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== Definition/Description ==
== Definition/Description ==
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Wilms’ Tumor, also known as nephroblastoma, is a solid tumor of the kidney that develops from immature kidney cells.<sup>1</sup> It is the most common cancer of the kidneys in children, and the fourth most common type of cancer in children.<sup>1,2</sup> Wilms’ tumors are most often unilateral, affecting only one kidney.<sup>3</sup> 5-10% of children with Wilms’ tumors have more than one tumor in the same kidney, and approximately 5% of children with the cancer have bilateral Wilms’ tumors, affecting both kidneys.<sup>3</sup>
== Prevalence ==
== Prevalence ==
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Wilms’ tumor is most common in children ages 3 to 4 and becomes much less common after the age of 5.
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sup>2</sup> Wilms’ tumor affects boys are girls equally, and can also be found in adults, though it is very rare.<sup>1</sup> On average, there are 460 new cases of Wilms’ tumor diagnosed in the United States every year. Approximately 1 in 8,000-10,000 children are affected.<sup>1</sup> About 5% of all cancers in children are Wilms’ tumors.<sup>3</sup
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== Characteristics/Clinical Presentation ==
== Characteristics/Clinical Presentation ==
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Wilms’ tumor is often hard to find early because the tumor can grow large without any signs or symptoms. These children may look and act normally.<sup>3 </sup>The most common first clinical sign is swelling or a hard mass in the abdomen. It is often firm and large enough to be palpated on both sides of the abdomen, and is usually not painful.<sup>3 </sup><br>Some children may also experience:<sup>2,3<br></sup>-Fever<br>-Loss of appetite<br>-Shortness of breath<br>-Constipation<br>-Nausea<br>-Blood in the urine<br>-Abdominal pain<br>Wilms’ tumors may also cause high blood pressure. The blood pressure can get high enough to cause bleeding in the eye or a change in consciousness in rare cases.<sup>3<br></sup>
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== Associated Co-morbidities ==
== Associated Co-morbidities ==
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== Etiology/Causes ==
== Etiology/Causes ==
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Wilms’ tumor is typically seen in otherwise healthy children, and the exact cause in most children is unknown.<sup>4,5</sup> However, approximately 10% of patients with the tumor have a congenital anomaly, including certain birth defect syndromes and genetic changes.5 Wilms’ tumor, whether hereditary or sporadic, shows evidence of changes in one or more of at least ten different genes.<sup>4</sup> <br>Common syndromes that are associated with Wilms’ tumor include the following: <br>WAGR Syndrome: WAGR stands for Wilms tumor, Aniridia (lack of the iris of the eyes), Genitourinary tract abnormalities, and mental retardation. Children who have WAGR syndrome have approximately 30-50% chance of developing a Wilms’ tumor, and often have them bilaterally. In this syndrome, cells are missing part of chromosome 11, which involves the WT1 gene.<sup>3,6</sup> <br>Beckwith-Wiedemann sydrome: These children are at a 5% risk of having a Wilms’ tumor. This syndrome is caused by a malformation of chromosome 11.<sup>3</sup> <br>Denys-Drash Syndrome: This syndrome is also caused by mutations to the WT1 gene. The risk for Wilms’ tumor in these patients is greater than 90%.<sup>6</sup>
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== Systemic Involvement ==
== Systemic Involvement ==