eMedicine Specialties > Pediatrics: General Medicine > Oncology

Wilms Tumor: Follow-up

Author: Arnold C Paulino, MD, Associate Professor, Department of Radiology, Division of Radiation Oncology, Associate Professor of Pediatrics, Baylor College of Medicine; Consulting Staff, Methodist Hospital and Texas Children's Hospital
Coauthor(s): Max J Coppes, MD, PhD, MBA, Senior Vice President, Children's National Medical Center (Center for Cancer and Blood Disorders); Director, Center for Cancer and Immunology Research, Children's Research Institute, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University
Contributor Information and Disclosures

Updated: Mar 3, 2009

Follow-up

Further Inpatient Care

• As many as one third of patients with Wilms tumor present with hypertension. Their blood pressure usually normalizes after nephrectomy, but they occasionally require prolonged therapeutic intervention.
• About 5-10% of patients present with acquired von Willebrand disease at the time of diagnosis. Several hypothesis have been postulated to explain acquired von Willebrand disease, including absorption of the von Willebrand factor (vWF) by tumor cells, hyperviscosity caused by elevated serum levels of hyaluronic acid, and an immunoglobulin G (IgG)–type antibody that prevents aggregation of normal platelet cells (immunologic inactivation).
• If present, excessive bleeding during surgery should be expected and prenephrectomy therapy should be started. Whenever possible, the use of blood derivatives should be avoided because of the potential to transmit viral infections. Instead, an initial trial of desmopressin (DDAVP), a drug that promotes the release of vWF from storage sites, is recommended. DDAVP has been effective in most patients with type I von Willebrand disease and in some with type II disease. If DDAVP is administered, fluid and electrolyte balance should be carefully monitored. If DDAVP is ineffective, cryoprecipitator (a specific vWF concentrate) should be administered.

Further Outpatient Care

• The patient must be examined at the follow-up clinic after he or she completes all therapy. The purpose of follow-up care is to check for recurrence and for late effects of therapy.
• Table 2 outlines the types and frequency of radiographic studies during follow-up according to the NWTSG.¹⁶  For follow-up of late effects and recommended studies, please refer to the Late Effects Guidelines from the Children's Oncology Group.   Table 2. Recommended Follow-Up Imaging Studies in Children with Wilms Tumor Without Metastasis at Diagnosis^*

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  Table

  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)

  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.

Inpatient & Outpatient Medications

• Inpatient and outpatient drugs depend on the patient's specific circumstances.

Complications

• Nephrectomy leaves the child with one functional kidney. In almost all patients, the remaining kidney can compensate and maintain adequate renal function. Additional treatment modalities after nephrectomy may damage several organs, such as the heart, lungs, liver, bones, and gonads.^17,18 In addition, both chemotherapy and radiation therapy can induce second malignant neoplasms.¹⁹
• Specific complications are as follows:
  • Impaired renal function: Children with Wilms tumor have a minimal risk for impaired renal function, primarily related to nephrectomy. In selected patients, (ie, those who receive radiation therapy), function of the remaining kidney can be further endangered. The development of compensatory postnephrectomy hypertrophy of the remaining kidney is well documented in patients with Wilms tumor. NWTSG data suggest that most patients with unilateral Wilms tumor do not develop serious long-term renal complications. By comparison, renal function can be impaired in those with bilateral disease. The most common cause of renal failure in patients with bilateral Wilms tumor is bilateral nephrectomy. Treatment-related injury (eg, radiation-induced damage, surgical complications) of the remaining kidney is the second leading cause of renal insufficiency.
  • Impaired cardiac function: Congestive heart failure is a well-known complication of the administration of anthracyclines. Therefore, patients with Wilms tumor who receive anthracyclines, most commonly doxorubicin, should be monitored for cardiac dysfunction.
  • Impaired pulmonary function: Because radiation therapy can affect pulmonary function, monitoring of pulmonary function is required in patients with metastatic Wilms tumors to the lung who are treated with bilateral pulmonary irradiation. The total lung capacity and vital capacity of patients receiving bilateral irradiation can be expected to decrease by 50-70% of the predicted values.
  • Impaired hepatic function
    • Several cytotoxic agents may damage the liver of patients treated for Wilms tumor, including dactinomycin and irradiation. Most early reports suggest that hepatic irradiation is the major etiologic factor in hepatic injury. However, reports have documented hepatic toxicity with the combination of vincristine and dactinomycin in nonirradiated children with Wilms tumor, suggesting that chemotherapeutic agents themselves can also damage the liver. In the fourth NWTSG report, the incidence of hepatotoxicity was 2.8-14.3% in patients who did not receive irradiation. The fact that patients who received less dactinomycin than others (ie, those with relatively low-stage disease) had a low incidence of 2.8% suggests a dose-related toxicity for dactinomycin.
    • Some patients with Wilms tumor have developed hepatic veno-occlusive disease (VOD). VOD is primarily a clinical diagnosis characterized by hepatomegaly or pain in the right upper quadrant, jaundice, ascites, and unexplained weight gain. The syndrome occurs both in patients with Wilms tumor undergoing nephrectomy first and in those receiving combination chemotherapy before surgery, the standard approach the SIOP recommends. Although treatment for VOD is primarily supportive, the administration of chemotherapeutic agents can be resumed after the signs of VOD have disappeared.
    • With the use of currently accepted radiotherapy techniques, radiation-induced hepatitis is rare in survivors of Wilms tumor.
  • Impaired gonadal function: Women who received whole-abdomen irradiation in childhood can develop ovarian failure. Recent data clearly suggest that a high risk of adverse pregnancy outcomes should be considered in the counseling and prenatal care of women who received abdominal radiation therapy to treat a Wilms tumor. Male patients are at risk for testicular failure after whole-abdomen radiation therapy or certain types of chemotherapy, most notably that involving alkylating agents.
  • Impaired musculoskeletal function: The effect of radiation therapy to the skeletal system is often predictable. Although radiation therapy may affect the growth of any given bone, the spine is most notably affected at doses of 20 Gy. A recent study from the University of Iowa showed a dose-response relationship in the induction of scoliosis and in the dose delivered. Most patients who received doses of more than 24 Gy with megavoltage beams developed asymptomatic scoliosis. Patients receiving current doses of 10-12 Gy may have a much reduced likelihood of developing scoliosis.²⁰
  • Second malignant neoplasms
    • Patients who survive Wilms tumor are at risk because both inherited disposition and treatment (eg, chemotherapy, irradiation) can induce second malignant neoplasms. Most secondary malignant neoplasms reported (eg, bone tumors, breast and thyroid cancers) have occurred in irradiated areas. Nevertheless, certain chemotherapeutic agents, including doxorubicin, dactinomycin, and vincristine, may contribute to an increased risk for secondary malignancies.
    • Fifteen years after initial diagnosis, the cumulative incidence of a secondary malignant neoplasm in patients registered with the NWTSG was 1.6% and increasing. According to NWTSG investigators, abdominal irradiation increases the risk of a secondary malignant neoplasm and doxorubicin potentiates the radiation effect. Treatment for relapse further increased the risk for a secondary malignant neoplasm by a factor of 4-5.
  • Relapses: The lungs are the most common site of relapse. This site is affected in more than two thirds of children who have a relapse. The tumor bed is the site of relapse only in about one fourth of patients. The brain and the bones are not usual sites of relapse for Wilms tumors with favorable histology.

Prognosis

• Approximately 80-90% of children with a diagnosis of Wilms tumor survive with current multimodality therapy.²¹
• Patients who have tumors with favorable histology have an overall survival rate of at least 80% at 4 years after the initial diagnosis, even in patients with stage IV disease.
• The 4-year relapse-free and overall survival rates in patients with favorable-histology Wilms tumor are shown in Table 3. Table 3. Survival Rates in Patients with Favorable-Histology Wilms Tumor

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  Table

  Stage	Relapse-Free Survival, %	Overall Survival, %
  I	92	98
  II	85	96
  III	90	95
  IV	80	90

  Stage	Relapse-Free Survival, %	Overall Survival, %
  I	92	98
  II	85	96
  III	90	95
  IV	80	90

• Patients with synchronous bilateral tumors have a 70-80% survival rate,^22,23 whereas those with metachronous tumors have a 45-50% survival rate.²⁴
• Patients with anaplastic Wilms tumor have a worse prognosis compared with favorable histology Wilms tumor; the 4-year overall survival rates are 83%, 83%, 65% and 33% for stages I, II, III, and IV, respectively.²⁵
• The prognosis for patients who have a relapse is not as good as newly diagnosed Wilms tumor, with 40-80% expected to survive after salvage therapy. Patients who relapse after receiving vincristine and actinomycin D have a better survival with relapse treatment compared with those who initially received vincristine, actinomycin D and doxorubicin.^26,27

Patient Education

• The parents and patient must know that long-term follow-up care is essential because of the late effects of treatment.

Miscellaneous

Medicolegal Pitfalls

• Informed consent is necessary before treatment. Discuss potential late effects of treatment, including, but not limited to, bony and soft-tissue abnormalities, second malignancy, interstitial pneumonitis, veno-oclusive disease (VOD), congestive heart failure, ovarian failure, and bowel obstruction.
• For primary care physicians, the failure to diagnose an abdominal mass in a timely fashion has become a legal issue.

Special Concerns

• Whether patients younger than 6 months who have stage III disease should receive radiation therapy is controversial. In the NWTS-5 protocol, physicians were required to call an NWTS-5 radiation oncologist to discuss guidelines. The late toxicity of irradiating young children has been a concern for many, even with relatively low doses of radiation.
• Female patients have an increased risk of giving birth prematurely. Possible explanations include loss of elasticity of the uterine wall, surgical adhesions, and a high incidence of uterine anomalies.

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