Wilms Tumor

by Brian Alverson, MD

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    00:01 In this lecture, we will review the Wilms tumor, which is a renal tumor in children.

    00:06 This is basically a kidney tumor arising from pluripotent embryonic renal precursor cells.

    00:12 This represents about 5 to 7% of pediatric malignancies diagnosed each year and its peak age of diagnosis is around 2 to 3 years of age.

    00:24 So, this tumor is associated with multiple genetic syndromes including the Beckwith-Wiedemann syndrome, as well as WAGR syndrome which is Wilms, Aniridia, GU abnormalities, and mental retardation.

    00:38 Also, it's associated with the Denys'Drash syndrome which is pseudohermaphroditism and degenerative renal disease and the WT1 and WT2 gene mutations which are vital to kidney development and this represents about 20% of cases.

    00:56 So, to diagnose the Wilms tumor, we'll do a good history, a physical exam, and get some imaging.

    01:02 From a historical perspective, there's generally a history of a painless abdominal mass.

    01:08 It doesn't usually hurt.

    01:09 Patients may have nausea or vomiting.

    01:11 They may experience weight loss.

    01:13 They may have lethargy and fatigue.

    01:15 They may notice abdominal pain or distension but it'll be non-specific, not on the mass, and what's key is they'll often have gross hematuria.

    01:25 So, a child with gross hematuria at the age of 2 to 3, that's something we're worried about.

    01:30 Next, we'll do a physical exam.

    01:32 So, this should be a painless, palpable, firm, fixed abdominal mass.

    01:37 It is a friable tumor however, so you should be cautious when palpating the abdomen if you know this patient has a Wilms or if you suspect it might be a Wilms as with rough palpation, the tumor may rupture.

    01:50 The patient may also have hypertension as associated with this disease.

    01:56 They may have pallor or they may have a varicocele or an inguinal hernia.

    02:02 Additionally, they may have abdominal vein distension and you may see potential stigmata of those other genetic syndromes we mentioned before.

    02:11 Next, if we suspect the disease, we'll do some diagnostic imaging.

    02:17 First, regarding lab findings, we'll often get a CBC with differential.

    02:22 What's unique about this tumor is patients may present with polycythemia from increased epo expression from the tumor.

    02:31 Patients will also have hematuria, so a urinalysis is indicated, and then we'll generally do a biopsy to confirm the diagnosis.

    02:39 We would usually image them prior to biopsying to verify the presence of a tumor and typically what we'll see is something like this, which is a large tumor showing characteristic mixed tissue densities, cystic and solid, and arising from the kidney and enveloping a remnant of normal renal tissue.

    02:58 So, it's generally found on imaging though with a renal ultrasound.

    03:05 That's usually our first choice for how we image these patients and then, we'll get a CT scan of the chest, abdomen and pelvis for staging purposes and for further characterizing the lesion, although ultrasound is usually how we first make the diagnosis.

    03:20 Then, we will provide multimodal therapy depending on the stage and histology of the tumor.

    03:27 So we will usually surgically resect these, we may do radiation, and we may also do chemotherapy.

    03:35 Generally, the prognosis for this disease is very good depending on stage of disease.

    03:41 So, for stage I disease, event-free survival is upwards of more than 95%.

    03:48 It has a great prognosis.

    03:49 Stage II or III, 80 to 95%, and even stage IV disease has a 75% event-free survival.

    03:57 So that's my review of Wilms tumor in children. Thanks.

    About the Lecture

    The lecture Wilms Tumor by Brian Alverson, MD is from the course Pediatric Oncology. It contains the following chapters:

    • Wilms Tumor
    • Physical Exam Findings
    • Multimodal Therapy

    Included Quiz Questions

    1. CHARGE syndrome
    2. Beckwith-Wiedemann syndrome
    3. WAGR syndrome
    4. Denys-Drash syndrome
    5. WT1 WT2 gene mutations
    1. It does not metastasize.
    2. Median age at diagnosis is about 3 years.
    3. It commonly presents as an asymptomatic abdominal mass.
    4. Patients may have polycythemia.
    5. Patients may have gross hematuria.
    1. Biopsy or surgical resection of the lesion
    2. Bilateral renal ultrasound
    3. Renal CT scan without contrast
    4. Renal CT scan with contrast
    5. Magnetic resonance imaging

    Author of lecture Wilms Tumor

     Brian Alverson, MD

    Brian Alverson, MD

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    Excellent lecture
    By Jalil Z. on 25. August 2020 for Wilms Tumor

    Excellent lecture as usual. Very easy to understand and remember. Thanks a lot!