Wilms' tumour_Clinical Features and Management

Posted by e-Medical PPT Friday, April 1, 2011
Wilms' tumour or nephroblastoma is cancer of the kidneys that typically occurs in children, rarely in adults.
Wilms tumor may arise in 3 clinical settings, the study of which resulted in the discovery of the genetic abnormalities that lead to the disease. Wilms tumors can arise sporadically, can develop in association with genetic syndromes, or can be familial.
Most cases of Wilms tumor are not part of a genetic malformation syndrome and occur in the absence of a family history of the malignancy; however, familial Wilms tumor is very common in certain families. Genetic syndromes that predispose to and may include Wilms tumor include the following:
    *Beckwith-Wiedemann syndrome (macroglossia, gigantism, and umbilical hernia)
    *Congenital aniridia
    *Wilms tumor, aniridia, genitourinary malformations, and mental retardation (WAGR syndrome)
    *Denys-Drash syndrome (Wilms tumor, pseudohermaphroditism, and glomerulopathy)
    *Trisomy 18 mutation
The most common feature at presentation is an abdominal mass. Abdominal pain occurs in 30%-40% of cases. Other signs and symptoms of Wilms tumor include hypertension, fever caused by tumor necrosis, hematuria, and anemia.
Most nephroblastomas are unilateral, being bilateral in less than 5% of cases. They tend to be encapsulated and vascularized tumors that do not cross the midline of the abdomen. In cases of metastasis it is usually to the lung. A rupture of Wilms' tumor puts the patient at risk of hemorrhage and peritoneal dissemination of the tumor.

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