Wilms Tumor Lecture Notes

These flashcards cover epidemiology, genetics, risk factors, clinical features, diagnosis, staging, treatment, and prognosis of Wilms tumor based on the lecture notes.

What is Wilms tumor and where does it originate?

A rare childhood cancer that starts in the kidney (nephroblastoma).

Wilms tumor is the ____ most common type of childhood cancer.

Fourth.

At what ages is Wilms tumor most prevalent?

Between four and five years old; it becomes much less common after age five.

Are most Wilms tumors unilateral or bilateral?

Unilateral (affect only one kidney).

What percentage of Wilms tumor cases are bilateral?

About 5–10 %.

To which organ does Wilms tumor most commonly metastasize?

The lungs.

What genetic genes are most often implicated in Wilms tumor?

Mutations of WT1 or WT2 genes on chromosome 11.

Name three genetic syndromes linked to WT1 mutations that increase Wilms tumor risk.

Fraser syndrome, WAGR syndrome, and Denys-Drash syndrome.

Which syndrome associated with WT2 mutations raises Wilms tumor risk?

Beckwith-Wiedemann syndrome.

List four additional genetic or chromosomal conditions that raise Wilms tumor risk.

Perlman syndrome, Soto syndrome, Bloom syndrome, Trisomy 18 (Edwards syndrome), or Simpson-Golabi-Behmel syndrome (any four).

What embryologic lesions are considered precursors to Wilms tumor?

Nephrogenic rests.

Name two birth defects that increase Wilms tumor risk.

Aniridia and hemihypertrophy.

Which racial group has a higher incidence of Wilms tumor?

African-American children.

State five common symptoms of Wilms tumor.

Any five: abdominal pain, abdominal swelling/mass, fever, hematuria or discolored urine, loss of appetite, weakness/fatigue, nausea/vomiting, high blood pressure, constipation, unexplained weight loss.

What imaging tests are commonly used to help diagnose Wilms tumor?

Ultrasound, CT scan, and MRI scan.

What are the two surgical options for Wilms tumor?

Partial nephrectomy and radical nephrectomy.

Briefly describe Stage 1 Wilms tumor.

Cancer confined to the kidney and completely removable by surgery.

Which stage of Wilms tumor involves spread to distant organs such as lungs, brain, liver, or bone?

Stage 4.

What defines Stage 5 Wilms tumor?

Cancer is present in both kidneys (bilateral).

Besides surgery, name two other standard treatment modalities for Wilms tumor.

Chemotherapy and radiation therapy.

How often does the American Cancer Society recommend screening exams for high-risk children, and until what age?

Every 3–4 months until the child reaches 8 years old.

Why can rupture of a Wilms tumor be dangerous?

It may cause bleeding and peritoneal dissemination of cancer cells.

What is the general prognosis for Wilms tumor with appropriate treatment?

Very good; survival rates are high due to advances in diagnosis and therapy.

Which basic laboratory studies help assess kidney function in suspected Wilms tumor cases?

Blood and urine tests.

Name one imaging study used specifically to determine lung metastasis in Wilms tumor patients.

Chest X-ray or chest CT scan.