Wilms Tumor

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Wilms tumor, or nephroblastoma, is the most common childhood abdominal malignancy. The median age at diagnosis of Wilms tumor (see the image below) is approximately 3.5 years. With current multimodality therapy, approximately 80-90% of children with a diagnosis of Wilms tumor survive.

See Wilms Tumor: A Pediatric Oncology Success Story, a Critical Images slideshow, to help identify the clinical features, staging evaluation, prognostic factors, and therapeutic options for this disease.

Clinical findings include the following:

Asymptomatic abdominal mass (in 80% of children at presentation)

Abdominal pain or hematuria (25%)

Urinary tract infection and varicocele (less common)

Hypertension, gross hematuria, and fever (5-30%)

Hypotension, anemia, and fever (from hemorrhage into the tumor; uncommon)

Respiratory symptoms related to lung metastases (in patients with advanced disease; rare)

The following lab studies are indicated:

CBC

Chemistry profile – Including kidney function tests and routine measurements of electrolytes and calcium

Urinalysis

Coagulation studies

Cytogenetics studies, including 1p and 16q deletion

Imaging studies are as follows:

Renal ultrasonography (often the initial study)

Four-field chest radiography (to detect lung metastases)

Abdominal and chest CT

Abdominal MRI

Histologic confirmation in North America is with immediate nephrectomy, with exploration of the contralateral kidney to ensure that the disease is unilateral, and lymph node biopsy sampling for staging purposes. Immediate nephrectomy is not performed in patients with bilateral disease at presentation.

The usual approach is nephrectomy followed by chemotherapy, with or without postoperative radiotherapy. Chemotherapy regimens typically comprise vincristine and dactinomycin; doxorubicin and then cyclophosphamide and etoposide are added for increasingly high-risk disease. Children with loss of heterozygosity at 1p and 16q receive more aggressive chemotherapy.

Wilms tumor, or nephroblastoma, is the most common childhood abdominal malignancy. Over the past 5 decades, the multidisciplinary approach to this tumor has become an example for successful cancer treatment (see the images below). (See Treatment and Medications.)

At present, survival rates of children with Wilms tumor are approximately 80-90%. This is in contrast to the rate 50 years ago, when only 10% of children survived. The addition of radiation therapy to surgery alone improved survival rates to approximately 40%. With the use of chemotherapy, survival rates climbed to their current values. (See Prognosis.)

The National Wilms Tumor Study Group (NWTSG) and the International Society of Pediatric Oncology (SIOP) have identified several chemotherapeutic agents through clinical trials. When used together, these agents lead to a cure in most children with this renal tumor. In addition, the guidelines for surgical treatment and the role of radiation therapy are better defined now than ever before. (See Treatment and Medications.)

With the great improvement in survival rates, therapeutic trials came to focus on limiting treatment-related toxicity. ^[1] Understanding of the molecular mechanisms that contribute to the development of Wilms tumor has also greatly increased, making Wilms tumorigenesis a model for the understanding of the development of other tumors. (See Etiology.)

Stage I tumors have the following characteristics:

The tumor is limited to kidney and is completely resected

The renal capsule is intact

The tumor was not ruptured or biopsied prior to removal

The vessels of the renal sinus are not involved

No evidence of tumor is present at or beyond the margins of resection

Stage II tumors have the following characteristics:

The tumor is completely resected

No evidence of tumor at or beyond the margins of resection is noted

The tumor extends beyond the kidney (penetration of renal capsule, involvement of renal sinus)

Stage III tumors have the following characteristics:

A residual, nonhematogenous tumor is present following surgery and is confined to the abdomen

Positive lymph nodes in the abdomen or pelvis are noted

Penetration through the peritoneal surface is observed

Peritoneal implants are present

Gross or microscopic tumor remains postoperatively, including positive margins of resection

Tumor spillage is noted, including biopsy

The tumor is treated with preoperative chemotherapy

The tumor is removed in more than 1 piece

Stage IV tumors are characterized by the following:

Hematogenous metastases (eg, lung, liver, bone, brain) or lymph node metastases beyond the abdomen or pelvis are noted

Stage V tumors are characterized by the following:

Bilateral renal involvement by the tumor is present at diagnosis

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

Wilms tumor is thought to be caused by alterations of genes responsible for normal genitourinary development. Examples of common congenital anomalies associated with Wilms tumor are cryptorchidism, a double collecting system, horseshoe kidney, and hypospadias. Environmental exposures, although considered, seem relatively unlikely to play a role.

In the early 1970s, Knudson and Strong proposed a genetic model for the development of Wilms tumor. ^[2] WT1, the first Wilms tumor suppressor gene at chromosomal band 11p13, was identified as a direct result of the study of children with Wilms tumor who also had aniridia, genitourinary anomalies, and mental retardation (WAGR syndrome). ^[3]

Karyotypic analysis revealed constitutional deletions within the short arm of 1 copy of chromosome 11. The 11p13 locus was subsequently demonstrated to encompass numerous contiguous genes, including the aniridia gene PAX6 and the Wilms tumor suppressor gene WT1, which was cloned in 1990. WT1 encodes a transcription factor critical to normal renal and gonadal development.

Characterization of this novel tumor suppressor gene has provided insight into the mechanisms underlying normal kidney development and Wilms tumorigenesis. The WT1 gene is the specific target of mutations and deletions in a subset of patients with sporadic Wilms tumors, as well as in the germline of some children (eg, those with Denys-Drash syndrome) with a genetic predisposition to develop this cancer. ^[4]

A second gene that predisposes individuals to develop the Wilms tumor has been identified (but has not yet been cloned) telomeric of WT1, at 11p15. This locus was proposed on the basis of studies in patients with both Wilms tumor and Beckwith-Wiedemann syndrome (BWS), another congenital Wilms-tumor predisposition syndrome linked to chromosomal band 11p15. ^[3]

BWS is an overgrowth syndrome characterized by visceromegaly, macroglossia, and hyperinsulinemic hypoglycemia. In addition, patients with BWS are predisposed to have several embryonal neoplasms, including Wilms tumor. Thus far, a few candidate loci for Wilms tumor and BWS have been proposed. These loci include the insulinlike growth factor II gene (IGFII), H19 (for an untranslated ribonucleic acid [RNA]), and that encoding for p57kip2.

Results of linkage analyses in large pedigrees with familial transmission of susceptibility to the Wilms tumor suggest the existence of additional genetic loci.

Finally, loci at 16q, 1p, 7p, and 17p have also been implicated in the biology of Wilms tumor, although these loci do not seem to predispose individuals to develop a Wilms tumor. Instead, they seem to be associated with the phenotype or the outcome. ^{[1, 5]}

Wilms tumor affects approximately 10 children and adolescents per 1 million before age 15 years. Therefore, it accounts for 6-7% of all childhood cancers in North America. As a result, about 450-500 new cases are diagnosed each year on this continent. In 5-10% of patients, both kidneys are affected at the same time (synchronous bilateral Wilms tumor) or one after the other (metachronous bilateral Wilms tumor).

Wilms tumor appears to be relatively more common in Africa and least common in East Asia. ^[6] The incidence in Europe is similar to that reported in North America.

Wilms tumor is relatively more common in blacks than in whites and is rare in East Asians. Estimates suggest 6-9 cases per million person years in whites, 3-4 cases per million person years in East Asians and more than 10 cases per million person years among black populations. ^[6]

Among patients with unilateral Wilms tumor enrolled in all NWTSG protocols, the male-to-female ratio was 0.92:1. For patients with bilateral disease, the male-to-female ratio was 0.60:1.

The median age at diagnosis of Wilms tumor is approximately 3.5 years. The median age is highest for patients with unilateral unicentric disease (36.1 mo) and lowest for those with synchronous bilateral Wilms tumors (25.5 mo). ^[6]

A study by Heck et al looked to determine whether the risk for childhood cancers among Hispanic children varies by maternal birthplace. The study on more than 4 million children of non-Hispanic white mothers, more than 2 million children of U.S. born Hispanic mothers and 4 million children of non-U.S. born Hispanic mothers from 1983 to 2011 found that for certain cancer types, like glioma and astrocytoma, solid neuroblastoma, and Wilms tumor of the kidney, children of non-U.S. born Hispanic mothers had the lowest risk. ^{[7, 8]}

Approximately 80-90% of children with a diagnosis of Wilms tumor survive with current multimodality therapy. ^[9] 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 (Open Table in a new window)

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, ^{[10, 11]} whereas those with metachronous tumors have a 45-50% survival rate. ^[12]

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. ^[13]

Children with a loss of heterozygosity at 1p and 16q have a worse prognosis than do children without this heterozygosity loss. ^[5]

The prognosis for patients who have a relapse is not as good as it is for those with a newly diagnosed Wilms tumor, with 40-80% of relapse patients expected to survive after salvage therapy. Patients who relapse after having received vincristine and actinomycin D have a better survival with relapse treatment compared with those who initially received vincristine, actinomycin D, and doxorubicin. ^{[14, 15]}

Nephrectomy leaves the child with 1 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. ^{[16, 17]} In addition, chemotherapy and radiation therapy can induce second malignant neoplasms. ^[18]

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.

In a study of 7950 patients in the National Wilms Tumor Studies (from 1969-2002), 100 patients, or approximately 1%, developed end-stage renal disease. The primary risk factors included stromal predominant histology, intralobar rests, age at diagnosis of younger than 24 months, metachronous bilateral Wilms tumor, and WT1 mutation etiology. ^[19] The particularly high frequency of renal failure associated with metachronous bilateral Wilms tumor was due to surgery for progressive Wilms tumor.

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.

With the use of currently accepted radiotherapy techniques, radiation-induced hepatitis is rare in survivors of Wilms tumor.

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 in patients with Wilms tumor undergoing nephrectomy first and in those receiving combination chemotherapy before surgery, the standard approach that SIOP recommends. Although treatment for VOD is primarily supportive, the administration of chemotherapeutic agents can be resumed after the signs of VOD have disappeared.

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.

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.

Women who received whole-abdomen irradiation in childhood can develop ovarian failure. 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.

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 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]

Patients who survive Wilms tumor are at risk because 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.

In a study of 2,492 female subjects from the National Wilms Tumor Studies, Lange and colleagues found that female survivors of Wilms tumor had a 9.1-fold increased risk of developing invasive breast cancer and had an estimated cumulative risk of invasive breast cancer at age 40 of 4.5%. A total of 29 cases of invasive breast cancer were identified among 28 participants, all but four occurring before age 40. ^[21]

Relative to the general population, the risk was highest among women who had been treated with chest radiotherapy, who had a 27.6-fold increase and a cumulative risk at age 40 of 14.8%. Of the women who developed breast cancer after chest radiotherapy, most received doses of 12 Gy; the remainder received higher doses. No significant difference was found in breast cancer rates between women treated or not treated with abdominal irradiation. ^{[22, 21]}

Coppes MJ, Pritchard-Jones K. Principles of Wilms’ tumor biology. Urol Clin North Am. 2000 Aug. 27(3):423-33, viii. [Medline].

Knudson AG, Strong LC. Mutation and cancer: a model for Wilms’ tumor of the kidney. J Natl Cancer Inst. 1972 Feb. 48(2):313-24. [Medline].

Coppes MJ, Haber DA, Grundy PE. Genetic events in the development of Wilms’ tumor. N Engl J Med. 1994 Sep 1. 331(9):586-90. [Medline].

Coppes MJ, Huff V, Pelletier J. Denys-Drash syndrome: relating a clinical disorder to genetic alterations in the tumor suppressor gene WT1. J Pediatr. 1993 Nov. 123(5):673-8. [Medline].

Grundy PE, Breslow NE, Li S, et al. Loss of heterozygosity for chromosomes 1p and 16q is an adverse prognostic factor in favorable-histology Wilms tumor: a report from the National Wilms Tumor Study Group. J Clin Oncol. 2005 Oct 10. 23(29):7312-21. [Medline].

Breslow N, Olshan A, Beckwith JB, Green DM. Epidemiology of Wilms tumor. Med Pediatr Oncol. 1993. 21(3):172-81. [Medline].

Heck JE, Park AS, Contreras ZA, Davidson TB, Hoggatt KJ, Cockburn M, et al. Risk of Childhood Cancer by Maternal Birthplace: A Test of the Hispanic Paradox. JAMA Pediatr. 2016 Apr 25. [Medline].

Doyle K. Kids’ Cancer Risk Might Be Tied to Where Mom Was Born. Reuters Health Information. Available at http://www.medscape.com/viewarticle/862503. April 27, 2016; Accessed: April 27, 2016.

Green DM. The treatment of stages I-IV favorable histology Wilms’ tumor. J Clin Oncol. 2004 Apr 15. 22(8):1366-72. [Medline].

Montgomery BT, Kelalis PP, Blute ML, et al. Extended followup of bilateral Wilms tumor: results of the National Wilms Tumor Study. J Urol. 1991 Aug. 146(2 ( Pt 2)):514-8. [Medline].

Paulino AC, Wilimas J, Marina N, et al. Local control in synchronous bilateral Wilms tumor. Int J Radiat Oncol Biol Phys. 1996 Oct 1. 36(3):541-8. [Medline].

Paulino AC, Thakkar B, Henderson WG. Metachronous bilateral Wilms’ tumor: the importance of time interval to the development of a second tumor. Cancer. 1998 Jan 15. 82(2):415-20. [Medline].

Dome JS, Cotton CA, Perlman EJ, et al. Treatment of anaplastic histology Wilms’ tumor: results from the fifth National Wilms’ Tumor Study. J Clin Oncol. 2006 May 20. 24(15):2352-8. [Medline].

Green DM, Cotton CA, Malogolowkin M, et al. Treatment of Wilms tumor relapsing after initial treatment with vincristine and actinomycin D: a report from the National Wilms Tumor Study Group. Pediatr Blood Cancer. May/ 2007. 48:493-9. [Medline].

Malogolowkin M, Cotton CA, Green DM, et al. Treatment of Wilms tumor relapsing after initial treatment with vincristine, actinomycin D, and doxorubicin. A report from the National Wilms Tumor Study Group. Pediatr Blood Cancer. 2008 Feb. 50(2):236-41. [Medline].

Green DM, Donckerwolcke R, Evans AE, D’Angio GJ. Late effects of treatment for Wilms tumor. Hematol Oncol Clin North Am. 1995 Dec. 9(6):1317-27. [Medline].

Egeler RM, Wolff JE, Anderson RA, Coppes MJ. Long-term complications and post-treatment follow-up of patients with Wilms’ tumor. Semin Urol Oncol. 1999 Feb. 17(1):55-61. [Medline].

Evans AE, Norkool P, Evans I, et al. Late effects of treatment for Wilms’ tumor. A report from the National Wilms’ Tumor Study Group. Cancer. 1991 Jan 15. 67(2):331-6. [Medline].

Lange J, Peterson SM, Takashima JR, et al. Risk Factors for End Stage Renal Disease in Non-WT1-Syndromic Wilms Tumor. J Urol. 2011 Aug. 186(2):378-86. [Medline]. [Full Text].

Paulino AC, Wen BC, Brown CK, et al. Late effects in children treated with radiation therapy for Wilms’ tumor. Int J Radiat Oncol Biol Phys. 2000 Mar 15. 46(5):1239-46. [Medline].

Lange JM, Takashima JR, Peterson SM, Kalapurakal JA, Green DM, Breslow NE. Breast cancer in female survivors of Wilms tumor: A report from the National Wilms Tumor late effects study. Cancer. 2014 Oct 27. [Medline].

Boggs W. Increased Breast Cancer Risk in Female Survivors of Wilms Tumor. Medscape Medical News. Available at http://www.medscape.com/viewarticle/833933. Accessed: October 30, 2014.

van den Heuvel-Eibrink MM, Grundy P, Graf N, et al. Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: A collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups. Pediatr Blood Cancer. 2008 Jun. 50(6):1130-4. [Medline].

Refaie HD, Sarhan M, Hafez A. Role of CT in assessment of unresectable Wilms’ tumor response after preoperative chemotherapy in pediatrics. ScientificWorldJournal. 2008 Jul 13. 8:661-9. [Medline].

Mitchell C, Pritchard-Jones K, Shannon R, et al. Immediate nephrectomy versus preoperative chemotherapy in the management of non-metastatic Wilms’ tumour: results of a randomised trial (UKW3) by the UK Children’s Cancer Study Group. Eur J Cancer. 2006 Oct. 42(15):2554-62. [Medline].

Reinhard H, Semler O, Burger D, et al. Results of the SIOP 93-01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms Tumor. Klin Padiatr. 2004 May-Jun. 216(3):132-40. [Medline].

Coppes MJ, Arnold M, Beckwith JB, et al. Factors affecting the risk of contralateral Wilms tumor development: a report from the National Wilms Tumor Study Group. Cancer. 1999 Apr 1. 85(7):1616-25. [Medline].

D’Angio GJ, Breslow N, Beckwith JB, et al. Treatment of Wilms’ tumor. Results of the Third National Wilms’ Tumor Study. Cancer. 1989 Jul 15. 64(2):349-60. [Medline].

Kalapurakal JA, Li SM, Breslow NE, et al. Influence of radiation therapy delay on abdominal tumor recurrence in patients with favorable histology Wilms’ tumor treated on NWTS-3 and NWTS-4: a report from the National Wilms’ tumor Study Group. Int J Radiat Oncol Biol Phys. 2003. 57:495-9. [Medline].

Green DM, Breslow NE, Beckwith JB, et al. Treatment with nephrectomy only for small, stage I/favorable histology Wilms tumor: a report from the National Wilms Tumor Study Group. J Clin Oncol. Sep 1/ 2001. 19:3719-24. [Medline].

Meisel JA, Guthrie KA, Breslow NE, Donaldson SS, Green DM. Significance and management of computed tomography detected pulmonary nodules: a report from the National Wilms Tumor Study Group. Int J Radiat Oncol Biol Phys. 1999 Jun 1. 44(3):579-85. [Medline].

Grundy PE, Green DM, Dirks AC, et al. Clinical significance of pulmonary nodules detected by CT and Not CXR in patients treated for favorable histology Wilms tumor on national Wilms tumor studies-4 and -5: a report from the Children’s Oncology Group. Pediatr Blood Cancer. 2012 Oct. 59(4):631-5. [Medline]. [Full Text].

Verschuur A, Van Tinteren H, Graf N, Bergeron C, Sandstedt B, de Kraker J. Treatment of pulmonary metastases in children with stage IV nephroblastoma with risk-based use of pulmonary radiotherapy. J Clin Oncol. 2012 Oct 1. 30(28):3533-9. [Medline].

Hamilton TE, Ritchey ML, Haase GM, Argani P, Peterson SM, Anderson JR, et al. The management of synchronous bilateral wilms tumor: a report from the national wilms tumor study group. Ann Surg. 2011 May. 253(5):1004-10. [Medline].

D’Angio GJ, Rosenberg H, Sharples K, et al. Position paper: imaging methods for primary renal tumors of childhood: costs versus benefits [published erratum appears in Med Pediatr Oncol 1993;21(9):695]. Med Pediatr Oncol. 1993. 21(3):205-12. [Medline].

Pritchard-Jones K, Bergeron C, de Camargo B, van den Heuvel-Eibrink MM, Acha T, Godzinski J, et al. Omission of doxorubicin from the treatment of stage II-III, intermediate-risk Wilms’ tumour (SIOP WT 2001): an open-label, non-inferiority, randomised controlled trial. Lancet. 2015 Sep 19. 386 (9999):1156-64. [Medline].

Green DM. Doxorubicin in stage II-III, intermediate-risk Wilms’ tumour. Lancet. 2015 Sep 19. 386 (9999):1118-9. [Medline].

Boggs W. Doxorubicin Unnecessary for Some Children With Intermediate-Risk Wilms’ Tumor. Reuters Health Information. Available at http://www.medscape.com/viewarticle/848234. July 20, 2015; Accessed: April 27, 2016.

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. ^[28] Postoperative radiotherapy is started within 14 days of nephrectomy. ^[29] 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. ^[30] Children younger than 24 months with small (< 550 g) Wilms tumors with favorable histology are noted in the current COG protocol.

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.

Arnold C Paulino, MD Professor of Radiation Oncology, Methodist Hospital and Weill-Cornell Medical College; Associate Professor of Pediatrics, Baylor College of Medicine

Arnold C Paulino, MD is a member of the following medical societies: Radiological Society of North America, Children’s Oncology Group, American Society of Clinical Oncology, International Society of Paediatric Oncology, American Medical Association, American Radium Society, American Society for Radiation Oncology

Disclosure: Received royalty from Elsevier, Inc for author of book.

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, Children’s Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Jennifer Reikes Willert, MD Associate Clinical Professor, Department of Pediatrics, Division of Pediatric Hematology/Oncology, Section of Stem Cell Transplantation, Stanford University Medical Center, Lucile Packard Children’s Hospital

Jennifer Reikes Willert, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society for Blood and Marrow Transplantation, Children’s Oncology Group, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, Western Society for Pediatric Research

Disclosure: Nothing to disclose.

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