What are the syndromes associated with Wilms tumor?
WAGR (Wilms tumor-Aniridia-Genitourinary malformation-Retardation) syndrome. Denys-Drash syndrome (DDS) Frasier syndrome (FS) Genitourinary anomalies (abnormalities of the reproductive and urinary systems) syndrome.
What causes WAGR syndrome?
WAGR syndrome is caused by a deletion of genetic material on the short (p) arm of chromosome 11. The size of the deletion varies among affected individuals. The signs and symptoms of WAGR syndrome are related to the loss of multiple genes on the short arm of chromosome 11.
What are characteristics of Wilms tumor?
Some affected children have abdominal pain, fever, a low number of red blood cells (anemia ), blood in the urine (hematuria), or high blood pressure (hypertension). Additional signs of Wilms tumor can include loss of appetite, weight loss, nausea, vomiting, and tiredness (lethargy).
Why is called Wilms tumor?
Wilms’ tumor, also known as nephroblastoma, is a cancer of the kidneys that typically occurs in children, rarely in adults. It is named after Max Wilms, the German surgeon (1867–1918) who first described it.
What is Denys Drash Syndrome?
Denys-Drash syndrome is characterized by kidney disease that begins within the first few months of life. Affected individuals have a condition called diffuse glomerulosclerosis, in which scar tissue forms throughout glomeruli, which are the tiny blood vessels in the kidneys that filter waste from blood.
What is the biologic behavior of Wilms tumor?
The biologic behavior of Wilms tumor is difficult to predict on the basis of histopathologic findings alone[4]. The mean age at diagnosis is 3.5 years. The most common feature at presentation is an abdominal mass. Other signs and symptoms include abdominal pain, hypertension, fever, hematuria and anemia.
What is Wilms tumor and how is it treated?
Treatment for Wilms’ tumor usually involves surgery and chemotherapy, and sometimes radiation therapy. Treatments may vary by the stage of the cancer. Because this type of cancer is rare, your child’s doctor may recommend that you seek treatment at a children’s cancer center that has experience treating this type of cancer.
What causes Wilms tumor?
Causes and risk factors for Wilms’ tumor. It’s not clear what exactly causes Wilms’ tumors.
What are the first symptoms of Wilms tumor?
Abdominal pain
What is the difference between neuroblastoma and Wilms tumor?
calcification uncommon: 10-15% (10% rule of Wilms tumor)