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Current challenges in Wilms' tumor management

Abstract

Wilms' tumor is a renal cancer that predominantly affects children during the first 2 years of life. The continuing success of clinical trials in Wilms' tumor patients over the past 30 years has led to an overall survival of 85%, and treatment-related morbidity has been reduced. Less-aggressive chemotherapeutic regimes are available for patients with validated good prognostic factors, such as low stage and favorable histology. It is becoming increasingly apparent that treatment can be optimized through stratification of patients according to tumor stage and histology. Established treatments for Wilms' tumor include perioperative vincristine and actinomycin, with or without doxorubicin or radiotherapy. Relapsed patients have the option of salvage chemotherapy with ifosfamide, carboplatin and etoposide, as well as high-dose chemotherapy regimes and autologous hemopoietic stem-cell rescue. Further research is required to refine these regimes and identify further the role of additional prognostic factors in this childhood disease. In this article we discuss the most-debated issues and advances that have been made in the management of Wilms' tumor.

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Correspondence to Manit Arya.

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Glossary

CRYPTORCHIDISM

A congenital disorder where one or both of a newborn's testicles fail to descend into the scrotum

ANIRIDIA

A rare congenital eye condition leading to the absence of the iris of the eye

BECKWITH–WIEDEMANN SYNDROME (BWS)

A syndrome of unknown etiology but probable genetic cause, whereby children have organ enlargement, large body size, umbilical hernia and neonatal hypoglycemia

PERLMAN SYNDROME

A rare autosomal-recessive condition with macrosomia and a high incidence of Wilms' tumor

SOTOS SYNDROME

A relatively common neurologic disorder characterized by prenatal and postnatal overgrowth, advanced bone maturation, large skull with characteristic facial features, and significant developmental delay

SIMPSON–GOLABI–BEHEMEL SYNDROME

Characterized by a wide variety of clinical manifestations including prenatal and postnatal overgrowth syndrome

BLOOM SYNDROME

An inherited genetic condition caused by an unusually high number of chromosomal breaks; typical features include stunted growth, skin color alterations, susceptibility to infections, respiratory illness and cancer

DENYS–DRASH SYNDROME

A rare disorder consisting of the triad of congenital nephropathy, Wilms tumor, and intersex disorders resulting from mutations in the WT1 tumor-suppressor gene

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Gommersall, L., Arya, M., Mushtaq, I. et al. Current challenges in Wilms' tumor management. Nat Rev Clin Oncol 2, 298–304 (2005). https://doi.org/10.1038/ncponc0196

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  • DOI: https://doi.org/10.1038/ncponc0196

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