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.

Bilateral Wilms' tumor in a child with Denys-Drash syndrome: novel frameshift variant disrupts the WT1 nuclear location signaling region.

OBJECTIVES: Wilm's Tumor (WT) is the most common pediatric kidney cancer. Whereas most WTs are isolated, approximately 5% are associated with syndromes such as Denys-Drash (DDS), characterized by early onset nephropathy, disorders of sex development and predisposition to WT. CASE PRESENTATION: A 46,XY patient presenting with bilateral WT and genital ambiguity without nephropathy was heterozygous for the novel c.851_854dup variant in WT1 gene sequence. This variant affects the protein generating the frameshift p.(Ser285Argfs*14) that disrupts a nuclear localization signal (NLS) region. CONCLUSIONS: This molecular finding is compatible with the severe scenario regarding the Wilm's tumor presented by the patient even though nephropathy was absent.

Compromiso renal de los pacientes con tumor de Wilms bilateral / Kidney involvement in patients with bilateral Wilms tumor

El tumor de Wilms (TW) es el tumor renal maligno más frecuente en la infancia. Entre el 5-7% de los pacientes se presentan con enfermedad TW bilateral, de forma sincrónica o metacrónica. El TW bilateral generalmente ocurre en pacientes pequeños y más frecuentemente en niñas. La cirugía es un componente crítico en el tratamiento para lograr una alta tasa de curación preservando la mayor cantidad de masa renal. La quimioterapia preoperatoria a menudo reduce significativamente el tamaño del tumor, facilitando la cirugía posterior. Los pacientes con TW bilateral deben ser tratados en centros con experiencia en esta patología. El manejo de estos pacientes es aún un desafío (AU)

Wilms tumor (WT) is the most common malignant kidney tumor in childhood. Between 5-7% of the patients present with synchronous or metachronous bilateral WT. Bilateral WT typically occurs in young patients and most frequently in girls. Surgery is a critical component of the treatment as it has a high rate of cure while preserving the largest kidney mass. Preoperative chemotherapy often significantly reduces the size of the tumor, facilitating subsequent surgery. Patients with bilateral WT should be managed at centers with experience treating this disease. The management of these patients is still a challenge (AU)