WILMS TUMOR
PRAKHAR
218
INTRODUCTION
Wilms Tumor also known as Nephroblastoma, is the
most common solid renal Tumor of childhood,
accounting for roughly 5% of childhood cancer.
It arises from embryonic connective tissues
containing epithelial and connective tissue
elements.
It is located in one of the poles of the kidney.
It is bilateral in 5% cases.
It is common in first 4 years of life.
ETIOPATHOGENESIS
The etiopathogenesis of Wilms Tumor involves genetic and
environmental factors
1. GENETIC FACTORS:-
Wilms Tumor associated with mutation in specific gene such as
WT1 gene on chromosome 11p13 and the WT2 gene on
chromosome 11p15.
These mutation can disrupt normal kidney development and lead
to formation of Tumor.
Children with some genetic syndromes are predisposed to Wilms
Tumor these include WAGR( Wilms Tumor, aniridia, genitourinary
abnormalities and mental retardation), Beckwith-Weidmann
syndrome and Denys- Drash syndrome are linked to an increased
risk of developing Wilms Tumor.
ETIOPATHOGENESIS
1.
2. ENVIRONMENTAL FACTORS:-
Exposure to certain prenatal factors, such as
maternal smoking during pregnancy or
maternal use of curtain medication, may
increase the risk of Wilms Tumor in children.
Radiation exposure either in utero or
postnatally, is a non risk factor for the
development of Wilms Tumor.
CLINICAL FEATURES
1. Abdominal mass : is large, firm, unilateral(most
common presentation-80%), smooth, typically
doesn’t cross the midline
2. Hypertension(25%)
3. Hematuria (10-25%)
4. Abdominal pain (30%)
5. Fever (20%)
6. Anorexia and vomiting
COMPLICATIONS
1. Spread of tumor to the lungs, lymph nodes, liver, bones
or brain.
2. High blood pressure & kidney damage as the result of
the Tumor or its treatment.
3. Heart failure
4. Lungs problem
5. Kidney failure
6. Bowel obstruction
STAGING OF WILMS TUMOR
1. Disease confined to kidney & completely resectable
2. Tumor extends beyond kidney,but can be excised
completely
3. Residual disease after resection with positive lymph
node
4. Blood-borne metastasis
5. Bilateral disease
INVESTIGATIONS
1. Abdominal ultrasound
2. CT scan
3. Chest X ray
4. Bone scan, skeletal survey
5. Brain imaging ( MRI, CT scan )
6. Fine Needle aspiration cytology
MANAGEMENT
A. CHEMOTHERAPY :
Favorble histology Tumor undergo surgical resection and have
adjuvant chemotherapy with vincristine and dactinomycin
combination.
Patients with unfavourable Tumor are receiving vincristine,
doxorubicin, cyclophosphamide and ectoposide .
A. SURGICAL MEASURES :
For the patient with unilateral kidney involvement whose
Tumors are Deemed surgically resectable( tumors not crossing
the midline or involving adjacent visceral organs) radical
nephrectomy via a transabdominal inscion is the procedure of
choice.
1.
2.
MANAGEMENT
RADIATION THERAPY :
3.
Post operative radiation is recommended for patients
with stage 3 or 4 disease with favourable histology,
stage 2-4 with focal anaplasia and clear cell carcinoma
& all stages of rhabdoid Tumor.
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