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ADULT WILMS TUMOUR

By
Dr Purav B. Goel
introduction

Wilms tumour, named after the German surgeon Dr.
Carl Max Wilhelm Wilms , is also referred to as
nephroblastoma.

The tumour probably develops from primitive
metanephric blastemal remnants of kidney.
IntroductIon cont


a disease of young children accounting for
approximately 8% of all childhood malignancies
most common abdominal tumour as well as the most
common renal tumour of childhood(3&4)
It is rare in adults(<0.5% of all renal neoplasmsAWT7)
less than 1% over 15 years of age
exact number is unknown as a large number are either
insufficiently documented or incorrectly diagnosed(5).
histopathology
three phase embryonic renal tumors made up of
varying properties of blastemic, epithelial and
mesenchymal structures
The histological appearance is characterized by
marked structural diversity.


Clinical features
>15 years
Flank pain and haematuria
Abdominal palpation
No difference was found between the left and right
sides in frequency of tumor.(AWT5)
Incidental finding on USG/CT scan.
Compared to their pediatric counterparts, adult
patients have more aggressive clinical course & present
more frequently with advanced clinical stages
(metastases: 29% in adult Wilms tumor versus 10% in
childhood Wilms tumor) [7].

diagnosis
The presence of a large, rapidly growing abdominal
mass in an otherwise healthy young patient that shows
a complex mass on sonography and as an
inhomogeneous mass with variable enhancement and
pseudocapsule on CT
Hypovascularity of the mass on arteriography with a
few zigzag lines of neovascularity extending to the
mass is also characteristic(3).
The histopathological study confirms the diagnosis.
Diagnosis
Adult Wilms' tumour is diagnosed based on criteria given
by Kilton, Mathews and Cohen. These include

(a) the tumour under consideration should be a primary
renal neoplasm;

(b) presence of primitive blastemic spindle or round cell
component;

(c) formation of abortive or embryonal tubules or
glomerular structures;
dIagnosIs cont
(d) no area of tumour diagnostic of renal cell
carcinoma;

(e) pictorial : confirmation of histology and

(f ) patient's age > 15 years.

In 1980 they reported a series of 35 cases of adult
Wilms tumour with all above said criteria.

dIagnosIs cont
various undifferentiated tumors must be considered in
the differential diagnoses especially when the tumor is
predominantly monophasic [8]

treatment
lack of a defined therapeutic protocol [9]
The current treatment regime is modeled on the
pediatric regimen recommended by the National
Wilms Tumor Study Group. This consists of radical
nephrectomy and adjuvant chemotherapy with or
without radiotherapy, depending on the disease stage
[10].
little information is available with respect to
alternative treatment for adults where initial
chemotherapy fails or the disease recurs
treatment cont
little information is available with respect to
alternative treatment for adults where initial
chemotherapy fails or the disease recurs
Unlike in children,the issue of infertility associated
with radiotherapy and subsequent compliance further
complicates management
ongoing debate with respect to the dose of
radiotherapy in adult protocols and a clinical trial
(SIOPWT) is looking at the possibility of avoiding
both anthracyclines (which are cardiotoxic) and
radiotherapy [4].

treatment cont
various new modalities such as recombinant interferon
alpha for the treatment of recurrent AWT are being
explored
prognosis
Poorer stage for stage prognosis
However since 1979, imrovement in response and
survival.
Calcification may be a sign of slow tumour growth and
possibly a favourable prognosis in cases of adult
Wilms tumour
Discussion
National Wilms Tumour Study (NWTS) was established in
USA in 1969 .

An update from NWTS group about treatment outcomes
in adults with favourable histology Wilms tumour (FHWT)
described 45 patients treated in the modern era.

The overall survival rate was 82 % .

NWTS and other studies have recommended multimodal
therapy for the disease with surgery, chemotherapy for 15
months and tumour bed irradiation in the case of stage 3
disease
Conclusion

To conclude in an adult patient presenting with flank
pain and a renal mass on imaging, though rare,
possibility of Wilms tumour should also be
considered.

Although the prognosis in adult Wilms tumour is
poorer as compared to that in children, the outcome
for adult patients is improving steadily with
multimodal therapy.
PET scanning was used to diagnose early
relapse.(AWT2)

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