rica consulted our Center for Liver Transplantation and Hepatobiliary Surgery for the evaluation of a clinical case involving a 6-year-old female patient with progressive Budd-Chiari syndrome caused by a Wilms' tumor of the right kidney with tumor thrombosis of the inferior vena cava reaching the right atrium. A multistage surgical safety strategy combining liver transplant techniques and cardiac surgery was thereafter designed and implemented, achieving complete excision of the tumor thrombus from the inferior vena cava with right nephrectomy. Postoperatively, the patient exhibited complete clinical resolution of Budd-Chiari syndrome and has remained tumor free with excellent quality of life while pursuing her second grade of primary school education 22 months after the successful implementation of this multistage surgical safety strategy. The combination of liver transplantation techniques and cardiac surgery based on a multistage surgical safety strategy minimized the occurrence of unexpected intraoperative events and allowed for complete renal tumor resection and level IV thrombectomy for the first time in a pediatric patient of a public health system in a developing country within the Central American region.

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PURPOSE OF REVIEW: To update epidemiological, diagnostic, and therapeutic information on primary synovial sarcoma of the kidney. RECENT FINDINGS: A total of 96 studies were analyzed; age at presentation was 38.6±14.2 years, predominant location of tumor was right kidney; frequent reported symptoms at diagnosis were hematuria and pain. For definitive diagnosis, cytogenetic technique was used. Detected oncogene was available in 37.8% cases with fusion of SS18-SSX in most patients. Surgery is treatment of choice, with adjuvant chemotherapy; most frequently ifosfamide-based associated with doxorubicin or epirubicin. Overall median survival was 34 months. Mortality was 29% of the cases which reported death and the recurrence rate was 39.8%. Risk of death was increased in patients with metastases at diagnosis Primary RSS occurs more often in young men. RSS often presents with symptoms and in an advanced stage. Surgical treatment is the most commonly used and chemotherapy for advanced or recurrent treatment.

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BACKGROUND: Gastric carcinoma (GC) with second primary malignancy (SPM) is the most frequent combination within the multiple primary malignancies (MPM) group. The presentation of a GC associated with a synchronized SPM in the kidney is extremely rare and unusual. This study presents a rare case of synchronous tumors, describes the main associated risk factors, and emphasizes the need to rule out SPM. MAIN BODY: We present the case of a 63-year-old Hispanic woman with a history of smoking, weight loss, and gastrointestinal (GI) bleeding. GC was diagnosed by endoscopy, and during her workup for metastatic disease, a synchronous SPM was noted in the left kidney. The patient underwent resection of both tumors with a satisfactory postoperative course. A systematic review of the literature was performed using the Medline/PubMed, Science Direct, Scopus, and Google Scholar databases. A search of the literature yielded 13 relevant articles, in which the following main risk factors were reported: the treatment utilized, the grade and clinical stage, histopathological report, and in some cases survival. It is concluded that advanced age (> 60 years) and smoking are the main associated risk factors. CONCLUSION: Gastric carcinoma is the second most frequent neoplasm of the GI tract and the main neoplasm that presents a SPM. MPM screening is recommended in patients with gastric cancer. The clinical discovery of MPM of renal origin is rare and hence the importance of the current report.

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Primary renal neoplasms are infrequent in domestic animals and birds, being budgerigars an exception. This report describes a papillary renal adenoma in a toco toucan (Ramphastos toco), which was euthanized due to poor prognosis after surgical intervention of phalanx fractures in both wings. Grossly, the caudal pole of the right kidney had a nodular lesion measuring 3.5 x 1.7 x 1.5 cm. Microscopically, there was a well delimited neoplasm arranged in large and coalescent papillary structures composed by a single layer of epithelial cells. There was mild to moderate nuclear and cytoplasmic pleomorphism, whereas mitotic figures were absent. In conclusion, this tumor was considered as an accidental finding, which is similar to the majority of adenomas reported in domestic mammals.

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Primary renal neoplasms are infrequent in domestic animals and birds, being budgerigars an exception. This report describes a papillary renal adenoma in a toco toucan (Ramphastos toco), which was euthanized due to poor prognosis after surgical intervention of phalanx fractures in both wings. Grossly, the caudal pole of the right kidney had a nodular lesion measuring 3.5 x 1.7 x 1.5 cm. Microscopically, there was a well delimited neoplasm arranged in large and coalescent papillary structures composed by a single layer of epithelial cells. There was mild to moderate nuclear and cytoplasmic pleomorphism, whereas mitotic figures were absent. In conclusion, this tumor was considered as an accidental finding, which is similar to the majority of adenomas reported in domestic mammals.(AU)

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Introducción: el adenocarcinoma renal representa hasta el 80 por ciento de los tumores sólidos de este órgano, además de ser el de peor pronóstico. Objetivo: analizar la influencia del estadio tumoral inicial en la sobrevida de pacientes con adenocarcinoma renal. Métodos: se realizó un estudio descriptivo en una muestra de 69 pacientes con diagnóstico de adenocarcinoma renal, atendidos en el Hospital Militar Central Dr. Luis Díaz Soto de enero de 2005 a diciembre de 2014. Las variables estudiadas fueron: edad, sexo, signos y síntomas, exámenes imaginológicos utilizados, estadio tumoral, modalidades terapéuticas empleadas, complicaciones presentadas y la sobrevida posterior al tratamiento. Resultados: prevaleció el grupo de edades de 60-69 años con 46 pacientes (66,7 por ciento) y del sexo masculino con 42 para un 61 por ciento. Predominó el diagnóstico incidental en 52 pacientes (75,4 por ciento). El ultrasonido se empleó en la totalidad de los casos y fue positivo en el 98,6 por ciento. Preponderaron las lesiones en el estadio I en 32 pacientes (46,3 por ciento). La modalidad terapéutica más empleada fue el tratamiento quirúrgico en 65 pacientes (94,2 por ciento), al ser la nefrectomía total por vía lumbar la más practicada con 44 pacientes (63,8 por ciento). La sobrevida a los 5 años fue del 93,7 por ciento. Conclusiones: El diagnóstico del adenocarcinoma renal en estadios iniciales, posibilitó mejor pronóstico y mayor sobrevida de los pacientes. Existió relación directa entre los factores pronósticos, clínicos y tumorales, con la evolución y la mortalidad por adenocarcinoma renal(AU)

Introduction: the renal adenocarcinoma represents until 80 percent of the solid tumors of this organ, besides being that of worse prognosis. Objective: to analyze the influence of the initial tumoral stage in the survival of patient with renal adenocarcinoma. Methods: A descriptive was carried in to sample of 69 patients with diagnosis of renal adenocarcinoma, assisted in the Central Military Hospital Dr. Luis Díaz Soto from January of 2005 to December of 2014. The studied variables were: age, sex, signs and symptoms, imaginologic exams used, tumoral stage, therapeutic modalities employed, complications and the survival later to the treatment. Results: the group of 60-69 year-old ages prevailed with 46 patients (66.7 percent) and of the masculine sex with 42 for 61 percent. The incidental diagnosis prevailed in 52 patients (75.4 percent). The ultrasound was used in the entirety of the cases and it was positive in 98.6 percent. Prevailed the lesions in the I stage in 32 patients (4.3 percent). The therapeutic modality more employed was the surgical treatment in 65 patients (94.2 percent), being the total nephrectomy for lumbar via were practiced in 44 patients (63.8 percent). The survival to the 5 years was of the one 93.7 percent. Conclusions: The diagnosis of the renal adenocarcinoma, in initial stages, facilitates a better prognosis and a bigger survival of the patients. Direct relationship exists among the prognostic factors, clinical and tumoral, with the evolution and the mortality for renal(AU)

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Resumen Objetivo: describir las características clínicas del grupo de niños diagnosticados con tumor de Wilms en Costa Rica y cuál es la evolución clínica de acuerdo con los diversos tratamientos que reciben en el Servicio de Oncohematología del Hospital Nacional de Niños. Métodos: este es un estudio descriptivo retrospectivo que analiza un periodo de 20 años de pacientes con diagnóstico de tumor de Wilms confirmado por biopsia. Se revisó 69 expedientes de pacientes con edades entre 0 y 13 años, en los cuales se analizaron los datos clínicos, terapéuticos (quimioterapia, radioterapia) y pronósticos (sobrevida global y libre de enfermedad) de cada uno. Los resultados obtenidos se compararon con los de otros estudios latinoamericanos y europeos. Se realizaron modificaciones en el manejo de los pacientes costarricenses, con base en los resultados obtenidos. Resultados: se analizó una muestra total de 69 casos. Entre los resultados se encontró que los pacientes presentaban una edad promedio diagnóstica de 41,3 meses. El seguimiento promedio fue de 7,4 años. La masa abdominal fue el hallazgo clínico predominante (55%). Además, el estadio III fue el más común (31,8%). La nefrectomía total sin ruptura tumoral fue el procedimiento quirúrgico en la mayoría de los casos. El 80% de los pacientes presentó histología favorable en el análisis histopatológico. En el 51% de los casos se utilizó quimioterapia prequirúrgica. Un 17% de los pacientes presentó metástasis pulmonar. La sobrevida global fue del 73,3% y la sobrevida libre de enfermedad, del 69%. Conclusiones: los pacientes costarricenses con tumor de Wilms localizados tuvieron una sobrevida inferior a la de los países desarrollados, y similar en casos metastásicos.

Abstract Objective: to describe the clinical characteristics of the group of children diagnosed with Wilms' tumor in Costa Rica and what was the clinical evolution according to the different treatments received in the Oncohematology Division of the National Children's Hospital. Methods: this is a retrospective descriptive study that analyzed the patients with diagnosis of Wilms tumor confirmed by biopsy in period of 20 years. We reviewed 69 records of patients aged between 0 and 13 years, in which the clinical, therapeutic (chemotherapy, radiotherapy) and prognostic data (global and disease-free survival) of each one were analyzed. Results: a total sample of 69 cases was analyzed. Among the results, it was found that the patients presented an average age of 41.3 months at the moment of diagnosis. The average follow-up was 7.4 years. The presence of an abdominal mass was the predominant clinical finding (55%). In addition, stage III was the most common (31.8%). Total nephrectomy without tumor rupture was the surgical procedure in the majority of cases. 80% of the patients presented favorable histology in the histopathological analysis. In 51% of cases, pre-surgical chemotherapy was used. 17% of the patients presented pulmonary metastasis. Overall survival was 73.3% and disease free survival was 69%. Conclusions: Costa Rican patients with localized Wilms' tumor had poorer results than that of patients in developed countries, but the survival was similar in metastatic cases.

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