History and Physical Examination
History
The most common manifestation of Wilms tumor is an asymptomatic abdominal mass; an abdominal mass occurs in 80% of children at presentation. Abdominal pain or hematuria occurs in 25%. Urinary tract infection and varicocele are less common findings than these. Hypertension, gross hematuria, and fever are observed in 5-30% of patients. A few patients with hemorrhage into their tumor may present with hypotension, anemia, and fever. Rare patients with advanced disease may present with respiratory symptoms related to lung metastases.
Physical examination
Examination often reveals a palpable abdominal mass. Pay special attention to features of those syndromes (WAGR syndrome and Beckwith-Wiedemann syndrome [BWS]) associated with Wilms tumor (ie, aniridia, genitourinary malformations, and signs of overgrowth).
The abdominal mass should be carefully examined. Palpating a mass too vigorously could lead to the rupture of a large tumor into the peritoneal cavity.
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CT scan in a patient with a right-sided Wilms tumor with favorable histology.
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CT scan of child with a stage IV Wilms tumor with favorable histology. Note the bilateral pulmonary metastases.
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Gross nephrectomy specimen shows a Wilms tumor pushing the normal renal parenchyma to the side.
Tables
Stage |
Relapse-Free Survival, % |
Overall Survival, % |
I |
92 |
98 |
II |
85 |
96 |
III |
90 |
95 |
IV |
80 |
90 |
Stage and Histology |
Surgery |
Chemotherapy |
Radiation Therapy* |
Stage I or II favorable histology without loss of heterozygosity (LOH) 1p and 16q† |
Nephrectomy |
Vincristine, dactinomycin |
No |
Stage I or II favorable histology with LOH 1p and 16q |
Nephrectomy |
Vincristine, dactinomycin, doxorubicin |
No |
Stage III and IV favorable histology without LOH 1p and 16q |
Nephrectomy |
Vincristine, dactinomycin, doxorubicin |
Yes |
Stage III and IV favorable histology with LOH 1p and 16q |
Nephrectomy |
Vincristine, dactinomycin, doxorubicin, cyclophosphamide, etoposide |
Yes |
* The current dose for radiation therapy for favorable histology Wilms tumor is approximately 1080 cGy for the abdomen and 1200 cGy for the lung. [29] Postoperative radiotherapy is started within 14 days of nephrectomy. [30] Patients with stage IV favorable histology Wilms tumor and lung metastases whose pulmonary lesions do not disappear after 6 weeks of chemotherapy receive whole-lung radiation therapy. † Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone. [31] Children younger than 24 months with small (< 550 g) Wilms tumors with favorable histology are noted in the current COG protocol. |
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Stage and Type of Wilms Tumor |
Imaging Studies |
Off-Treatment Schedule |
Stages I, II, and III with favorable histology; stages I, II, and III with anaplastic histology |
Chest radiography |
6 wk and 3 mo after surgery, then every 3 mo (5 times), then every 6 mo (3 times), then yearly (2 times) |
All stages in patients aged < 48 mo at diagnosis with nephrogenic rests |
Abdominal ultrasonography |
Every 3 mo for 6 y |
All stages in patients aged >48 mo at diagnosis with nephrogenic rests |
Abdominal ultrasonography |
Every 3 mo for 4 y |
Stages I and II with favorable histology |
Abdominal ultrasonography |
Yearly (6 times) |
Stage III with favorable histology |
Abdominal ultrasonography |
6 wk and 3 mo after surgery, then every 3 mo (5 times), then every 6 mo (3 times), then yearly (2 times) |
All stages with unfavorable histology |
Abdominal ultrasonography |
Every 3 mo (4 times), then every 6 mo (4 times) |
* Subsequent imaging studies should be performed as clinically indicated. |
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