Bilateral Renal Tumors in Children: The First 5 Years' Experience of National Centralization in The Netherlands and a Narrative Review of the Literature
van Peer, Sophie E.; Hol, Janna A.; van der Steeg, Alida F.W.; van Grotel, Martine; Tytgat, Godelieve A.M.; Mavinkurve‐groothuis, Annelies M.C.; Janssens, Geert O.R.; Littooij, Annemieke S.; de Krijger, Ronald R.; Jongmans, Marjolijn C.J.; Lilien, Marc R.; Drost, Jarno; Kuiper, Roland P.; van Tinteren, Harm; Wijnen, Marc H.W.A.; van den Heuvel‐Eibrink, Marry M.
(2021) Journal of Clinical medicine, volume 10, issue 23, pp. 1 - 20
(Article)
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
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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.
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Keywords: bilateral, Pediatric, RCC, Renal tumor, Stage V, Wilms tumor, stage V, renal tumor, pediatric, General Medicine, Journal Article
ISSN: 2077-0383
Publisher: Multidisciplinary Digital Publishing Institute (MDPI)
Note: Funding Information: Funding: This research was (partly) funded by KiKa Stichting Kinderen Kankervrij, grant number 278. Publisher Copyright: © 2021 by the author. Licensee MDPI, Basel, Switzerland.
(Peer reviewed)