Surgery in Bilateral Wilms Tumor-A Single-Center Experience.

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.

Management of Pediatric Tumors With Vascular Extension.

Background: Pediatric tumors can present with vascular extension to the inferior vena cava and right atrium, which impacts the surgical strategy and can be challenging during surgical treatment. Wilms tumor (WT) is the most common retroperitoneal tumor that can present with vascular extension, but also adrenal tumors, clear cell tumors from the kidney, and hepatoblastomas can present with this situation. Surgical aims include obtaining complete tumor resection without risk for patients, to avoid severe bleeding, cardiac arrest, and embolization, and to avoid cardiac bypass if possible. Objective: To describe and discuss the surgical strategies to deal with pediatric tumors with vascular extension and propose a protocol. Method: Retrospectivly review the experience of treating patients with vascular extension in a single institution, describing different scenarios and a decision making fluxogram based on the preoperative evaluation regarding the surgical techniques and the need for cardiac bypass that are adequate for each situation. Image studies are important to guide the surgical strategy. Depending on the quality of image available, computerized tomography (CT) or magnetic resonance imaging (MRI) can be enough to give the information needed for surgical decisions. Ultrasonography (US) with Doppler is helpful to confirm diagnosis and describes factors to guide the adequate surgical strategy, like the upper level extension and presence or absence of blood flow around the thrombus. Neoadjuvant chemotherapy is indicated in most cases, in order to reduce the upper level of extension (and avoid the need for cardiac bypass) and to lower the risk of embolization. The approach is based on the upper level of the thrombus and can include cavotomy or cavectomy, sometimes with cardiac bypass and cardiac arrest with hypothermia, when the thrombus reaches the diaphragmatic level or above. Pathology analysis of the thrombus can guide staging and the need for radiotherapy postoperatively. Results: A decision making fluxogram protocol is presented focusing on the surgical treatment of such condition. Conclusion: Surgery strategy is highly impacted by the presence of vascular extension in pediatric tumors. Surgeons should be aware of potential complications and how to prevent them. Such cases should be treated in reference centers.

Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report.

INTRODUCTION: Desmoplastic small round cell tumor is an extremely rare and aggressive cancer that affects mainly adolescents and young adults. Despite multiple therapeutic strategies, most patients have resistant disease with very poor survival rates. CASE PRESENTATION: We present a case of a 10-year-old Caucasian boy with a desmoplastic small round cell tumor refractory to conventional treatment who exhibited a good response to alternative treatment. With use of irinotecan and vincristine in association with radiation therapy, a reduction of 96.9% of the dimensions of the target lesions compared with the initial image was observed. CONCLUSION: This chemotherapy regimen, in association with radiation therapy, demonstrated efficacy for refractory desmoplastic small round cell tumor in our patient, and it is cost-effective.

DO THE RADIOLOGICAL CRITERIA WITH THE USE OF RISK FACTORS IMPACT THE FORECASTING OF ABDOMINAL NEUROBLASTIC TUMOR RESECTION IN CHILDREN?

BACKGROUND: The treatment of neuroblastoma is dependent on exquisite staging; is performed postoperatively and is dependent on the surgeon's expertise. The use of risk factors through imaging on diagnosis appears as predictive of resectability, complications and homogeneity in staging. AIM: To evaluate the traditional resectability criteria with the risk factors for resectability, through the radiological images, in two moments: on diagnosis and in pre-surgical phase. Were analyzed the resectability, surgical complications and relapse rate. METHODS: Retrospective study of 27 children with abdominal and pelvic neuroblastoma stage 3 and 4, with tomography and/or resonance on the diagnosis and pre-surgical, identifying the presence of risk factors. RESULTS: The mean age of the children was 2.5 years at diagnosis, where 55.6% were older than 18 months, 51.9% were girls and 66.7% were in stage 4. There was concordance on resectability of the tumor by both methods (INSS and IDRFs) at both moments of the evaluation, at diagnosis (p=0.007) and post-chemotherapy (p=0.019); In this way, all resectable patients by IDRFs in the post-chemotherapy had complete resection, and the unresectable ones, 87.5% incomplete. There was remission in 77.8%, 18.5% relapsed and 33.3% died. CONCLUSIONS: Resectability was similar in both methods at both pre-surgical and preoperative chemotherapy; preoperative chemotherapy increased resectability and decreased number of risk factors, where the presence of at least one IDRF was associated with incomplete resections and surgical complications; relapses were irrelevant.

Do the radiological criteria with the use of risk factors impact the forecasting of abdominal neuroblastic tumor resection in children? / Critérios radiológicos impactam a previsão de ressecabilidade em neuroblastomas abdominais na criança com o uso de fatores de risco?

ABSTRACT Background: The treatment of neuroblastoma is dependent on exquisite staging; is performed postoperatively and is dependent on the surgeon's expertise. The use of risk factors through imaging on diagnosis appears as predictive of resectability, complications and homogeneity in staging. Aim: To evaluate the traditional resectability criteria with the risk factors for resectability, through the radiological images, in two moments: on diagnosis and in pre-surgical phase. Were analyzed the resectability, surgical complications and relapse rate. Methods: Retrospective study of 27 children with abdominal and pelvic neuroblastoma stage 3 and 4, with tomography and/or resonance on the diagnosis and pre-surgical, identifying the presence of risk factors. Results: The mean age of the children was 2.5 years at diagnosis, where 55.6% were older than 18 months, 51.9% were girls and 66.7% were in stage 4. There was concordance on resectability of the tumor by both methods (INSS and IDRFs) at both moments of the evaluation, at diagnosis (p=0.007) and post-chemotherapy (p=0.019); In this way, all resectable patients by IDRFs in the post-chemotherapy had complete resection, and the unresectable ones, 87.5% incomplete. There was remission in 77.8%, 18.5% relapsed and 33.3% died. Conclusions: Resectability was similar in both methods at both pre-surgical and preoperative chemotherapy; preoperative chemotherapy increased resectability and decreased number of risk factors, where the presence of at least one IDRF was associated with incomplete resections and surgical complications; relapses were irrelevant.

RESUMO Racional: O tratamento do neuroblastoma é dependente de estadiamento primoroso, realizado no pós-cirúrgico e dependente da expertise do cirurgião. O uso de fatores de risco através da imagem ao diagnóstico surge como preditivo de ressecabilidade, complicações e homogeneidade no estadiamento. Objetivos: Avaliar o critério de ressecabilidade tradicional com os fatores de risco para ressecabilidade, através das imagens radiológicas, em dois momentos no diagnóstico e no pré-cirúrgico analisando a ressecabilidade, complicações cirúrgicas e índice de recidiva. Métodos: Estudo retrospectivo em 27 crianças com neuroblastoma estádios 3 e 4 em abdome e pelve, e com tomografia e/ou ressonância no diagnóstico e pré-cirúrgico, identificando-se a presença de fatores de risco. Resultados: A idade média das crianças foi de 2,5 anos ao diagnóstico, onde 55,6% estavam acima dos 18 meses, 51,9% eram meninas e 66,7% tinham estádio 4. Houve concordância da ressecabilidade do tumor pelos dois métodos avaliados (INSS e IDRFs) e em ambos os momentos da avaliação, ao diagnóstico (p=0,007) e pós-quimioterapia (p=0,019). Desta forma todos pacientes ressecáveis por IDRFs no pós-quimioterapia tiveram ressecção completa; já nos irressecáveis, 87,5% tiveram ressecção incompleta. Houve remissão em 77,8%, 18,5% recaíram e 33,3% morreram. Conclusões: Aressecabilidade foi semelhante em ambos os métodos tanto no diagnóstico como no pré-cirúrgico. A quimioterapia pré-operatória aumentou a ressecabilidade e diminuição do número de fatores de risco, onde a presença de ao menos um IDRF associou-se às ressecções incompletas e complicações cirúrgicas. As recidivas foram irrelevantes.

Variation of the Rex shunt for treating concurrent obstruction of the portal and superior mesenteric veins.

Children with extrahepatic portal vein obstruction can be managed successfully by surgical intervention and should be evaluated for potential meso-Rex bypass. A Rex shunt variation is described to treat portal and superior mesenteric vein thrombosis. This technique uses the internal jugular vein as a conduit between the splenic vein and the left portal vein with splenic preservation.