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Max Wilms, MD

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 Background

90%
80%
70%
60%
50% Survival Rates in
40% Children with Wilm's
30% Tumor
20%
10%
0%
at present
5o years ago
(Coppes & Pritchard-Jones, 2000)
Frequency

(ACS, 2010)
 Facts
In 7% of children with Wilms tumor, both kidneys are
involved at diagnosis.

In only 1% of the children who have a kidney removed due

to Wilms tumor, does the cancer re-appear later in the other
kidney.

Possible causable factors in the environment of parents or

their child that might lead to Wilms tumor have not been
determined.

There is a family history of the disease in only 1% of cases.

(NWTSG)
 Race

10 cases 6-9 cases 3-4 cases

per million per million per million

(Breslow, Olshan, Beckwith & Green, 1993)

 Gender

:
0.6 1
(Breslow, Olshan, Beckwith & Green, 1993)
Age

(Breslow, Olshan, Beckwith & Green, 1993)

Associated
Signs & Overgrowth
Abnormalities
Syndromes

Beckwith-
hemihypertrophy Wiedemann
Syndrome
Omphalocele
The WAGR
Syndrome

Wilms Tumor

Aniridia

Genito-urinary
Malformations

Mental
Retardation
Clinical Features
Abdominal mass 80%

Abdominal pain or Hematuria 25%

UTI and varicocele are less common

findings

Hypertension, gross hematuria, and

fever 5-30%
 Diagnoses
Laboratory Studies

Imaging Studies

Histological Findings

Staging
 Diagnoses
Laboratory Studies

Chemistry Cytogenetics
profile Coagulation studies
CBC count Urinalysis
studies
(KFT & LFT) (WT1 gene)
 Diagnoses

Imaging Studies

Renal
CT scanning MRI scanning
ultrasonography
 Diagnoses

Histological Findings
Nephrogenic rests
(NRs)

believed to represent
precursor lesions.
 Staging
Stage I.
The tumor is limited to the kidney, is encapsulated, and can
be completely removed surgically.
Stage II.
The tumor extends beyond the kidney but is completely
removed.
Stage III.
The tumor is not completely removed surgically, but
disease is still limited to the abdomen.
Stage IV.
The disease has spread to the lung, liver, bone, brain, or to
lymph nodes outside the pelvic region.
Stage V.
Both kidneys contain tumor at diagnosis.
(NWTSG)
 Treatment & Medication
Table 1. Current Approach to Favorable Histology Wilms Tumo by Stage

Stage and Histology Surgery Chemotherapy Radiation Therapy*

Stage I or II favorable
histology without LOH 1p Nephrectomy Vincristine, dactinomycin No
and 16q

Stage I or II favorable
Vincristine, dactinomycin,
histology with LOH 1p and Nephrectomy No
doxorubicin
16q

Stage III and IV favorable

Vincristine, dactinomycin,
histology without LOH 1p Nephrectomy Yes
doxorubicin
and 16q

Vincristine, dactinomycin,
Stage III and IV favorable
doxorubicin,
histology with LOH 1p and Nephrectomy Yes
cyclophosphamide,
16q
etoposide

(Dome, et al 2006)
 References
1. Coppes MJ, Pritchard-Jones K. Principles of Wilms' tumor biology. Urol
Clin North Am. Aug 2000;27(3):423-33, viii.

2. Breslow N, Olshan A, Beckwith JB, Green DM. Epidemiology of Wilms

tumor. Med Pediatr Oncol. 1993;21(3):172-81

3. Dome JS, Cotton CA, Perlman EJ, et al. Treatment of anaplastic

histology Wilms' tumor: results from the fifth National Wilms' Tumor
Study. J Clin Oncol. May 20 2006;24(15):2352-8.

4. Coppes MJ, Arnold M, Beckwith JB, et al. Factors affecting the risk of
contralateral Wilms tumor development: a report from the National
Wilms Tumor Study Group. Cancer. Apr 1 1999;85(7):1616-25.