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Update on Management of Pediatric Renal Neoplasms

  • Pediatric Urology (BA VanderBrink and RP Pramod, Section Editors)
  • Published:
Current Treatment Options in Pediatrics Aims and scope Submit manuscript

Abstract

Purpose of Review

The treatment of pediatric renal tumors is nuanced and complex. This review highlights these nuances and aims to prepare the surgeon for treating these complex patients.

Recent Findings

Cooperative group study has improved survival for several pediatric renal tumors significantly, namely, Wilms tumor and clear cell sarcoma. There are several more rare tumors however, which have not achieved the same level of success and still require more study to find the optimal treatment regimen to achieve excellent overall survival. These tumors include rhabdoid tumor and renal medullary carcinoma. Several COG Wilms tumor protocols have recently closed and new ones are set to release soon. It is anticipated that these studies will continue to shift focus to minimizing adverse events/late effects by potentially eliminating or reducing chemotherapeutic exposures while maintaining excellent overall survival. Specifically, new discovery of molecular markers in Wilms tumor will play a significant role with respect to risk stratification. Already, loss of heterozygosity at both 1p and 16q prompts treatment intensification as they are associated with decreased survival. However, gain of 1q, independent of 1p/16q mutations, seems to be more common and even more tightly associated with poor outcomes, which will likely be a key factor involved with adjuvant therapy considerations.

Summary

New treatment protocols for Wilms tumor are forthcoming and will include more molecular markers for risk stratification. Other COG studies on more rare renal tumors are underway to further improve survival for patients with these malignancies.

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Abbreviations

ADC:

Apparent diffusion coefficient

AI:

Artificial intelligence

AYA:

Adolescent and young adult

CCSK:

Clear cell sarcoma of the kidney

CMN:

Congenital mesoblastic nephroma

COG:

Children’s Oncology Group

CT:

Computed tomography

DWI:

Diffusion weighted imaging

EFS:

Event-free survival

FH:

Favorable histology

IV:

Intravenous

IVC:

Inferior vena cava

LOH:

Loss of heterozygosity

LN:

Lymph node

MRI:

Magnetic resonance imaging

NSS:

Nephron sparing surgery or partial nephrectomy

NWTS:

National Wilms Tumor Study

OS:

Overall survival

RCC:

Renal cell carcinoma

RTK:

Rhabdoid tumor of the kidney

SIOP:

International Society for Pediatric Oncology

tRCC:

Translocation renal cell carcinoma

UH:

Unfavorable histology or anaplasia

US:

Ultrasound

WT:

Wilms tumor

XRT:

Radiation

Y:

Years

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Authors and Affiliations

  1. Division of Pediatric Urology, Monroe Carell Jr. Children’s Hospital at Vanderbilt, Nashville, TN, USA

    Leslie Peard MD

  2. Division of Pediatric Urology, Texas Children’s Hospital, Houston, TX, USA

    Niccolo Passoni MD

  3. Department of Urology, Atrium Health Levine Children’s Hospital, Charlotte, NC, USA

    Jonathan P. Walker MD

  4. Departments of Urology & Pediatrics, University of Kentucky, Lexington, KY, USA

    Amanda F. Saltzman MD

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  1. Leslie Peard MD
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All authors wrote the main manuscript text, created tables and reviewed the manuscript.

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Correspondence to Amanda F. Saltzman MD.

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Leslie Peard declares that she has no conflict of interest. Niccolo Passoni declares that he has no conflict of interest. Jonathan P. Walker declares that he has no conflict of interest. Amanda F. Saltzman declares that she has no conflict of interest.

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Peard, L., Passoni, N., Walker, J.P. et al. Update on Management of Pediatric Renal Neoplasms. Curr Treat Options Peds 9, 147–164 (2023). https://doi.org/10.1007/s40746-023-00287-w

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