Agrawal M, Chowhan AK. Paediatric renal tumors: An insight into molecular characteristics, histomorphology and syndromic association. World J Nephrol 2025; 14(2): 99380 [DOI: 10.5527/wjn.v14.i2.99380]
Corresponding Author of This Article
Amit K Chowhan, MD, Professor, Department of Pathology and Lab Medicine, All India Institute of Medical Sciences (AIIMS), Raipur 492099, Chhattisgarh, India. chowhanpath@aiimsraipur.edu.in
Research Domain of This Article
Urology & Nephrology
Article-Type of This Article
Review
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Nephrol. Jun 25, 2025; 14(2): 99380 Published online Jun 25, 2025. doi: 10.5527/wjn.v14.i2.99380
Paediatric renal tumors: An insight into molecular characteristics, histomorphology and syndromic association
Mousmi Agrawal, Amit K Chowhan
Mousmi Agrawal, Amit K Chowhan, Department of Pathology and Lab Medicine, All India Institute of Medical Sciences (AIIMS), Raipur 492099, Chhattisgarh, India
Author contributions: Agrawal M contributed to material preparation, first draft of manuscript and literature search; Chowhan AK contributed to literature search and editing of manuscript.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Open Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Amit K Chowhan, MD, Professor, Department of Pathology and Lab Medicine, All India Institute of Medical Sciences (AIIMS), Raipur 492099, Chhattisgarh, India. chowhanpath@aiimsraipur.edu.in
Received: July 21, 2024 Revised: December 19, 2024 Accepted: January 14, 2025 Published online: June 25, 2025 Processing time: 262 Days and 3.7 Hours
Abstract
Paediatric renal tumors are rare and accounts for about 7% of all paediatric malignant tumors. The spectrum of paediatric renal tumors ranges from benign to malignant. Benign tumors include cystic nephroma, metanephric tumors and ossifying renal tumor of infancy. Tumor with low grade malignancy includes mesoblastic nephroma. Malignant tumors are nephroblastoma, clear cell sarcoma, malignant rhabdoid tumor, anaplastic sarcoma and Ewing sarcoma. Additionally, there are molecularly defined renal tumors, which includes renal cell carcinoma (RCC) with MiT translocations, ALK-rearranged RCC, eosinophilic solid and cystic RCC and SMARCB1- deficient renal medullary carcinoma. These tumors apart from having characteristic clinical presentation and histomorphology, also carry typical molecular mutations and translocations. Certain renal tumors have association with various genetic syndromes such as Beckwith-Weidmann syndrome, Wilm’s tumor, aniridia, genitourinary anomalies and mental retardation syndrome, Denys-Drash syndrome, rhabdoid tumor predisposition syndrome and DICER syndrome. This review article focusses on molecular characteristics, histomorphology and syndromic association of pediatric renal tumors, their immunohistochemical approach to diagnosis with recent updates in molecularly defined renal tumors.
Core Tip: This article outlines about paediatric renal tumors. They can have asymptomatic to symptomatic presentation. Every tumor has its own unique histomorphology, immunohistochemistry and molecular pathology. Certain tumors have association with genetic syndromes, which makes it prognostically more challenging for children. Knowledge and awareness of these tumors are essential for their accurate diagnosis and early treatment.
