Wilms tumor: diagnosis and updates

This review walks through how pathologists identify Wilms tumor today, covering its typical appearance under the microscope and the range of variant forms it can take. It also looks at the growing list of molecular and histological markers used to classify tumors and support increasingly precise risk assessment.

 

Diagnostic Pathology — September 2nd, 2026

Although Wilms tumor is the most common malignant kidney tumor in childhood, it remains uncommon overall and can vary considerably from one case to another. The authors discuss the conditions that must be distinguished from Wilms tumor and the roles of histology, immunohistochemistry and molecular testing in establishing an accurate diagnosis.

Read more about Wilms tumor pathology and diagnosis updates​

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PRAME-specific T-cell therapy in advanced pediatric nephroblastoma

A new report describes the use of PRAME-targeted T-cell therapy in a child with advanced Wilms tumor, providing an early indication of how highly targeted immunotherapy might eventually contribute to treatment of difficult-to-treat disease.

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The New England Journal of Medicine — August 13th, 2026

In this individual patient, treatment produced a 69% reduction in tumor volume, with molecular, metabolic and histological evidence of complete remission reported at 200 days. This is a single-patient report rather than evidence of an established treatment, but the response provides an important proof-of-concept for further investigation of targeted cellular therapies in Wilms tumor.

Read more about PRAME T-cell therapy and Wilms tumor

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Lymph node sampling and outcomes in Wilms tumor

A large Children's Oncology Group analysis investigates the importance of lymph node assessment during Wilms tumor surgery and whether the number and proportion of positive nodes can provide additional prognostic information.

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2026

Lymph node evaluation is an important component of accurate Wilms tumor staging. The study analyzed more than 2,000 children with Stage I–III favorable-histology disease, adding to evidence that careful and standardized lymph node sampling remains an important part of Wilms tumor surgery.

Read more about lymph node sampling and Wilms tumor outcomes​

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Developmental reprogramming underlies chemotherapy resistance in favorable-histology Wilms tumor

Researchers have identified biological changes that may help explain why some favorable-histology Wilms tumors develop resistance to chemotherapy, potentially revealing new targets for future treatment research.

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Cell Reports — March 24th, 2026

Using laboratory models and molecular analysis, researchers found evidence that resistant Wilms tumor cells can undergo developmental reprogramming and acquire more stem-like characteristics. The study identified changes involving genes and pathways including LIN28B and ABCB1, providing new insight into the biology of chemotherapy-resistant disease.

Read more about chemotherapy resistance in Wilms tumor

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Prognostic molecular biomarkers in Wilms tumors

A major systematic review brings together evidence surrounding molecular biomarkers that could help identify children with Wilms tumor who face different risks of relapse or poorer outcomes.

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JCO Precision Oncology — May 18th, 2026

The researchers reviewed evidence involving molecular abnormalities including 1q gain, 1p and 16q changes, TP53, MYCN, WT1, SIX1/SIX2 and several microRNA-processing genes. Better understanding of these biomarkers could contribute to increasingly precise risk-adapted treatment strategies.

Read more about Wilms tumor biomarkers and genetic risk​

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Residual blastema as a predictor of Wilms tumor outcomes

Research using SIOP WT 2001 data investigates whether the absolute amount of blastema remaining following preoperative chemotherapy could provide additional information about a child's risk.

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Journal of Clinical Oncology — 2026

Blastema is one of the characteristic cellular components of Wilms tumor. Measuring how much viable blastema remains following chemotherapy could potentially refine current histological risk classification and help researchers better identify children at increased risk of recurrence.

Read more about residual blastema and Wilms tumor prognosis

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Single-nucleus RNA sequencing reveals Wilms tumor heterogeneity

Researchers have used single-nucleus RNA sequencing to examine Wilms tumor at the cellular level, revealing biological differences between cells that may not be apparent through conventional pathology alone.

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JCI Insight — 2026

The study examined the transcriptional characteristics of Wilms tumor cells and their relationship to normal kidney development. Research of this kind is helping scientists understand the developmental origins and biological diversity of Wilms tumor and could eventually contribute to more precise tumor classification.

Read more about single-cell research in Wilms tumor

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DNA methylation and transcriptomic profiles reveal new insights into Wilms tumor

Researchers have combined epigenetic and gene-expression analysis to investigate molecular characteristics that may contribute to Wilms tumor development and potentially improve tumor classification.

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International Journal of Cancer — July 30th, 2026

The study examined DNA methylation alongside gene-expression patterns to identify deregulated genes and epigenetic processes relevant to Wilms tumor biology. The findings add to growing evidence that molecular characteristics could complement conventional pathology when researchers classify tumors and investigate differences in risk.

Read more about DNA methylation and Wilms tumor epigenetics​

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Long-term effects of modern Wilms tumor treatment

A major review examines the long-term health effects that can follow successful Wilms tumor treatment and considers how follow-up can be tailored to the treatments an individual survivor received.

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Nature Reviews Urology — January 26th, 2026

Potential late effects include chronic kidney disease, cardiovascular disease, infertility and second primary cancers. The authors emphasize the importance of understanding which survivors are most vulnerable to particular treatment-related complications so that long-term monitoring and survivorship care can be better individualized.

Read more about Wilms tumor late effects and survivorship​

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Bilateral Wilms tumor: looking for the best surgical strategy

Researchers have examined how surgeons can balance cancer control with preservation of healthy kidney tissue in children whose Wilms tumor affects both kidneys.

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Annals of Surgical Oncology — June 4th, 2026

The study analyzed 96 children with synchronous bilateral Wilms tumor treated within the SIOP 2001 framework. The authors support nephron-sparing surgery when technically possible and emphasize individualized planning based on tumor anatomy, response to chemotherapy and vascular involvement.

Read more about bilateral Wilms tumor surgery

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