Wilm’ s tumor associated with A/E
Correct Answer: Bilateral polycystic kidney disease
Description: The genitourinary anomalies most commonly associated with Wilms tumor are
hypoplasia,
fusion and ectopia of the kidney,
duplications of the collecting systems,
hypospadias, and
cryptorchidism
(Bilateral polycystic kidney disease is not associated with the Wilm's tumor)
Syndromes Associated with Wilms Tumor
Several syndromes and congenital abnormalities commonly are reported in patients with Wilms tumor.
WAGR syndrome (contiguous gene deletion of WT1 & PAX6 on 11P13)
It consists of
Wilms tumor,
Aniridia,
Genitourinary abnormalities (cryptorchidism, streak ovaries, bicornuate uterus, ambiguous genitalia), and
Mental retardation.
Patients with this syndrome have a constitutional deletion of chromosome 11p13 where the Wilms tumor gene,
WT1, and the aniridia gene, PAX6, are located.
Denys- Drash syndrome(missense mutation in WT1 gene)
It is characterized by
Male pseudohermaphrodism,
Early-onset renal failure characterized by mesangial sclerosis and
Increased risk of Wilms tumor.
Patients with this syndrome typically carry a missense mutation in the WT1 gene.
Beckwith-Wiedemann syndrome (WT2 gene abnormality on chromosome 11P15.5)
It is characterized by
Hemihypertrophy,
Macroglossia,
Visceromegaly,
Omphalocele
Risk of developing Wilms tumor (3-5%)
A variety of 11p15.5 abnormalities have been reported in patients with this syndrome, and it is postulated that a second Wilms tumor gene, WT2, is located in this region. Loss of imprinting of the insulin-like growth factor 2 gene, an epigenetic process, also is associated with Wilms tumor.
Other syndromes or conditions with an increased risk of Wilms tumor include:
Hemihypertrophy,
Sporadic aniridia,
Genitourinary anomalies,
Pearlman syndrome,
Sotos syndrome,
Neurofibromatosis (von Recklinghausen disease), and Von Willebrand disease.
About option 'c' Hypertension.
"Hypertension also has been described and probably is due to renal ischemia."- Nelson
More about Wilm's tumor (also k/a nephroblastoma):
Wilm's tumor is the most common renal neoplasm & 2nd most common malignant abdominal tumor, in children (Most common abdominal malignant tumor is neuroblastoma)
Peak incidence is between 2 and 5 yrs of age.
5-10% are bilateral; either both kidneys are involved simultaneously (synchronous) or one after the other (metachronous)
Clinical features of W.T.
Abdominal lump (most common presenting feature)
Abdominal pain
Vomiting
Hematuria
Hypertension
Treatment:
High cure rates of 80-90% can be achieved with multimodality treatment using surgery, chemotherapy & radiotherapy
Surgical resection is done in all stages followed by chemotherapy (stage I&II) or chemotherapy + radiotherapy (stage III, IV, V)
Pre op chemotherapy is given in inoperable & bilateral cases
Chemotherapeutic agents used are Vincristine, Dactinomycin, Doxorubicin & Cyclophosphamide.
Category:
Surgery
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