Wilms’ TumorNephroblastoma

AbdulQadir J. Nashwan

Road Map

Max Wilms, MD

To know more about Max Wilms

Background

Wilms’ tumor, or nephroblastoma, is the most common childhood abdominal malignancy

at present5o years ago

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

Survival Rates in Children with Wilm's Tumor

(Coppes & Pritchard-Jones, 2000)

Frequency

• Wilms’ tumor affects approximately 10 children and

adolescents per 1 million before the age of 15 years.

• Accounts for 6-7% of all childhood cancers in North America.

• About 450-500 new cases are diagnosed each year

(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

6-9 cases per million

3-4 cases per million

10 cases per million

(Breslow, Olshan, Beckwith & Green, 1993)

Gender

10.6

:

(Breslow, Olshan, Beckwith & Green, 1993)

Age

The median age at diagnosis is

approximately 3.5 years

(Breslow, Olshan, Beckwith & Green, 1993)

Wilms’ Tumor Experience at King Hussein Medical Center

Saudi Journal of Kidney Disease and Transplantation

2000

Isam Haddadin, Pediatric Oncologist

Issa Hazza, Pediatric Nephrologist

Overgrowth Abnormalities

hemihypertrophyBeckwith-

Wiedemann Syndrome

Omphalocele

Associated Signs & Syndromes

The WAGR Syndrome

Wilms’ Tumor

Aniridia

Genito-urinary Malformations

Mental Retardation

Wilms’ Tumor

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

CBC countChemistry

profile

(KFT & LFT)Urinalysis Coagulation

studies

Cytogenetics studies

(WT1 gene)

Diagnoses

Imaging Studies

Renal ultrasonography CT scanning MRI scanning

Diagnoses

Histological Findings

Nephrogenic rests (NRs)

believed to represent precursor lesions.

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.

Staging

(NWTSG)

Treatment & MedicationStage and Histology Surgery Chemotherapy Radiation Therapy*

Stage I or II favorable histology without LOH 1p

and 16q Nephrectomy Vincristine, dactinomycin No

Stage I or II favorable histology with LOH 1p and

16qNephrectomy

Vincristine, dactinomycin, doxorubicin

No

Stage III and IV favorable histology without LOH 1p

and 16qNephrectomy

Vincristine, dactinomycin, doxorubicin

Yes

Stage III and IV favorable histology with LOH 1p and

16qNephrectomy

Vincristine, dactinomycin, doxorubicin,

cyclophosphamide, etoposide

Yes

(Dome, et al 2006)

Table 1. Current Approach to Favorable Histology Wilms Tumo by Stage

References1. 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.