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Wilms' tumor

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. more...

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Approximately 500 cases are diagnosed in the U.S. annualy. Majority (75%) occur in otherwise normal children; a minority (25%) is associated with other developmental abnormalities.


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

It can be associated with a WAGR complex. This complex includes Wilms' Tumor, aniridia, genitourinary malformation, and mental motor retardation.


Pathologically, 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.

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:

  • Favorable - Contains well developed components mentioned above
  • Anaplastic - Contains diffuse anaplasia (poorly developed cells)

Staging and treatment

Staging is determined by combination of imaging studies, and pathologic findings if the tumor is operable (adapted from Treatment strategy is determined by the stage:

Stage I (43% of patients)

For stage I Wilms' tumor, 1 or more of the following criteria must be met: - Tumor is limited to the kidney and is completely excised. - The surface of the renal capsule is intact. - The tumor is not ruptured or biopsied (open or needle) prior to removal. - No involvement of renal sinus vessels. - No residual tumor apparent beyond the margins of excision.

Treatment: Nephrectomy + 18 weeks of chemotherapy

Outcome: 98% 4-year survival; 85% 4-year survival if anaplastic

Stage II (23% of patients)

For Stage II Wilms' tumor, 1 or more of the following criteria must be met: - Tumor extends beyond the kidney but is completely excised. - No residual tumor apparent at or beyond the margins of excision. - Any of the following conditions may also exist: -- Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma. -- The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank.


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Immunohistochemical Expression of Wilms Tumor Gene Protein in Different Histologic Subtypes of Ovarian Carcinomas
Context.-Immunohistochemical expression of Wilms tumor gene protein (WT1) has previously been described in primary ovarian carcinomas. Objective.-To evaluate ...
Wilms' tumor
Wilms' tumor is a cancerous tumor of the kidney that usually occurs in young children.
Protocol for the examination of specimens from patients with Wilms tumor (nephroblastoma) or other renal tumors of childhood
The College of American Pathologists offers these protocols to assist pathologists in providing clinically useful and relevant information when reporting ...
Adult extrarenal wilms tumor occurring the uterus: A case report and review of the literature
A Case Report and Review of the Literature Five previous cases of extrarenal Wilms tumor (EWT) occurring in the uterus have been reported. The oldest ...
Diagnosis and referral of Wilms' Tumor
ABSTRACT Wilms' Tumor, also know as nephroblastoma, is a childhood renal tumor. The assessment and diagnosis of a Wilms' tumor is one of the many challenges ...
Teratoid Wilms tumor arising as a botryoid growth within a supernumerary ectopic ureteropelvic structure
We report a case of a teratoid Wilms tumor arising within a supernumerary ectopic ureteropelvic structure in a 7year-old boy. The tumor was near the ...
Protocol for the examination of specimens from patients with carcinomas of renal tubluar origin, exclusive of Wilms tumor tumors of urothelial origin
This protocol is intended to assist pathologists in providing clinically useful and relevant information as a result of the examination of surgical specimens.
Wilms' tumor
Definition Wilms' tumor is a cancerous tumor of the kidney that usually occurs in young children. Description When an unborn baby is developing, ...

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