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. 2021 Nov 26;10(23):5558.
doi: 10.3390/jcm10235558.

Bilateral Renal Tumors in Children: The First 5 Years' Experience of National Centralization in The Netherlands and a Narrative Review of the Literature

Sophie E van Peer  1 , Janna A Hol  1 , Alida F W van der Steeg  1 , Martine van Grotel  1 , Godelieve A M Tytgat  1 , Annelies M C Mavinkurve-Groothuis  1 , Geert O R Janssens  1   2 , Annemieke S Littooij  1   3 , Ronald R de Krijger  1   4 , Marjolijn C J Jongmans  1   5 , Marc R Lilien  1   6 , Jarno Drost  1   7 , Roland P Kuiper  1   5 , Harm van Tinteren  1 , Marc H W A Wijnen  1 , Marry M van den Heuvel-Eibrink  1
Affiliations

Bilateral Renal Tumors in Children: The First 5 Years' Experience of National Centralization in The Netherlands and a Narrative Review of the Literature

Sophie E van Peer et al. J Clin Med. .

Abstract

Survival of unilateral Wilms tumors (WTs) is exceeding 90%, whereas bilateral WTs have an inferior outcome. We evaluated all Dutch patients with bilateral kidney tumors, treated in the first five years of national centralization and reviewed relevant literature. We identified 24 patients in our center (2015-2020), 23 patients had WT/nephroblastomatosis and one renal cell carcinoma. Patients were treated according to SIOP-RTSG protocols. Chemotherapy response was observed in 26/34 WTs. Nephroblastomatosis lesions were stable (n = 7) or showed response (n = 18). Nephron-sparing surgery was performed in 11/22 patients undergoing surgery (n = 2 kidneys positive margins). Local stage in 20 patients with ≥1 WT revealed stage I (n = 7), II (n = 4) and III (n = 9). Histology was intermediate risk in 15 patients and high risk in 5. Three patients developed a WT in a treated nephroblastomatosis lesion. Two of 24 patients died following toxicity and renal failure, i.e., respectively dialysis-related invasive fungal infection and septic shock. Genetic predisposition was confirmed in 18/24 patients. Our literature review revealed that knowledge is scarce on bilateral renal tumor patients with metastases and that radiotherapy seems important for local stage III patients. Bilateral renal tumors are a therapeutic challenge. We describe management and outcome in a national expert center and summarized available literature, serving as baseline for further improvement of care.

Keywords: RCC; Wilms tumor; bilateral; pediatric; renal tumor; stage V.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Figures

Figure 1
Figure 1
Age distribution of the 24 bilateral renal tumor patients. WT: Wilms tumor; NB: nephroblastomatosis; RCC: renal cell carcinoma; *: the patient with RCC was 15 years old at diagnosis.
Figure 2
Figure 2
Response to preoperative chemotherapy according to histological subtype, depicted per lesion (total evaluable n = 58, Wilms tumor = 33, nephrogenic rests n = 25). LR: low-risk; IR: intermediate-risk; DA: diffuse anaplasia; high-risk blastemal type; NR: nephrogenic rest; NB: nephroblastomatosis. Response: >10% volume reduction of tumor, stable: less than 10% increase or decrease in tumor volume, progressive: >10% increase in tumor volume.
Figure 3
Figure 3
Flow diagram revealing applied surgery modality. WT: Wilms tumor; n: number of patients; NR: nephrogenic rests; NSS: nephron-sparing surgery; TN: tumor nephrectomy.
Figure 4
Figure 4
Flow diagram of stage and histology per kidney. n: number; WT: Wilms tumor, NR: nephrogenic rests; RCC: renal cell carcinoma; LR: low-risk histology; IR: intermediate-risk histology; HR: high-risk histology; + margins: positive resection margins; LN+: lymph nodes positive for tumor cells.
See this image and copyright information in PMC

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