Tumor of Wilms

The tumor of Wilms or néphroblastome represents more than 90% of the tumors of the kidney in the child. This cancerous pathology is responsible for 6 to 8% of cancers of the child.

This tumor develops starting from fabric at the origin of the kidney: Blastema metanephrotic of the embryo. The current models of carcinogenesis indicate that a genetic mutation lets persist this embryonic fabric after the intra-uterine life.

The tumor of Wilms can be isolated or fall under a generally genetic syndrome. The very large majority of the children reached of tumor of Wilms syndromic have embryonic vestiges:

intralobulaire the embryonic presence of fabric is associated with two genetic diseases per change of the gene WT1 the Syndrome WAGR and the Syndrome of Denys-Drash.
périlobulaire the embryonic presence of fabric is associated the Syndrome with Beckwith-Wiedemann.

The tumor of Wilms has a local and general malignant potential. It is generally presented in the form of an intra-abdominal mass in a quite bearing child. Abdominal pains, fever, a hématurie or an anemia meet only in one third of the cases.

5 to 10% of the children have a bilateral attack of the kidneys or with metastases. The Middle Age of the diagnosis lies between 42 and 47 months in the event of unilateral tumor and 30 and 33 months in the event of bilateral tumor. If there exist intra-uterine forms with beginning, this pathology becomes very rare after the 8 years age.

The diagnosis is suspecté by the medical imagery while starting with echography. The scanner and the I.R.M are essential in the second time. But the final diagnosis rests on the anatomo-pathological examination (biopsy). In 10 to 15% of the cases the tumor of Wilms is regarded as hereditary.

The surgery is the base of the treatment of this tumor, either from the start, or after a chemotherapy. chemotherapy associates the Actinomycine D and the Vincristine.

Sources

Jeffrey S Dome, Vicki Huff, Wilms Tumor Overview In GeneTests: Medical Genetics Information Resource (database online). Copyright, University off Washington, Seattle. 1993-2006

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