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Wilms Tumor (Nephroblastoma)

Most common pediatric kidney cancer arising from embryonic renal tissue.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Onkoloji department. Book Appointment →

What is Wilms Tumor (Nephroblastoma)?

Wilms tumor (nephroblastoma) is the most common renal malignancy of childhood, accounting for around 6% of pediatric cancers. Median age at diagnosis is 3-4 years, with 75% diagnosed before age 5. The tumor arises from embryonic nephroblasts. Nephrogenic rests are precursor lesions found in 30-40% of resected tumors.

Histology defines favorable (90%) and unfavorable (10%, anaplastic) types. Anaplastic histology, especially diffuse, has worse prognosis. Bilateral disease occurs in 5-10% and is associated with WT1 mutations and constitutional syndromes. Risk syndromes include WAGR (Wilms, aniridia, GU anomalies, mental retardation; WT1 deletion), Beckwith-Wiedemann (chromosome 11p15 imprinting), Denys-Drash (WT1 missense), Perlman, Sotos, and Bloom syndromes.

Patients typically present with painless abdominal mass, hematuria, hypertension, abdominal pain, or fever. Workup includes abdominal ultrasound, CT or MRI, chest CT (lung is most common metastatic site), and assessment for syndromic features. Two main treatment frameworks exist: COG (Children's Oncology Group, US) starts with primary nephrectomy then adjuvant chemo/RT; SIOP (Europe) gives preoperative chemotherapy followed by nephrectomy. Both achieve over 90% overall survival in favorable histology localized disease.

Symptoms

Painless abdominal mass (most common)
Abdominal pain
Hematuria (microscopic or gross)
Hypertension
Fever
Anemia, fatigue
Anorexia, weight loss
Vomiting, abdominal distention
Varicocele in boys (renal vein extension)
Acute hemorrhage from tumor rupture (rare)
Lung metastasis: cough, dyspnea
Aniridia (WAGR syndrome)
Hemihypertrophy (Beckwith-Wiedemann)
Genitourinary anomalies (WT1 syndromes)
Macroglossia, omphalocele (Beckwith-Wiedemann)
Acquired von Willebrand disease (rare paraneoplastic)

Risk Factors

Age 1-5 years
WAGR syndrome (WT1 deletion)
Beckwith-Wiedemann syndrome
Denys-Drash syndrome
Perlman syndrome
Sotos syndrome
Bloom syndrome
Aniridia
Hemihypertrophy
GU anomalies (cryptorchidism, hypospadias)
Family history of Wilms tumor (rare)
Nephrogenic rests on imaging
Female sex (slight predominance)
African ancestry (slightly higher incidence)
Bilateral disease (younger age, syndromic)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Abdominal mass in young child
  • Hematuria in child
  • New hypertension in young child
  • Abdominal pain with fever
  • Aniridia or hemihypertrophy at birth
  • Family history of Wilms tumor
  • Beckwith-Wiedemann syndrome surveillance
  • Acute abdominal symptoms in child with abdominal mass

Treatment Methods

01
Pediatric oncology and surgical oncology referral
02
Abdominal ultrasound (initial), CT or MRI of abdomen
03
Chest CT for pulmonary metastasis
04
Echocardiogram baseline before doxorubicin
05
Genetic testing in syndromic features and bilateral disease
06
Avoid biopsy when possible (pre-treatment biopsy upstages COG patients)
07
Surgery: radical nephrectomy with lymph node sampling (COG)
08
Nephron-sparing surgery for bilateral disease or syndromic predisposition
09
Preoperative chemotherapy (vincristine + actinomycin-D ± doxorubicin) per SIOP for selected cases over 6 months
10
Risk stratification by stage, histology, age, weight, LOH 1p/16q, response
11
Adjuvant chemotherapy: stage I FH (vincristine + actinomycin-D), stage II-III/IV (add doxorubicin), anaplastic (intensified regimens)
12
Flank radiotherapy for stage III, anaplastic, bilateral, with intra-abdominal spillage
13
Whole-lung radiotherapy for pulmonary metastasis (with FH may avoid in good chemo response)
14
Bilateral disease: bilateral nephron-sparing surgery after preoperative chemo
15
Recurrent disease: salvage chemotherapy, autologous stem cell rescue, irinotecan-cyclophosphamide regimens
16
Manage nephrotic syndrome in Denys-Drash with bilateral nephrectomy and dialysis-transplant
17
Surveillance ultrasound every 3 months for 3 years in syndromic predisposition
18
Monitor cardiotoxicity (doxorubicin), renal function, growth, fertility
19
Long-term echocardiogram and screening for second cancers
20
Genetic counseling for syndromic patients
21
Beckwith-Wiedemann: ultrasound every 3 months until age 8
22
Psychosocial support, school accommodations
23
Nutrition, growth, endocrine surveillance
24
Survivorship clinic for late effects

Which Department to Visit?

You can visit our Onkoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.