Wilms Tumor in WAGR Syndrome
WILMS TUMOR
IN
WAGR SYNDROME
What Is WAGR syndrome?
WAGR
syndrome is a rare genetic condition which
includes
•
W
ilms Tumor
•
A
niridia
•
G
enital and urinary tract abnormalities
•
R
ange of developmental delays
WILMS TUMOR IN WAGR SYNDROME
•
A form of kidney cancer
•
Occurs mostly in children
•
Also called “nephroblastoma”
What is Wilms tumor?
•
Typical child
1 in 10,000
•
Child with W
AGR syndrome
1 in 2
WILMS TUMOR IN WAGR SYNDROME
Who is at risk for Wilms tumor?
•
Immature cells in the kidney called
nephrogenic rests
•
These cells are more common in children with
genetic disorders like WAGR syndrome
What causes Wilms tumor?
WILMS TUMOR IN WAGR SYNDROME
WILMS TUMOR IN WAGR SYNDROME
•
Immature cells in the kidney
•
Have the potential to develop into Wilms
tumor
•
Clusters of these cells may look like Wilms
tumor on ultrasound
What are nephrogenic rests?
•
Important for kidney
development before birth and
function throughout life
•
Mutation or deletion of this
gene increases the risk for
Wilms tumor
WILMS TUMOR IN WAGR SYNDROME
WT1: the Wilms tumor gene
Chromosome 11
WT1
gene
●
Ultrasound imaging every 3 months beginning at
birth/diagnosis of WAGR syndrome
●
If a mass is detected, additional imaging such as MRI or CT
may be needed
●
Definitive diagnosis is made by examining the cells under a
microscope
WILMS TUMOR IN WAGR SYNDROME
H
o
w
i
s
W
i
l
m
s
t
u
m
o
r
d
i
a
g
n
o
s
e
d
?
Nephrogenic rests
•
Are very common in children with WAGR syndrome
•
Are benign (not cancer)
•
May or may not progress to Wilms tumor
•
May be present at the same time as Wilms tumor
•
Often hard to tell the difference on ultrasound or MRI
•
May require same treatment as Wilms tumor
WILMS TUMOR IN WAGR SYNDROME
N
e
p
h
r
o
g
e
n
i
c
r
e
s
t
o
r
W
i
l
m
s
t
u
m
o
r
?
Treatment is tailored to the individual and may include
•
Surgery
•
Chemotherapy
•
Radiation therapy
WILMS TUMOR IN WAGR SYNDROME
Treatment
WILMS TUMOR IN WAGR SYNDROME
Types of Surgery
•
Radical nephrectomy
Tumor, kidney, and ureter are removed
•
Partial nephrectomy
Only the tumor is removed
•
Biopsy
A tumor sample is removed to examine the cells under a microscope
Chemotherapy may be given
•
Before surgery
•
After surgery
•
Before and after surgery
A combination of drugs may be used, including
•
Actinomycin D (dactinomycin)
•
Vincristine
•
Adriamycin (doxorubicin)
WILMS TUMOR IN WAGR SYNDROME
Chemotherapy
•
Long-term survival rates are high
•
Surveillance for Wilms tumor should
continue throughout life
•
Guidelines for long-term follow-up care
for childhood cancer survivors:
http://www.survivorshipguidelines.org
WILMS TUMOR IN WAGR SYNDROME
Post-treatment Care
The IWSA is working with researchers around the world
to improve diagnosis and treatment
www.wagr.org
The mission of the International WAGR Syndrome Association is to
promote awareness, stimulate research, and to support families affected
by WAGR syndrome
WILMS TUMOR IN WAGR SYNDROME
WAGR syndrome is a rare genetic condition associated with Wilms Tumor, aniridia, and developmental delays. Wilms Tumor is a form of kidney cancer mostly affecting children and is linked to genetic disorders like WAGR syndrome. The risk of Wilms Tumor is higher in children with WAGR syndrome, with potential causes being immature cells in the kidney known as nephrogenic rests. Diagnosis involves regular ultrasound imaging and examination of cells under a microscope. Nephrogenic rests, while common in WAGR syndrome, may progress to Wilms Tumor and require similar treatment. Understanding the WT1 gene and its role in kidney development is crucial in managing Wilms Tumor in WAGR syndrome.
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Presentation Transcript
WILMS TUMOR IN WAGR SYNDROME
What Is WAGR syndrome? WAGR syndrome is a rare genetic condition which includes Wilms Tumor Aniridia Genital and urinary tract abnormalities Range of developmental delays
What is Wilms tumor? A form of kidney cancer Occurs mostly in children Also called nephroblastoma WILMS TUMOR IN WAGR SYNDROME
Who is at risk for Wilms tumor? Typical child 1 in 10,000 Child with WAGR syndrome 1 in 2 WILMS TUMOR IN WAGR SYNDROME
What causes Wilms tumor? Immature cells in the kidney called nephrogenic rests These cells are more common in children with genetic disorders like WAGR syndrome WILMS TUMOR IN WAGR SYNDROME
What are nephrogenic rests? Immature cells in the kidney Have the potential to develop into Wilms tumor Clusters of these cells may look like Wilms tumor on ultrasound WILMS TUMOR IN WAGR SYNDROME
WT1: the Wilms tumor gene Chromosome 11 Important for kidney development before birth and function throughout life Mutation or deletion of this gene increases the risk for Wilms tumor WT1 gene WILMS TUMOR IN WAGR SYNDROME
How is Wilms tumor diagnosed? Ultrasound imaging every 3 months beginning at birth/diagnosis of WAGR syndrome If a mass is detected, additional imaging such as MRI or CT may be needed Definitive diagnosis is made by examining the cells under a microscope WILMS TUMOR IN WAGR SYNDROME
Nephrogenic rest or Wilms tumor? Nephrogenic rests Are very common in children with WAGR syndrome Are benign (not cancer) May or may not progress to Wilms tumor May be present at the same time as Wilms tumor Often hard to tell the difference on ultrasound or MRI May require same treatment as Wilms tumor WILMS TUMOR IN WAGR SYNDROME
Treatment Treatment is tailored to the individual and may include Surgery Chemotherapy Radiation therapy WILMS TUMOR IN WAGR SYNDROME
Types of Surgery Radical nephrectomy Tumor, kidney, and ureter are removed Partial nephrectomy Only the tumor is removed Biopsy A tumor sample is removed to examine the cells under a microscope WILMS TUMOR IN WAGR SYNDROME
Chemotherapy Chemotherapy may be given Before surgery After surgery Before and after surgery A combination of drugs may be used, including Actinomycin D (dactinomycin) Vincristine Adriamycin (doxorubicin) WILMS TUMOR IN WAGR SYNDROME
Post-treatment Care Long-term survival rates are high Surveillance for Wilms tumor should continue throughout life Guidelines for long-term follow-up care for childhood cancer survivors: http://www.survivorshipguidelines.org WILMS TUMOR IN WAGR SYNDROME
The IWSA is working with researchers around the world to improve diagnosis and treatment www.wagr.org The mission of the International WAGR Syndrome Association is to promote awareness, stimulate research, and to support families affected by WAGR syndrome WILMS TUMOR IN WAGR SYNDROME
