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Wilms’ tumor or nephroblastoma is a tumor of the kidneys that typically occurs in children, rarely in adults.[1] Its common name is an eponym, referring to Dr. Max Wilms, the German surgeon (1867–1918) who first described this kind of tumor.[2]
Approximately 500 cases are diagnosed in the U.S. annually. The majority (75%) occur in otherwise normal children; a minority (25%) is associated with other developmental abnormalities. It is highly responsive to treatment, with about 90% of patients surviving at least five years.
Pathologically, a triphasic nephroblastoma comprises three elements:
Wilms’ tumor is a malignant tumor containing metanephric blastema, stromal and epithelial derivatives. Characteristic is the presence of abortive tubules and glomeruli surrounded by a spindled cell stroma. The stroma may include striated muscle, cartilage, bone, fat tissue, fibrous tissue. The tumor is compressing the normal kidney parenchyma.
The mesenchymal component may include cells showing rhabdomyoid differentiation. The rhabdomyoid component may itself show features of malignancy (rhabdomyosarcomatous Wilms).
Wilms tumor may be separated into 2 prognostic groups based on pathologic characteristics:
Mutations of the WT1 gene on chromosome 11 are observed in approximately 20% of Wilms tumors.[3][4] At least half of the Wilms tumors with mutations in WT1 also carry mutations in CTNNB1, the gene encoding the proto-oncogene beta-catenin.[5]
A gene on the X chromosome, WTX, is inactivated in up to 30% of Wilms tumor cases, according to research published in 2007.[6]
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