Wilms tumor is a neoplasm of the kidneys that typically occurs in children. It is eponymously named after Dr Max Wilms, a German surgeon (1867-1918). It is also known as a nephroblastoma.

A triphasic nephroblastoma comprises three elements

• blastema
• mesenchyme
• epithelium

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. 1

The mesenchymal component may include cells showing rhabdomyoid differentiation . The rhabdomyoid component may itself show features of malignancy (rhabdomyosarcomatous Wilms).

Wilms tumors in Stage I or II of development lead to a full, recurrence free recovery in 95+% of cases. Usual treatment includes chemotheraphy with actinomycin and vincristine, and radiation treatment for tumors in late stage II and higher.

Wilms tumor may be sporadic or familial. Predisposition to this neoplasm forms part of some syndromes.

Wilms tumor can affect any child regardless of race, sex, country of origin, or parental occupation. The disease is mostly noticed around age three, but has been recorded in children as old as age sixteen. Most cases begin with experience of the following symptoms:

Abdominial mass

Blood in the urine

Fever

and less frequent anorexia, vomitting, and malaise

External links

Photos at: Atlas of Pathology