Multidisciplinary Treatment Strategies for Wilms Tumor: Recent Advances, Technical Innovations and Future Directions

Abstract

Significant progress has been made in the management of Wilms tumor (WT) in recent years, mostly as a result of collaborative efforts and the implementation of protocol-driven, multimodal therapy. This article offers a comprehensive overview of current multidisciplinary treatment strategies for WT, whilst also addressing recent technical innovations including nephron-sparing surgery (NSS) and minimally invasive approaches. In addition, surgical concepts for the treatment of metastatic disease, advances in tumor imaging technology and potentially prognostic biomarkers will be discussed. Current evidence suggests that, in experienced hands and selected cases, laparoscopic radical nephrectomy and laparoscopic-assisted partial nephrectomy for WT may offer the same outcome as the traditional open approach. While NSS is the standard procedure for bilateral WT, NSS has evolved as an alternative technique in patients with smaller unilateral WT and in cases with imminent renal failure. Metastatic disease of the lung or liver that is associated with WT is preferably treated with a three-drug chemotherapy and local radiation therapy. However, surgical sampling of lung nodules may be advisable in persistent nodules before whole lung irradiation is commenced. Several tumor markers such as loss of heterozygosity of chromosomes 1p/16q, 11p15 and gain of function at 1q are associated with an increased risk of recurrence or a decreased risk of overall survival in patients with WT. In summary, complete resection with tumor-free margins remains the primary surgical aim in WT, while NSS and minimally invasive approaches are only suitable in a subset of patients with smaller WT and low-risk disease. In the future, advances in tumor imaging technology may assist the surgeon in defining surgical resection margins and additional biomarkers may emerge as targets for development of new diagnostic tests and potential therapies.

Fetal Wilm's tumor detection preceding the development of isolated lateralized overgrowth of the limb: a case report and review of literature

“…However, this infant had several concerning risk factors including a large tumor (approximately 500 g), unknown status of loss of heterozygosity (LOH) of 1p/16q, and the absence of a genetic study. After discussing the case in the multidisciplinary meeting, we upgraded this tumor to intermediate risk ( 19 ). Therefore, the female infant received dose-adjusted weekly vincristine over a period of 10 weeks according to the SIOP Umbrella RTSG 2016 protocol ( 24 ) without any complications.…”

“…Thus, the diagnosis of triphasic WT or nephroblastoma was confirmed. Staging was completed with a computed tomography scan of the chest, which came back normal, and the tumor was classified as stage I according to the International Society of Pediatric Oncology (SIOP) and the National Wilms Tumor Study (NWTS) Group Staging System ( 19 ). Three weeks after surgery, adjuvant chemotherapy was started with weekly vincristine for a total of 10 weeks, without major adverse events.…”

Disseminated adult Wilms tumor in pregnancy: Leveraging multidisciplinary care in a low‐resource setting

“…This patient's diagnosis was made using an abdominal ultrasound and CT scan. Supportive tests included a full blood cell count to screen for tumor‐associated anemia and thrombocytopenia, kidney function test for potential derangement in kidney function, liver function test for altered liver enzymes in liver metastasis, and clotting profile for disrupted coagulation such as occurs in WT‐acquired von Willebrand disease 11 …”

Development and validation of a prognostic model based on a single-cell RNA-seq in Wilms tumor in children

“…2 WT affects over 90% of children with renal malignancies. 3 Its histology resembles that of a developing kidney, and is usually triphasic with blastemal, stromal, and epithelial components. 4 WT is a malignant solid tumor, mainly composed of stroma, germ, and epithelium, including epithelial tissue, muscle tissue, connective tissue, skeletal tissue, and nerve tissue.…”