What syndrome is Wilms tumor?

Wilms’ tumor is associated with several congenital syndromes such as WAGR (Wilms’ tumor, aniridia, genitourinary malformation, mental retardation) syndrome, Denys-Drash syndrome, Beckwith-Wiedemann syndrome, etc.

Which is the main difference between neuroblastoma and Wilms tumor?

Neuroblastoma (NBL) is the most common extra-cranial tumour in childhood [1] and commonly presents as an abdominal mass. Nephroblastoma, also more commonly known as a Wilms’ tumour, is the commonest renal tumour in childhood and also typically presents as abdominal pathology.

Is Wilms tumor encapsulated?

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 bleeding and peritoneal dissemination of the tumor.

Can Wilms tumor be cured?

Overall, about 9 of 10 children with Wilms tumors are cured. A great deal of progress has been made in treating this disease in recent decades. Much of this progress is the result of children with Wilms tumors taking part in clinical trials of new treatments.

What are the risk factors for wilms’tumor?

Risk factors. Family history of Wilms’ tumor. If someone in your child’s family has had Wilms’ tumor, then your child has an increased risk of developing the disease. Wilms’ tumor occurs more frequently in children with certain abnormalities or syndromes present at birth, including: Aniridia.

What is the prognosis for children with wilms’tumor?

Over the years, advancements in the diagnosis and treatment of Wilms’ tumor have greatly improved the outlook (prognosis) for children with this disease. With appropriate treatment, the outlook for most children with Wilms’ tumor is very good. Signs and symptoms of Wilms’ tumor vary widely, and some children don’t show any obvious signs.

What kind of pain does a Wilms tumor cause?

Some patients with Wilms tumor have developed hepatic veno-occlusive disease (VOD). VOD is primarily a clinical diagnosis characterized by hepatomegaly or pain in the right upper quadrant, jaundice, ascites, and unexplained weight gain.

Is there a connection between Wilms tumor and Frasier syndrome?

Denys-Drash syndrome is characterized by nephrotic syndrome caused by diffuse mesangial sclerosis, XY pseudohermaphroditism, and increased risk of Wilms tumor (>90%). Frasier syndrome is characterized by progressive nephropathy caused by focal segmental glomerulosclerosis, gonadoblastoma, and XY pseudohermaphroditism.