Wilms' tumor

Rare childhood cancer of the kidneys
Approximately 650 cases are diagnosed in the U. S. annually. The majority of cases occur in children with no associated genetic syndromes; however, a minority of children with Wilms' tumor have a congenital abnormality.

The majority of people with Wilms' tumor present with an asymptomatic abdominal mass which is noticed by a family member or healthcare professional. Renal tumors can also be found during routine screening in children who have known predisposing clinical syndromes. Once Wilms' tumor is suspected, an ultrasound scan is usually done first to confirm the presence of an intrarenal mass. In most cases, a biopsy is not done first because there is a risk of cancer cells spreading during the procedure. Treatment in North America is nephrectomy or in Europe chemotherapy followed by nephrectomy. A definitive diagnosis is obtained by pathological examination of the nephrectomy specimen. and to determine prognosis and treatments. Staging is based on anatomical findings and tumor cells pathology. According to the extent of tumor tissue at the time of initial diagnosis, four stages are considered, with a fifth classification for bilateral involvement.

Stage IV (10% of cases) Wilms' tumor is defined by the presence of hematogenous metastases (lung, liver, bone, or brain), or lymph node metastases outside the abdominopelvic region.

5% of Wilms' tumor cases at the time of initial diagnosis are bilateral involvements, which pose unique challenges to treatment. An attempt should be made to stage each side according to the above criteria (stage I to III) on the basis of extent of disease prior to biopsy. Bilateral Wilms' tumors are as a whole placed in Stage V.

Wilms tumor is the most common malignant renal tumor in children. There are a number of rare genetic syndromes that have been linked to an increased risk of developing Wilms Tumor. Screening guidelines vary between countries; however health care professionals are recommending regular ultrasound screening for people with associated genetic syndromes. People of African descent may have slightly higher rates of Wilms' tumor. A genetic predisposition to Wilms' tumor in individuals with aniridia has been established, due to deletions in the p13 band on chromosome 11.

Sidney Farber, founder of Dana–Farber Cancer Institute, and his colleagues achieved the first remissions in Wilms' tumor in the 1950s. By employing the antibiotic actinomycin D in addition to surgery and radiation therapy, they boosted cure rates from 40 to 89 percent. The use of computed tomography scan for the diagnosis of Wilms' tumor began in the early 1970s, thanks to the intuition of Mario Costici, an Italian physician. He discovered that in the direct radiograms and in the urographic images, you can identify determining elements for a differential diagnosis with the Wilms' tumor. This possibility was a premise for starting a treatment.