What are the syndromes associated with 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.

What does Wilms tumor look like on ultrasound?

A Wilms tumor may also appear as a multiloculated mass. Antenatal diagnosis is possible. On sonograms, mesoblastic nephroma is seen as a large, solitary, predominantly solid, coarse, and echogenic renal mass that may contain cystic areas. It may be associated with polyhydramnios.

What is Denys-Drash Syndrome?

General Discussion. Denys-Drash syndrome (DDS) is characterized by abnormal kidney function (congenital nephropathy), a cancerous tumor of the kidney called Wilms tumor, and disorders of sexual development in affected males. Most affected females have normal genitalia.

What is the most common presenting feature of Wilms tumour?

Wilms’ tumour (nephroblastoma) is the most common intrarenal malignancy of childhood. The most common presentation is of an asymptomatic abdominal mass (more than 80%), usually discovered incidentally. Abdominal pain occurs in 30% to 40% of patients, and hypertension, frank haematuria and fever are found in 5% to 30%.

What is the life expectancy of someone with Wilms tumor?

For Wilms tumors, survival is often measured using a 4-year survival rate. This refers to the percentage of children who live at least 4 years after their cancer is diagnosed. For example, a 4-year survival rate of 80% means that an estimated 80 out of 100 children who have that cancer are still alive 4 years after being diagnosed.

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?

Wilms’ tumor has many causes, which can broadly be categorized as syndromic and non-syndromic. Syndromic causes of Wilms’ tumor occur as a result of alterations to genes such as the Wilms Tumor 1 (WT1) or Wilms Tumor 2 (WT2) genes, and the tumor presents with a group of other signs and symptoms.

What are the first symptoms of Wilms tumor?

But most children with Wilms’ tumor experience one or more of these signs and symptoms: An abdominal mass you can feel Abdominal swelling Abdominal pain