TY - JOUR
T1 - Bilateral Renal Tumors in Children
T2 - The First 5 Years' Experience of National Centralization in The Netherlands and a Narrative Review of the Literature
AU - van Peer, Sophie E
AU - Hol, Janna A
AU - van der Steeg, Alida F W
AU - Grotel, Martine van
AU - Tytgat, Godelieve A M
AU - Mavinkurve-Groothuis, Annelies M C
AU - Janssens, Geert O R
AU - Littooij, Annemieke S
AU - de Krijger, Ronald R
AU - Jongmans, Marjolijn C J
AU - Lilien, Marc R
AU - Drost, Jarno
AU - Kuiper, Roland P
AU - van Tinteren, Harm
AU - Wijnen, Marc H W A
AU - van den Heuvel-Eibrink, Marry M
PY - 2021/11/26
Y1 - 2021/11/26
N2 - 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.
AB - 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.
U2 - 10.3390/jcm10235558
DO - 10.3390/jcm10235558
M3 - Article
C2 - 34884260
SN - 2077-0383
VL - 10
JO - Journal of clinical medicine
JF - Journal of clinical medicine
IS - 23
ER -