Wilms Tumor - Pathogenesis, diagnosis, treatment and prognosis
DOI:
https://doi.org/10.12775/QS.2022.08.04.004Keywords
wilms tumor, wilm's tumorAbstract
Introduction: Wilms tumor is the most common occuring malignant tumor of kidney among children. Mostly appears in infants and young children. The main symptom is enlargement od the abdomen. It is often diagnosed incidentally during USG examination.
Aim of the study: Summary of current knowledge on the pathogenesis, diagnosis, treatment and prognosis of Wilms Tumor.
Methods and matherials: A review of the literaturę available in the PubMed data base, using the following keywords: :„Wilms Tumor”,„Wilms tumor genetics”
State of knowledge: Wilms tumor appears more often among people with certain genetic syndromes. There are two to treat WT depending on the world region (SIOP, COG). According to SIOP, the diagnosis is made based on imaging. This is followed by neoadiuvant chemotherapy, and then it is surgically removed. According to COG, the first stage of treatment is surgical removal of the tumor, then chemotherapy. During chemotherapy, vincristine, actinomycin, doxodubicin are used in both schemes. Although both strategies differ in their pretreatment approach, they lead to similar overall survival rates.
Conclusions:Despite already advanced treatment techniques for the tumor, and extensive knowledge about it, its pathogenesis still remains somewhat unknown. Increased understanding of the aberrant molecular pathways active in Wilms tumorigenesis has identified many potential targeted therapeutic approaches that could be applied in a clinical setting
References
L. Pater et al., “Wilms tumor,” Pediatr Blood Cancer, vol. 68, no. S2, May 2021, doi: 10.1002/pbc.28257.
G. Pastore, A. Znaor, F. Spreafico, N. Graf, K. Pritchard-Jones, and E. Steliarova-Foucher, “Malignant renal tumours incidence and survival in European children (1978–1997): Report from the Automated Childhood Cancer Information System project,” Eur J Cancer, vol. 42, no. 13, pp. 2103–2114, Sep. 2006, doi: 10.1016/j.ejca.2006.05.010.
E. Steliarova-Foucher et al., “International incidence of childhood cancer, 2001–10: a population-based registry study,” Lancet Oncol, vol. 18, no. 6, pp. 719–731, Jun. 2017, doi: 10.1016/S1470-2045(17)30186-9.
K. Pritchard-Jones et al., “Omission of doxorubicin from the treatment of stage II–III, intermediate-risk Wilms’ tumour (SIOP WT 2001): an open-label, non-inferiority, randomised controlled trial,” The Lancet, vol. 386, no. 9999, pp. 1156–1164, Sep. 2015, doi: 10.1016/S0140-6736(14)62395-3.
F. Spreafico et al., “Wilms tumour,” Nat Rev Dis Primers, vol. 7, no. 1, p. 75, Oct. 2021, doi: 10.1038/s41572-021-00308-8.
T. D. Treger, T. Chowdhury, K. Pritchard-Jones, and S. Behjati, “The genetic changes of Wilms tumour,” Nat Rev Nephrol, vol. 15, no. 4, pp. 240–251, Apr. 2019, doi: 10.1038/s41581-019-0112-0.
J. H. Aldrink et al., “Update on Wilms tumor,” J Pediatr Surg, vol. 54, no. 3, pp. 390–397, Mar. 2019, doi: 10.1016/j.jpedsurg.2018.09.005.
T. Israels, M. Harif, and K. Pritchard-Jones, “Treatment of Wilms tumor in low-income countries: challenges and potential solutions,” Future Oncology, vol. 9, no. 8, pp. 1057–1059, Aug. 2013, doi: 10.2217/fon.13.81.
G. M. Vujanić et al., “The UMBRELLA SIOP–RTSG 2016 Wilms tumour pathology and molecular biology protocol,” Nat Rev Urol, vol. 15, no. 11, pp. 693–701, Nov. 2018, doi: 10.1038/s41585-018-0100-3.
C. v. Fernandez et al., “Outcome and Prognostic Factors in Stage III Favorable-Histology Wilms Tumor: A Report From the Children’s Oncology Group Study AREN0532,” Journal of Clinical Oncology, vol. 36, no. 3, pp. 254–261, Jan. 2018, doi: 10.1200/JCO.2017.73.7999.
M. M. van den Heuvel-Eibrink et al., “Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP–RTSG 2016 protocol,” Nat Rev Urol, vol. 14, no. 12, pp. 743–752, Dec. 2017, doi: 10.1038/nrurol.2017.163.
C. Pasqualini et al., “Outcome of patients with stage IV high-risk Wilms tumour treated according to the SIOP2001 protocol: A report of the SIOP Renal Tumour Study Group,” Eur J Cancer, vol. 128, pp. 38–46, Mar. 2020, doi: 10.1016/j.ejca.2020.01.001.
P. F. Ehrlich et al., “Surgical protocol violations in children with renal tumors provides an opportunity to improve pediatric cancer care: a report from the Children’s Oncology Group,” Pediatr Blood Cancer, vol. 63, no. 11, pp. 1905–1910, Nov. 2016, doi: 10.1002/pbc.26083.
M. v. Nelson, M. M. van den Heuvel-Eibrink, N. Graf, and J. S. Dome, “New approaches to risk stratification for Wilms tumor,” Curr Opin Pediatr, vol. 33, no. 1, pp. 40–48, Feb. 2021, doi: 10.1097/MOP.0000000000000988.
G. M. Vujani?, B. Sandstedt, D. Harms, A. Kelsey, I. Leuschner, and J. de Kraker, “Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood,” Med Pediatr Oncol, vol. 38, no. 2, pp. 79–82, Feb. 2002, doi: 10.1002/mpo.1276.
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Copyright (c) 2022 Maria Milczek, Paweł Warzyszak, Wojciech Żołyniak, Mikołaj Tomasik, Izabela Hawranik, Ziemowit Żaba, Szymon Niski, Róża Małek, Aleksandra Lisowska, Mateusz Skrzypek
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