In this lecture, we will review the Wilms tumor,
which is a renal tumor in children.
This is basically a kidney tumor
arising from pluripotent embryonic renal precursor cells.
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.
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.
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.
So, to diagnose the Wilms tumor,
we'll do a good history, a physical exam, and get some imaging.
From a historical perspective,
there's generally a history of a painless abdominal mass.
It doesn't usually hurt.
Patients may have nausea or vomiting.
They may experience weight loss.
They may have lethargy and fatigue.
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.
So, a child with gross hematuria at the age of 2 to 3,
that's something we're worried about.
Next, we'll do a physical exam.
So, this should be a painless, palpable, firm, fixed abdominal mass.
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.
The patient may also have hypertension as associated with this disease.
They may have pallor or they may have a varicocele or an inguinal hernia.
Additionally, they may have abdominal vein distension
and you may see potential stigmata
of those other genetic syndromes we mentioned before.
Next, if we suspect the disease, we'll do some diagnostic imaging.
First, regarding lab findings, we'll often get a CBC with differential.
What's unique about this tumor is patients may present with polycythemia
from increased epo expression from the tumor.
Patients will also have hematuria, so a urinalysis is indicated,
and then we'll generally do a biopsy to confirm the diagnosis.
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.
So, it's generally found on imaging though with a renal ultrasound.
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.
Then, we will provide multimodal therapy
depending on the stage and histology of the tumor.
So we will usually surgically resect these,
we may do radiation, and we may also do chemotherapy.
Generally, the prognosis for this disease
is very good depending on stage of disease.
So, for stage I disease, event-free survival is upwards of more than 95%.
It has a great prognosis.
Stage II or III, 80 to 95%,
and even stage IV disease has a 75% event-free survival.
So that's my review of Wilms tumor in children. Thanks.