Wilms Tumor
Wilms Tumor: Excerpt from The 5-Minute Pediatric Consult
David T. Teachey, MDTammy I. Kang, MD (4th Edition)
Wilms Tumor - BASICS
Wilms Tumor - description
Wilms tumor is a malignant tumor of the kidney occurring in the pediatric age group. It is also called nephroblastoma.
Wilms Tumor - epidemiology
More common in girls than boys
Wilms Tumor - incidence
- 1 in 10,000 live births
- Increased incidence in children with neurofibromatosis
Wilms Tumor - prevalence
- Most common primary malignant renal tumor of childhood
- 5–6% of all childhood cancer
Wilms Tumor - risk factors
Wilms Tumor - genetics
- 15–20% are hereditary
- Familial cases are more often bilateral and occur at an earlier age.
- A tumor-suppressor gene related to Wilms tumor (WT1) has been localized to chromosome 11p13.
- Mutations of this gene occur in ~20% of Wilms tumors.
- Heterozygous deletions on 11p13 are associated with congenital Wilms tumor/aniridia/genital anomaly/mental retardation syndrome.
- Another candidate tumor suppressor gene WT2 has been localized to 11p15.
Wilms Tumor - etiology
- 20% of Wilms tumors have a mutation in the WT1 tumor suppressor gene.
- Causes in the remaining 80% of patients are unknown.
Wilms Tumor - associated conditions
- May be associated with aniridia, hemihypertrophy, and cryptorchidism
- Associated syndromes: WAGR (Wilms tumor, aniridia, genitourinary [GU] abnormalities, mental retardation), Beckwith–Wiedemann syndrome (macroglossia, omphalocele, visceromegaly, hemihypertrophy), and Denys–Drash syndrome (ambiguous genitalia, progressive renal failure, and increased risk of Wilms tumor)
Wilms Tumor - DIAGNOSIS
Wilms Tumor - signs & symptoms
Wilms Tumor - history
- Abdominal distention
- Abdominal pain (20–30% of cases)
- Hematuria (20–30% of cases)
- Fever, anorexia, vomiting
- Family history of Wilms tumor
- Rapid increase in abdominal size (suggestive of hemorrhage in the tumor)
Wilms Tumor - physical exam
- Asymptomatic abdominal mass extending from flank toward midline (most common presentation)
- Anemia (secondary to hemorrhage in the tumor)
- Fever
- Hypertension (owing to increased renin production in 25% of cases)
- Varicocele (indicates obstruction to spermatic vein owing to tumor thrombus in renal vein or inferior vena cava)
- Aniridia, hemihypertrophy, cryptorchidism, hypospadias
- Signs of Beckwith–Wiedemann and neurofibromatosis
Wilms Tumor - tests
Wilms Tumor - lab
- CBC
- Electrolytes
- Urine analysis: For microscopic hematuria
- Liver and kidney function tests
Wilms Tumor - imaging
- Ultrasound of abdomen:
- Diagnostic of mass of renal origin
- Evaluate for extension of tumor into inferior vena cava.
- CT scan of abdomen: Useful if diagnosis in doubt
- Chest radiograph and chest CT: To evaluate for metastatic disease
- Bone scan: Only if clear cell sarcoma variety on pathology
- MRI of head: Only for clear cell sarcoma and rhabdoid tumors
Wilms Tumor - pathological findings
- Gross pathology:
- Often cystic with hemorrhages and necrosis
- Usually no calcification (useful in differentiating from neuroblastoma, which is calcified on plain radiograph)
- May extend into the inferior vena cava
- Histology:
- Triphasic pattern blastemal, epithelial, and stromal cell
- Blastemal cells aggregate into nodules like primitive glomeruli; the presence of diffuse anaplasia indicates a poor prognosis.
- Clinicopathologic staging:
- Stage I: Tumor is restricted to one kidney and completely resected. The renal capsule is intact.
- Stage II: Tumor extends beyond the kidney, but is completely excised.
- Stage III: Residual nonhematogenous tumor is confined to the abdomen.
- Stage IV: There is hematogenous spread to lungs, liver, bone, or brain.
- Stage V: bilateral disease
Wilms Tumor - differencial diagnosis
- Polycystic kidney
- Renal hematoma
- Renal abscess
- Neuroblastoma
- Other neoplasms of kidney: Clear-cell carcinoma, rhabdoid tumor
Rarely, Wilms tumor may present with polycythemia. It can present as fever of unknown origin without any other signs or symptoms.
Wilms Tumor - TREATMENT
Wilms Tumor - special therapy
Wilms Tumor - radiotherapy
- Not required for stage I and II patients
- Local radiotherapy with 1,000 cGy for stages III and IV
- Whole-lung radiation (1,200 cGy) for pulmonary metastasis
Wilms Tumor - medication
- Chemotherapy:
- For stages I and II favorable histology: Vincristine and actinomycin D every 3 weeks for 6 months
- For stages III and IV favorable histology: Vincristine, actinomycin D, and doxorubicin for 6–15 months
- Add cyclophosphamide and/or etoposide for higher-stage anaplastic tumors.
- Side effects of therapy:
- Temporary loss of hair
- Peripheral neuropathy
- Impaired function of the remaining kidney over years following radiation
- Cardiac toxicity with doxorubicin
- Second malignant neoplasms in few cases
Wilms Tumor - surgery
- Nephrectomy:
- Preoperative chemotherapy in case of very large tumors with inferior vena cava extension
- For bilateral disease, nephrectomy of more affected side and partial nephrectomy of the other side, followed by chemotherapy and radiation
Wilms Tumor - FOLLOW UP
Wilms Tumor - prognosis
- Stages I and II: >90% cured
- Stage III: 85% cured
- Stage IV: 70% cured
- Favorable prognostic factors:
- Tumor weight <250 g
- Age at presentation <24 months
- Stage I disease
- Favorable histology
- Poor prognostic factors:
- Diffuse anaplastic pathology
- Clear cell sarcoma variant
- Rhabdoid tumor variant
- Lymph node involvement
- Distant metastasis
- Tumors with loss of heterozygosity (LOH) of chromosomes 1p and/or 16q
Wilms Tumor - complications
- Extension into inferior vena cava
- Metastasis to lungs and liver
- Cardiac toxicity secondary to doxorubicin
- Liver dysfunction secondary to actinomycin D and radiation therapy
Wilms Tumor - patient monitoring
- Every 3 months for 18 months, every 6 months for 1 year, and then yearly
- Chest radiograph, urinalysis, and abdominal ultrasound at regular intervals
Wilms Tumor - bibliography
- Blakely ML, Ritchey ML. Controversies in the management of Wilms’ tumor. Semin Pediatr Surg. 2001;10:127–131.
- Coppes MJ, Haber DA, Grundy PE. Genetic events in development of Wilms tumor. N Engl J Med. 1994;331:586–590.
- Hohenstein P, Hastie ND. The many facets of the Wilms’ tumor gene (WT1). Hum Mol Genet. 2006;15 Spec No2;R196–R201.
- Kalapurakal JA, Dome JS, Perlmon EJ, et al. Management of Wilm’s tumour: Current practice and future goals. Lancet Oncol. 2004;5:37–42.
- Metzger ML, Dome JS. Current therapy for Wilms’ tumor. Oncologist. 2005;10:815–826.
- Neville HL, Ritchey ML. Wilms’ tumor. Overview of National Wilms’ Tumor Study Group results. Urol Clin North Am. 2000;27:435–442.
- Petruzzi MJ, Green DM. Wilms tumor. Pediatr Clin North Am. 1997;44:939–952.
Wilms Tumor - CODES
Wilms Tumor - icd9
189.0 Wilms’ tumor or neoplasm (nephroblastoma)
Wilms Tumor - FAQ
- Q: What should be done to protect the remaining kidney during sports?
- A: Children should wear a kidney guard to protect the unaffected kidney during contact sports.
- Q: Can a child grow and live normally with 1 kidney?
- A: Yes.
>>
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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