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Wilms' Tumour

This is the commonest intra-abdominal tumour of childhood (20% of all childhood malignancies). It is an undifferentiated mesodermal tumour of the intermediate cell mass (primitive renal tubules and mesenchymal cells). It may be sporadic, or familial.

Associations1 Wilms' tumor usually develops in otherwise healthy children, but approximately 10% occur in children with recognized malformations; either:

  • 'Overgrowth syndromes' (excessive prenatal and postnatal somatic growth resulting in macroglossia, nephromegaly, and hemihypertrophy) - most commonly Beckwith-Wiedemann syndrome or isolated hemihypertrophy. Others include Perlman syndrome, Sotos syndrome, and Simpson-Golabi-Behemel syndrome.
  • No 'overgrowth' - associated with with trisomy 18, Bloom syndrome, Denys-Drash syndrome;
    or Wilms' with Aniridia, Gonadoblastoma (GU malformations), and Retardation ('WAGR').

Familial Wilms' Tumour2 Hereditary Wilms' tumour (either bilateral tumours or a family history of the neoplasm) is uncommon. Several different families with Wilms' tumours have been identified. All are transmitted in an autosomal dominant manner, caused by mutations in one of a least 3 genes:

  • One related to the WT1 gene on chromosome 11 (11p13) - (includes those patients with WAGR3) encodes a protein which is a transcriptional repressor downregulating IGF-II, an insulin-like growth factor.4
  • Other families (including those with Beckwith-Wiedemann syndrome) have a different mutation - of the WT2 gene on chromosome 11 (11p15.5)
  • Other gene mutations, thought to be on chromosome 16 (WT3 - 16q) and/or chromosome 1p can also cause the tumour.

Presentation Median age: 3.5yrs. 95% are unilateral. Only 1-2% have a positive family history.
Clinical features: Fever, flank pain, abdominal mass. Haematuria is not common.

Investigations Urine cytology; ultrasound; IVU (may show renal pelvis distortion; hydronephrosis); renal angiography; CT/MRI scan.

Staging

Staging of Wilms' Tumour
Stage I
(43% of patients)		 Tumour limited to the kidney and completely excised. Renal capsule is intact.
The tumour is not ruptured before or during removal.
The vessels of the renal sinus are not involved.
There is no residual tumour apparent beyond the margins of excision.
Stage II
(23% of patients)		 Tumour extends beyond the kidney but is completely excised. No residual tumour is apparent at or beyond the margins of excision.
There may be:
  1. Regional extension of the tumour, i.e., penetration through the outer surface of the renal capsule into the perirenal soft tissue or more than 1 to 2 mm of tumor invasion into the renal sinus.
  2. Vessels outside the kidney are infiltrated or contain tumour thrombus.
  3. The tumour was biopsied or there was local spillage of tumour confined to the flank.
Stage III
(23% of patients)		 There is residual tumour confined to the abdomen. There may be one or more of the following:
  1. Tumour positive lymph nodes in the renal hilus, the periaortic chains, or other intra-abdominal sites on biopsy.
  2. There has been diffuse peritoneal contamination by the tumour, eg. spillage of tumour beyond the flank before or during surgery or by tumour growth penetrating through the peritoneal surface.
  3. Implants are found on the peritoneal surfaces.
  4. Tumour extends beyond the surgical margins either microscopically or grossly.
  5. Tumour is not completely resectable because of local infiltration into vital structures.
Stage IV
(10% of patients)		 Haematogenous metastases - beyond stage III, e.g., to the lung, liver, bone, or brain.
Stage V
(5% of patients)		 Bilateral renal involvement at initial diagnosis. Attempt to stage each side according to the above criteria on the basis of extent of disease prior to biopsy.

The 4-year survival was 94% for those patients whose most advanced lesion was stage I-II; 76% where it was stage III.

Management:5 Avoid renal biopsy. Nephrectomy, chemotherapy (eg vincristine and doxorubicin ±dactinomycin) with radiotherapy can be curative. Prognosis: 90% 5yr survival.

Screening Children with a predisposition to develop Wilms' tumor (e.g., Beckwith-Wiedemann, hemihypertrophy) should be screened with ultrasound every 3 months until they reach 6-8 years of age

References, footnotes and further reading

  1. Clericuzio CL; Clinical phenotypes and Wilms tumor. Medical and Pediatric Oncology 1993 21(3): 182-187.
  2. Online Mendelian Inheritance In Man - Genetic Database (OMIM)
  3. The WAGR syndrome is one of the best-studied 'contiguous gene syndromes' - children with WAGR invariably have a constitutional chromosomal deletion at 11p13, the location of the WT1 gene. Those with Denys-Drash syndrome (DDS) usually have a germline point mutation, which is predicted to result in an amino acid substitution (i.e., missense mutation), in the eighth or ninth exon of the Wilms tumor gene.
  4. Skuse GR, Ludlow JW. Tumour suppressor genes in disease and therapy. Lancet. 1995 Apr 8; 345:902-6.
  5. Cancernet - Wilms' Tumour

Acknowledgements The final copy has passed peer review of the independent Mentor GP authoring team. ©EMIS 2006.

Last issued 30 Aug 2006









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