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Wilms' tumor

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sargun Singh Walia M.B.B.S.[2],Savni Satoskar, Shanshan Cen, M.D. [3]

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Steven C. Campbell, M.D., Ph.D.;

Synonyms and keywords: Nephroblastoma

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]

Overview

Wilms’ tumor or nephroblastoma is a tumor of the kidneys that typically occurs in children, rarely in adults. It was first described by Dr. Osler in 1814. The tumour typically arises from mesodermal precursors of the renal parenchyma (metanephros). On microscopic histopathological analysis, tubules, solid sheets of small round cells, and stroma are characteristic findings of wilms’ tumor. It may be caused by either genetic mutations or chromosomal alterations. The risk factors of wilms’ tumor include family history, congenital anomalies, and associated syndromes. The complications of wilms’ tumor include metastasis, high blood pressure, and kidney damage. Prognosis is generally good. The 5-year survival rate for Wilms tumor in children is around 90%, whereas older patients suffer worse outcome. The common symptoms include swelling, abdominal pain, fever, hypertension, and hypercalcemia. On abdominal examination a painless mass may be palpated in the flank. CT scan, MRI, Ultrasound, PET, and biopsy may be helpful in the diagnosis. The predominant therapy for wilms’ tumor is surgical resection. Adjunctive chemotherapy and radiation may be required.

Historical Perspective

Wilm’s Tumor was first discovered by Dr. Osler, in 1814.The association between Wilm’s tumor 1 (WT1) and Wilm’s tumor was made in 1990. Wilm’s tumor is named after Dr. Max Wilms (1867-1918) who is a surgeon and pathologist from Germany. In 1969, the National Wilms Tumor Study (NWTS) group devised a therapy regimen for wilms tumor. 

Pathophysiology

Wilms tumor has a triphasic appearance. It is comprised of 3 types of cells which are stromal, epithelial and blastemal. All the 3 types are not required for the diagnosis of wilms tumor. Primitive tubules and glomeruli are often seen comprised of neoplastic cells. Beckwith and Palmer reported in NWTS the different histopathologic types of wilms tumor to categorize them based on prognosis. Lesions comprising of nephrogenic rests can lead to wilms tumor. Wilms tumor (hereditary or sporadic) appears to result from changes in one or more of at least ten genes. Based on a study wilms tumor is divided into 2 pathologic categories: favorable and anaplastic. Wilms tumor (hereditary or sporadic) appears to result from changes in one or more of at least ten genes. The changes may be somatic or germline. Aberrations in germline or clonal WT1, WT2, and Wnt activation when combined with stage of development of the nephron, characterize different subsets of wilms tumor that can be differentiated by using gene expression profiling. This genetic/ontogenic categorization describes some of the heterogeneity among wilms tumors.

Causes

Wilms’ tumor may be caused by either genetic mutations or chromosomal alterations.

Wilms’ tumor differentiating from other disease

Wilms’ tumor must be differentiated from neuroblastoma, cystic nephroma, and angiomyolipoma.

Epidemiology and Demographics

Wilms tumors are the most common pediatric renal mass. The incidence of Wilms tumor is estimated to be 0.71 cases per 10,00,000 children younger than 15 years. There is no significant gender predilection among unilateral cases, but the male to female ratio in bilateral cases is 0.60:1.00. It typically occurs in early childhood with peak incidence between 3 and 4 years of age. Asian individuals are less likely to develop wilms tumor.

Risk factors

Common risk factors in the development of wilms tumor include familial wilms tumor, congenital anomalies, WT-1 related syndromes and WT2-related syndromes. Less common risk factors in the development of wilms tumor include perlman syndrome, simpson-golabi-ehemel syndrome, Sotos syndrome, 9q22.3 microdeletion syndrome, Bloom syndrome, li-fraumeni syndrome and Alagille syndrome.  

Screening

Screening can be very helpful to diagnose wilms tumor in high risk patients.Screening is done with serial abdominal ultrasonography.  

Natural history, Complications and Prognosis

The symptoms of wilms tumor usually develop in the first decade of life, and start with symptoms such as an abdominal/flank mass. If left untreated, patients with wilms tumor may progress to develop hypertension, anemia, weight loss, renal failure and metastasis. The overall 5-year survival rate is approximately 63% for patients aged 10 to 16 years. Depending on various factors at the time of diagnosis, the prognosis may vary. These factors are histology of tumor, stage of tumor, genetic and molecular markers and age of the patient. 

Staging

Staging of wilms tumor is done based upon the extent of tumor anatomically and not done on the basis of its genetics, histology or molecular markers. Extensive disease and worse prognosis is denoted by a higher stage. The staging systems that are currently used are National Wilms Tumor Study (NWTS) system and International Society of Pediatric Oncology (SIOP) system. Both these systems divide wilms tumor into 5 stages. 

Diagnostic Study Of Choice

Histology of the biopsy sample taken during surgery is the gold standard for the diagnosis of wilms tumor. Most of the tumors of the kidney have a favorable histology(90%).If anaplastic changes(3-7%) are found then the outcome is poor. If a case of wilms tumor is suspected in North America, then nephrectomy is done immediately. Contralateral kidney is also explored to check for disease and lymph node biopsies done. If tumor spill occurs then whole abdomen radiotherapy has to be done.

History and Symptoms

Patients with wilms tumor may have a positive history of an asymptomatic abdominal mass, abdominal pain and urinary tract infection. Common symptoms of wilms tumor include abdominal tenderness and Hematuria. Less common symptoms of wilms tumor include Hypertension, Fever, respiratory symptoms, Anemia, Varicocele, and Hypercalcemia. 

Physical Examination

Physical examination of patients with wilms tumor is usually remarkable for high blood pressure, fever, pallor, aniridia and a palpable abdominal mass.

Lab Findings

The lab studies conducted in patients suspected with wilms tumor are Complete blood count (CBC), coagulation studies, urinalysis, liver function test, serum creatinine, serum calcium and cytogenetics studies. 

Electrocardiogram

There are no ECG findings specific to wilms tumor.

X Ray

Abdominal x-ray typically reveals a large soft tissue opacity displacing bowel. This finding is incidental and cannot be used as a diagnostic tool.

Echocardiography or Ultrasound

CT

CT scan may be helpful in the diagnosis of wilms’ tumor.

MRI

MRI may be helpful in the diagnosis of wilms’ tumor.

Ultrasound

Ultrasound may be helpful in the diagnosis of wilms’ tumor.

Other Diagnostic Studies

18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET)-CT and biopsy may be helpful in the diagnosis of wilms’ tumor.

Medical therapy

The predominant therapy for wilms’ tumor is surgical resection. Adjunctive chemotherapy and radiation may be required.

Surgery

Surgery is the mainstay of treatment for wilms’ tumor.

References

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Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sargun Singh Walia M.B.B.S.[2]

Overview

Wilms Tumor was first discovered by Dr. Osler, in 1814.The association between tumor 1 gene (WT1) and Wilms tumor was made in 1990. Wilms tumor is named after Dr. Max Wilms (1867-1918) who is a surgeon and pathologist from Germany. In 1969, the National Wilms Tumor Study (NWTS) group devised a therapy regimen for Wilms tumor.

Historical Perspective

  • Wilms Tumor was first discovered by Dr. Osler, in 1814.
  • The association between Wilms tumor 1 (WT1) gene and Wilms tumor was made in 1990.[1]
  • Wilms tumor is named after Dr. Max Wilms (1867-1918) who was a surgeon and pathologist from Germany.[2]
  • In 1969, the National Wilms Tumor Study (NWTS) Group devised a therapy regimen for Wilms tumor.[3]

References

  1. ↑ Ariza L, Cañete A, Rojas A, Muñoz-Chápuli R, Carmona R (April 2018). “Role of the Wilms’ tumor suppressor gene Wt1 in pancreatic development”. Dev. Dyn. doi:10.1002/dvdy.24636. PMID 29708625.
  2. ↑ Raffensperger J (February 2015). “Max Wilms and his tumor”. J. Pediatr. Surg. 50 (2): 356–9. doi:10.1016/j.jpedsurg.2014.10.054. PMID 25638637.
  3. ↑ Davidoff, Andrew M. (2012). “Wilms Tumor”. Advances in Pediatrics. 59 (1): 247–267. doi:10.1016/j.yapd.2012.04.001. ISSN 0065-3101.

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]Sargun Singh Walia M.B.B.S.[3]

Overview

Wilms tumor has a triphasic appearance. It is comprised of 3 types of cells which are stromal, epithelial and blastemal. All the 3 types are not required for the diagnosis of Wilms tumor. Primitive tubules and glomeruli are often seen comprised of neoplastic cells. Beckwith and Palmer reported in NWTS the different histopathologic types of Wilms tumor to categorize them based on prognosis. Lesions comprising of nephrogenic rests can lead to Wilms tumor. Wilms tumor (hereditary or sporadic) appears to result from changes in one or more of at least ten genes. Based on a study Wilms tumor is divided into 2 pathologic categories: favorable and anaplastic. Wilms tumor (hereditary or sporadic) appears to result from changes in one or more of at least ten genes. The changes may be somatic or germline. Aberrations in germline or clonal WT1, WT2, and Wnt activation when combined with stage of development of the nephron, characterize different subsets of Wilms tumor that can be differentiated by using gene expression profiling. This genetic/ontogenic categorization describes some of the heterogeneity among Wilms tumors.

Pathophysiology

  • Wilms tumor has a triphasic appearance.
  • It is comprised of 3 types of cells:
  • All the 3 types are not required for the diagnosis of Wilms tumor.
  • Primitive tubules and glomeruli are often seen comprised of neoplastic cells.
  • Beckwith and Palmer reported in NWTS the different histopathologic types of Wilms tumor to categorize them based on prognosis.[1]
  • Spindled cell stroma surrounding abortive tubules and glomeruli is characteristic.
  • The stroma may include:
  • Lesions comprising of nephrogenic rests can lead to Wilms tumor.
    • Nephrogenic rests may be be prognostic for a recurrence of Wilms tumor.
    • These rests may be:
  • Wilms tumor metastases to the lung are common.
  • Based on a study, Wilms tumor is divided into 2 pathologic categories:
    • Favorable
      • Contains well developed components mentioned above.
    • Anaplastic
      • Contains diffuse anaplasia (poorly developed cells).

Genetics

  • Wilms tumor (hereditary or sporadic) appears to result from changes in one or more of at least ten genes.
  • The changes may be somatic or germline.[2]
  • Aberrations in germline or clonal WT1, WT2, and Wnt activation when combined with stage of development of the nephron, characterize different subsets of Wilms tumor that can be differentiated by using gene expression profiling.
  • This genetic/ontogenic categorization describes some of the heterogeneity among Wilms tumors.[3]

Wilms tumor 1 gene (WT1)

  • The WT1 gene is located on the short arm of chromosome 11 (11p13).
    • The normal function of WT1 is required for normal genitourinary development and is important for differentiation of the renal blastema.
    • When modern molecular genetic techniques are used in testing, the incidence of germline WT1 mutations is about 11%.
  • Most of these mutations may be diagnosed, or at least highly suspected, on the basis of clinical syndromic findings at or before diagnosis of Wilms tumor.
  • In a United Kingdom Children’s Cancer Study Group study of patients entered in clinical trials, about 2% of Wilms tumor patients had germline mutations in WT1 but no genitourinary abnormalities.
    • These were mostly de novo mutations in children presenting before age 2 years, and the tumors were mostly unilateral with stromal histology.
  • The relatively low number of reports of parent and child pairs with Wilms tumors and WT1 mutations may be the result of decreased fertility.
    • However, the offspring of a child who has a parent with Wilms tumor and WT1 mutation will be at risk for developing Wilms tumor.
  • Because deletion of WT1 was the first mutation found to be associated with Wilms tumor, WT1 was assumed to be a conventional tumor suppressor gene.
    • However, non-inactivating mutations can result in altered WT1 protein function that also results in Wilms tumor, such as in Denys-Drash syndrome.

Imprinting Cluster Regions (ICR) on chromosome 11p15 (WT2) and Beckwith-Wiedemann syndrome

  • A second Wilms tumor locus, WT2, maps to an imprinted region of chromosome 11p15.5, which, when it is a germline mutation, causes Beckwith-Wiedemann syndrome.[4]
  • About 3% of children with Wilms tumors have germline epigenetic or genetic changes at the 11p15.5 growth regulatory locus without any clinical manifestations of overgrowth.
  • Like children with Beckwith-Wiedemann syndrome, these children have an increased incidence of bilateral Wilms tumor or familial Wilms tumor.
  • Approximately 80% of patients with Beckwith-Wiedemann syndrome have a molecular defect of the 11p15 domain.
  • Various molecular mechanisms underlying Beckwith-Wiedemann syndrome have been identified.
  • Some of these abnormalities are genetic (germline mutations of the maternal allele of CDKNIC, paternal uniparental isodisomy of 11p15 , or duplication of part of the 11p15 domain) but are more frequently epigenetic (loss of methylation of the maternal ICR2/KvDMR1 or gain of methylation of the maternal ICR1).
  • Several candidate genes at the WT2 locus comprise the two independent imprinted domains IGF2/H19 and KIP2/LIT1.
  • Loss of heterozygosity, which exclusively affects the maternal chromosome, has the effect of upregulating paternally active genes and silencing maternally active ones.
  • A loss or switch of the imprint for genes (change in methylation status) in this region has also been frequently observed and results in the same functional aberrations.
  • The overall tumor risk in patients with Beckwith-Wiedemann syndrome has been estimated between 5% and 10%, with a risk between 1% (loss of imprinting at IC2) and 30% (gain of methylation at IC1 and paternal 11p15 isodisomy). [5]
  • Patients with IC1 gain of methylation only developed Wilms tumor, whereas other tumors such as neuroblastoma or hepatoblastoma could occur in patients with paternal 11p15 isodisomy.
  • Loss of imprinting or gene methylation is rarely found at other loci, supporting the specificity of loss of imprinting at 11p15.5.
  • Interestingly, Wilms tumors in Asian children are not associated with either nephrogenic rests or IGF2 loss of imprinting.
  • Approximately one-fifth of patients with Beckwith-Wiedemann syndrome who develop Wilms tumor present with bilateral disease, and metachronous bilateral disease is also observed.
  • The prevalence of Beckwith-Wiedemann syndrome is about 1% among children with Wilms tumor reported to the National Wilms Tumor Study (NWTS).[6]

Wilms tumor gene on the X chromosome (WTX)

  • A third gene, WTX, has been identified on the X chromosome and plays a role in normal kidney development.[7]
    • This gene is inactivated in approximately one-third of Wilms tumors, but germline mutations have not been observed in patients with Wilms tumor.
    • WTX mutations are equally distributed between males and females.
    • WTX inactivation is a frequent, but late, event in tumorigenesis and has no apparent effect on clinical presentation or prognosis.

Other genes and chromosomal alterations

  • Additional genes have been implicated in the pathogenesis and biology of Wilms tumor, including the following:
    • 1q:[8]
      • Gain of 1q or overexpression of genes from 1q has been associated with an adverse outcome.
      • In an analysis of 212 patients from NWTS-4 and the Pediatric Oncology Group Wilms Biology study, 27% of patients displayed 1q gain.
      • A strong relationship between 1q gain and 1p/16q loss was observed.
      • The 8-year event-free survival (EFS) rate was 76% for patients with 1q gain and 93% for those lacking 1q gain.
      • The 8-year overall survival (OS) rate was 89% for those with 1q gain and 98% for those lacking 1q gain.
      • Gain of 1q was not found to correlate with disease stage.
      • After stratification for stage of disease, 1q gain was associated with a significantly increased risk of disease recurrence.
  • 16q and 1p:[9]
    • Additional tumor-suppressor or tumor-progressive genes may lie on chromosomes 16q and 1p as evidenced by loss of heterozygosity for these regions in 17% and 11% of Wilms tumors, respectively.
  • CACNA1E:[10]
    • Overexpression and amplification of the gene CACNA1E located at 1q25.3, which encodes the ion-conducting alpha-1 subunit of R-type voltage-dependent calcium channels, may be associated with relapse in FH Wilms tumor.
  • 7p21
  • SKCG-1
  • TP53 (tumor suppressor gene)
  • FBXW7
  • PTCH1
  • DICER1
  • MYCN

References

  1. ↑ Jolly RD, Stellwagen E, Babul J, Vodkaĭlo LV, Titov VL, Moldomusaev DM, Maianskiĭ AN (November 1975). “Mannosidosis of Angus Cattle: a prototype control program for some genetic diseases”. Adv Vet Sci Comp Med. 19 (23): 1–21. PMID 1978.
  2. ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq
  3. ↑ Ruteshouser EC, Robinson SM, Huff V (June 2008). “Wilms tumor genetics: mutations in WT1, WTX, and CTNNB1 account for only about one-third of tumors”. Genes Chromosomes Cancer. 47 (6): 461–70. doi:10.1002/gcc.20553. PMC 4332772. PMID 18311776.
  4. ↑ Crider-Miller SJ, Reid LH, Higgins MJ, Nowak NJ, Shows TB, Futreal PA, Weissman BE (December 1997). “Novel transcribed sequences within the BWS/WT2 region in 11p15.5: tissue-specific expression correlates with cancer type”. Genomics. 46 (3): 355–63. doi:10.1006/geno.1997.5061. PMID 9441738.
  5. ↑ Rump P, Zeegers MP, van Essen AJ (July 2005). “Tumor risk in Beckwith-Wiedemann syndrome: A review and meta-analysis”. Am. J. Med. Genet. A. 136 (1): 95–104. doi:10.1002/ajmg.a.30729. PMID 15887271.
  6. ↑ Rump P, Zeegers MP, van Essen AJ (July 2005). “Tumor risk in Beckwith-Wiedemann syndrome: A review and meta-analysis”. Am. J. Med. Genet. A. 136 (1): 95–104. doi:10.1002/ajmg.a.30729. PMID 15887271.
  7. ↑ Rivera MN, Kim WJ, Wells J, Driscoll DR, Brannigan BW, Han M, Kim JC, Feinberg AP, Gerald WL, Vargas SO, Chin L, Iafrate AJ, Bell DW, Haber DA (February 2007). “An X chromosome gene, WTX, is commonly inactivated in Wilms tumor”. Science. 315 (5812): 642–5. doi:10.1126/science.1137509. PMID 17204608.
  8. ↑ Truong HT, Pratt EA, Ho C (April 1991). “Interaction of the membrane-bound D-lactate dehydrogenase of Escherichia coli with phospholipid vesicles and reconstitution of activity using a spin-labeled fatty acid as an electron acceptor: a magnetic resonance and biochemical study”. Biochemistry. 30 (16): 3893–8. PMID 1850292.
  9. ↑ Grundy PE, Telzerow PE, Breslow N, Moksness J, Huff V, Paterson MC (May 1994). “Loss of heterozygosity for chromosomes 16q and 1p in Wilms’ tumors predicts an adverse outcome”. Cancer Res. 54 (9): 2331–3. PMID 8162576.
  10. ↑ Natrajan R, Little SE, Reis-Filho JS, Hing L, Messahel B, Grundy PE, Dome JS, Schneider T, Vujanic GM, Pritchard-Jones K, Jones C (December 2006). “Amplification and overexpression of CACNA1E correlates with relapse in favorable histology Wilms’ tumors”. Clin. Cancer Res. 12 (24): 7284–93. doi:10.1158/1078-0432.CCR-06-1567. PMID 17189400.
Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]Sargun Singh Walia M.B.B.S.[3]

Overview

Wilms’ tumor may be caused by either genetic mutations or chromosomal alterations.

Causes

  • Other genes and chromosomal alterations:
    • 1q[4]
    • 16q and 1p[5]
    • CACNA1E[6]
    • 7p21
    • SKCG-1
    • TP53 (tumor suppressor gene)
    • FBXW7
    • PTCH1
    • DICER1
    • MYCN

References

  1. ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq
  2. ↑ Crider-Miller SJ, Reid LH, Higgins MJ, Nowak NJ, Shows TB, Futreal PA, Weissman BE (December 1997). “Novel transcribed sequences within the BWS/WT2 region in 11p15.5: tissue-specific expression correlates with cancer type”. Genomics. 46 (3): 355–63. doi:10.1006/geno.1997.5061. PMID 9441738.
  3. ↑ Rivera MN, Kim WJ, Wells J, Driscoll DR, Brannigan BW, Han M, Kim JC, Feinberg AP, Gerald WL, Vargas SO, Chin L, Iafrate AJ, Bell DW, Haber DA (February 2007). “An X chromosome gene, WTX, is commonly inactivated in Wilms tumor”. Science. 315 (5812): 642–5. doi:10.1126/science.1137509. PMID 17204608.
  4. ↑ Truong HT, Pratt EA, Ho C (April 1991). “Interaction of the membrane-bound D-lactate dehydrogenase of Escherichia coli with phospholipid vesicles and reconstitution of activity using a spin-labeled fatty acid as an electron acceptor: a magnetic resonance and biochemical study”. Biochemistry. 30 (16): 3893–8. PMID 1850292.
  5. ↑ Grundy PE, Telzerow PE, Breslow N, Moksness J, Huff V, Paterson MC (May 1994). “Loss of heterozygosity for chromosomes 16q and 1p in Wilms’ tumors predicts an adverse outcome”. Cancer Res. 54 (9): 2331–3. PMID 8162576.
  6. ↑ Natrajan R, Little SE, Reis-Filho JS, Hing L, Messahel B, Grundy PE, Dome JS, Schneider T, Vujanic GM, Pritchard-Jones K, Jones C (December 2006). “Amplification and overexpression of CACNA1E correlates with relapse in favorable histology Wilms’ tumors”. Clin. Cancer Res. 12 (24): 7284–93. doi:10.1158/1078-0432.CCR-06-1567. PMID 17189400.

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Differentiating Wilms’ tumor from other Diseases

Differentiating Wilms’ tumor from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sargun Singh Walia M.B.B.S.[2]

Overview

Wilms’ tumor must be differentiated from neuroblastoma, cystic nephroma, and angiomyolipoma.

Wilms’ tumor differentiating from other disease

S.No. Disease Symptoms Signs Diagnosis Comments
Abdominal Pain Hematuria Headache Abdominal mass Abdominal tenderness Ultrasonography CT scan Histology
1. Wilms tumor + + – + +
  • Wilms tumor has a triphasic appearance.
  • It is comprised of 3 types of cells:
  • All the 3 types are not required for the diagnosis of Wilms tumor.
  • Primitive tubules and glomeruli are often seen comprised of neoplastic cells.
  • Beckwith and Palmer reported in NWTS the different histopathologic types of Wilms tumor to categorize them based on prognosis.[4]
2. Renal cell carcinoma + + +/- + –
  • Ultrasound (US) may be helpful when CT scan results are equivocal. It is noteworthy to mention that not all renal cell carcinomas are detectable on ultrasound.
 Both CT and MRI may be used to detect neoplastic masses that may define renal cell carcinoma or metastasis of the primary cancer. CT scan and use of intravenous (IV) contrast is generally used for work-up and follow-up of patients with renal cell carcinoma. The histological pattern of renal cell carcinoma depends whether it is papillary, chromophobe or collecting duct renal cell carcinoma.
3. Rhabdoid kidney disease + + – + –
  • CT scan may be diagnostic of malignant rhabdoid tumor. Findings on CT scan suggestive of malignant rhabdoid tumor include a large, heterogenous, centrally located mass, which is lobulated with individual lobules separated by intervening areas of decreased attenuation, relating to either previous hemorrhage or necrosis. Enhancement is similarly heterogeneous. Calcification is relatively common, observed in 20-50% of cases and is typically linear and tends to outline tumor lobules.
  • Malignant rhabdoid tumor is characterized by the round blue tumor cells of high cellularity composed of atypical cells with eccentric nuclei, small nucleoli, and abundant amounts of eosinophilic cytoplasm with frequent mitotic figures.
4. Polycystic kidney disease + + + (from hypertension) + –

Ultrasound may be helpful in the diagnosis of polycystic kidney disease. Findings on an ultrasound diagnostic of polycystic kidney disease include:[5][6]

  • At least three unilateral or bilateral cysts in patients 15 – 39 years old
  • Atleast two cysts in each kidney in patients 40 – 59 years old
  • Atleast four cysts in each kidney in patients 60 years of age or older

Renal CT scan may be helpful in the diagnosis of polycystic kidney disease. Findings on CT scan diagnostic of ADPKD include:

  • Numerous renal cysts of varying size and shape with little intervening parenchyma with water attenuation and very thin wall.
  • Reduction in sinus fat due to expansion of the cortex
  • Occasional complex cysts with hyperdense appearance, with possible septations or calcifications
  • Multiple homogeneous and hypoattenuating cystic lesions in the liver in patients with liver involvement
  • On microscopic histopathological analysis, interstitial fibrosis, tubular atrophy, thickening and lamellation of tubular basement membranes, microcysts and negative immunofluorescence for complement and immunoglobulin are characteristic findings of ADPKD.[7][8][9][10]
5. Pheochromocytoma – – + (as a part of the hypertension paroxysm) – –
  • CT is the preferred imaging modality for the diagnosis of pheochromocytoma.
 The following findings may be observed on CT scan:[11]
  • On microscopic pathology, Pheochromocytoma typically demonstrates a nesting (Zellballen) pattern on microscopy. This pattern is composed of well-defined clusters of tumor cells containing eosinophilic cytoplasm separated by fibrovascular stroma.
6. Burkitt lymphoma +/- (in non-endemic or sporadic form of the disease) – – – –
  • On microscopic histopathological analysis, characteristic findings of Burkitt’s lymphoma include:[16]
  • Medium-sized (~1.5-2x the size of a RBC) with uniform size (“monotonous”) — key feature (i.e. tumor nuclei size similar to that of histiocytes or endothelial cells)
  • Round nucleus
  • Small nucleoli
  • Relatively abundant cytoplasm (basophilic)
  • Brisk mitotic rate and apoptotic activity
  • Cellular outline usually appears squared off
  • “Starry-sky pattern”:
  • The stars in the pattern are tingible-body macrophages (macrophages containing apoptotic tumor cells.
  • The tumour cells are the sky
7. Intussusception + – – +/- +
  • Ultrasound is the gold standard imaging modality used to diagnose intussusception[17]
    • Target or doughnut sign[18]
      • Edematous intussuscipien forms an external ring around the centrally located intussusceptum
      • Target sign is usually seen in right lower quadrant
    • Layers of intussusception forms pseudo-kidney appearance on the transverse view
  • CT scan may be helpful in the diagnosis of intussusception. CT scan maybe used when other image modalities like x-ray and ultrasound have not given positive results but suspicion of intussusception is high.
  • Intussusception occurs if there is an imbalance between the longitudinal and radial smooth muscle forces of intestine that maintain its normal structure. This imbalance leads to a segment of intestine to invaginate into another segment and cause entero-enteral intussusception. Etiology of intussusception is either idiopathic or pathologic (lead point). 
8. Hydronephrosis + +/- – – + (CVA tenderness in case of pyelonephritis)
  • In the case of renal colic (one sided loin pain usually accompanied by a trace of blood in the urine) the initial investigation is usually an intravenous urogram. This has the advantage of showing whether there is any obstruction of flow of urine causing hydronephrosis as well as demonstrating the function of the other kidney. Many stones are not visible on plain x ray or IVU but 99% of stones are visible on CT and therefore CT is becoming a common choice of initial investigation.
  • The kidney undergoes extensive dilation with atrophy and thinning of the renal cortex.
9. Dysplastic kidney N/A N/A N/A N/A N/A

MCDK is usually diagnosed by ultrasound examination before birth.

  • Mass of non-communicating cysts of variable size.
  • Unlike severe hydronephrosis, in which the largest cystic structure (the renal pelvis) lies in a central location and is surrounded by dilated calices, in multicystic dysplastic kidney the cyst distribution shows no recognizable pattern.
  • Dysplastic, echogenic parenchyma may be visible between the cysts, but no normal renal parenchyma is seen.
  • MCKD can be discovered accidentally on CT scan.
  • CT scan shows myltiple cysts with absence of renal parenchyma.
  • MCKD is the result of abnormal differentiation of the renal parenchyma.
10. Pediatric Neuroblastoma + – – +/- +/-
  • CT scan is the investigation of choice for the diagnosis of neuroblastoma.[20]
  • On CT scan, neuroblastoma is characterized by:[21]
  • On microscopic histopathological analysis the presence of round blue cells separated by thin fibrous septa are characteristic findings of neuroblastoma.
  • Other findings of neuroblastoma on light microscopy may include:[22]
  • Homer-Wright rosettes (rosettes with a small meshwork of fibers at the center)
  • Neuropil-like stroma (paucicellular stroma with a cotton candy-like appearance)
11. Pediatric Rhabdomyosarcoma + +/- +/- – +/- On CT scan, rhabdomyosarocma is characterized by:
  • Soft tissue density
  • Some enhancement with contrast
  • Adjacent bony destruction (over 20% of cases)
12. Mesoblastic nephroma + + – + –
  • Ultrasound may be helpful in the diagnosis of mesoblastic nephroma.
  • Mesoblastic nephroma may presents as a well-defined mass with low-level homogeneous echoes.[23]
  • The presence of concentric echogenic and hypoechoic rings can be a helpful diagnostic feature of mesoblastic nephroma.
  • CT scan may be helpful in the diagnosis of mesoblastic nephroma.
  • Findings on CT scan suggestive of mesoblastic nephroma include:
  • Solid hypoattenuating renal lesion
  • Variable contrast enhancement
  • No calcification

Classic mesoblastic nephroma

Cellular mesoblastic nephroma

  • Plump cells with vesicular nuclei
  • Well-defined border
  • Mitotically active

Mixed mesoblastic nephroma

  • Both classic pattern and cellular pattern areas are present
 Most common renal tumor that occurs in 1st month of life

References

  1. ↑ Hartman DS, Sanders RC (April 1982). “Wilms’ tumor versus neuroblastoma: usefulness of ultrasound in differentiation”. J Ultrasound Med. 1 (3): 117–22. PMID 6152936.
  2. ↑ De Campo JF (1986). “Ultrasound of Wilms’ tumor”. Pediatr Radiol. 16 (1): 21–4. PMID 3003660.
  3. ↑ Cahan LD (1985). “Failure of encephalo-duro-arterio-synangiosis procedure in moyamoya disease”. Pediatr Neurosci. 12 (1): 58–62. PMID 4080660.
  4. ↑ Jolly RD, Stellwagen E, Babul J, Vodkaĭlo LV, Titov VL, Moldomusaev DM, Maianskiĭ AN (November 1975). “Mannosidosis of Angus Cattle: a prototype control program for some genetic diseases”. Adv Vet Sci Comp Med. 19 (23): 1–21. PMID 1978.
  5. ↑ Chapman AB, Devuyst O, Eckardt KU, Gansevoort RT, Harris T, Horie S, Kasiske BL, Odland D, Pei Y, Perrone RD, Pirson Y, Schrier RW, Torra R, Torres VE, Watnick T, Wheeler DC (July 2015). “Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference”. Kidney Int. 88 (1): 17–27. doi:10.1038/ki.2015.59. PMC 4913350. PMID 25786098.
  6. ↑ Pei Y, Obaji J, Dupuis A, Paterson AD, Magistroni R, Dicks E, Parfrey P, Cramer B, Coto E, Torra R, San Millan JL, Gibson R, Breuning M, Peters D, Ravine D (January 2009). “Unified criteria for ultrasonographic diagnosis of ADPKD”. J. Am. Soc. Nephrol. 20 (1): 205–12. doi:10.1681/ASN.2008050507. PMC 2615723. PMID 18945943.
  7. ↑ Stavrou C, Koptides M, Tombazos C, Psara E, Patsias C, Zouvani I, Kyriacou K, Hildebrandt F, Christofides T, Pierides A, Deltas CC (October 2002). “Autosomal-dominant medullary cystic kidney disease type 1: clinical and molecular findings in six large Cypriot families”. Kidney Int. 62 (4): 1385–94. doi:10.1111/j.1523-1755.2002.kid581.x. PMID 12234310.
  8. ↑ Bleyer AJ, Kmoch S, Antignac C, Robins V, Kidd K, Kelsoe JR, Hladik G, Klemmer P, Knohl SJ, Scheinman SJ, Vo N, Santi A, Harris A, Canaday O, Weller N, Hulick PJ, Vogel K, Rahbari-Oskoui FF, Tuazon J, Deltas C, Somers D, Megarbane A, Kimmel PL, Sperati CJ, Orr-Urtreger A, Ben-Shachar S, Waugh DA, McGinn S, Bleyer AJ, Hodanová K, Vylet’al P, Živná M, Hart TC, Hart PS (March 2014). “Variable clinical presentation of an MUC1 mutation causing medullary cystic kidney disease type 1”. Clin J Am Soc Nephrol. 9 (3): 527–35. doi:10.2215/CJN.06380613. PMC 3944763. PMID 24509297.
  9. ↑ Faguer S, Decramer S, Chassaing N, Bellanné-Chantelot C, Calvas P, Beaufils S, Bessenay L, Lengelé JP, Dahan K, Ronco P, Devuyst O, Chauveau D (October 2011). “Diagnosis, management, and prognosis of HNF1B nephropathy in adulthood”. Kidney Int. 80 (7): 768–76. doi:10.1038/ki.2011.225. PMID 21775974.
  10. ↑ Heidet L, Decramer S, Pawtowski A, Morinière V, Bandin F, Knebelmann B, Lebre AS, Faguer S, Guigonis V, Antignac C, Salomon R (June 2010). “Spectrum of HNF1B mutations in a large cohort of patients who harbor renal diseases”. Clin J Am Soc Nephrol. 5 (6): 1079–90. doi:10.2215/CJN.06810909. PMC 2879303. PMID 20378641.
  11. ↑ Bravo EL (1991). “Pheochromocytoma: new concepts and future trends”. Kidney Int. 40 (3): 544–56. PMID 1787652.
  12. ↑ Whalen RK, Althausen AF, Daniels GH (1992). “Extra-adrenal pheochromocytoma”. J Urol. 147 (1): 1–10. PMID 1729490.
  13. ↑ Baid SK, Lai EW, Wesley RA, Ling A, Timmers HJ, Adams KT; et al. (2009). “Brief communication: radiographic contrast infusion and catecholamine release in patients with pheochromocytoma”. Ann Intern Med. 150 (1): 27–32. PMC 3490128. PMID 19124817.
  14. ↑ Bravo EL (1991). “Pheochromocytoma: new concepts and future trends”. Kidney Int. 40 (3): 544–56. PMID 1787652.
  15. ↑ Burkitt lymphoma. MedlinePlus. https://www.nlm.nih.gov/medlineplus/ency/article/001308.htm Accessed on September 30, 2015
  16. ↑ Bellan C, Lazzi S, De Falco G, Nyongo A, Giordano A, Leoncini L (2003). “Burkitt’s lymphoma: new insights into molecular pathogenesis”. J. Clin. Pathol. 56 (3): 188–92. PMC 1769902. PMID 12610094. Unknown parameter |month= ignored (help)
  17. ↑ Ko HS, Schenk JP, Tröger J, Rohrschneider WK (2007). “Current radiological management of intussusception in children”. Eur Radiol. 17 (9): 2411–21. doi:10.1007/s00330-007-0589-y. PMID 17308922.
  18. ↑ Boyle MJ, Arkell LJ, Williams JT (1993). “Ultrasonic diagnosis of adult intussusception”. Am. J. Gastroenterol. 88 (4): 617–8. PMID 8470658.
  19. ↑ Neuroblastoma. Radiopaedia (2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 8 2015
  20. ↑ Colon NC, Chung DH (2011). “Neuroblastoma”. Adv Pediatr. 58 (1): 297–311. doi:10.1016/j.yapd.2011.03.011. PMC 3668791. PMID 21736987.
  21. ↑ Neuroblastoma. Radiopaedia (2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 8 2015
  22. ↑ Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015
  23. ↑ Mesoblastic nephroma.Dr Ayush Goel and Dr Yuranga Weerakkody et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/mesoblastic-nephroma
Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sargun Singh Walia M.B.B.S.[2]Shanshan Cen, M.D. [3]

Overview

Wilms tumors are the most common pediatric renal mass. The incidence of Wilms tumor is estimated to be 0.71 cases per 10,00,000 children younger than 15 years. There is no significant gender predilection among unilateral cases, but the male to female ratio in bilateral cases is 0.60:1.00. It typically occurs in early childhood with peak incidence between 3 and 4 years of age. Asian individuals are less likely to develop Wilms tumor.

Epidemiology and Demographics

Prevalence

  • Wilms tumours are the most common paediatic renal mass, accounting for over 85% of cases and accounts for 6% of all childhood cancers.[1]

Incidence

  • The incidence of Wilms tumor is 7.1 cases per 1 million children younger than 15 years.[2]
  • Approximately 500 cases of Wilms tumor are diagnosed in the United States each year.[3]

Age

  • It typically occurs in early childhood (1-11 years) with peak incidence between 3 and 4 years of age.
  • Approximately 80% of these tumors are found before the age of 5 years.
  • When part of a syndrome they occur even earlier, typically between 2 and 24 months of age.[4]
  • The mean age at diagnosis is 44 months in unilateral cases of Wilms tumor and 31 months in bilateral cases.

Sex

  • The male to female ratio in unilateral cases of Wilms tumor is 0.92:1.00, but in bilateral cases it is 0.60:1.00.

Race

  • The incidence is substantially lower in Asians.

References

  1. ↑ Wallenstein G, Rebohle E, Voigt U, Schneider WD, Bergmann I (August 1976). “[Trypsin-inhibitory – capacity in humans to respirable allergens or irritants (author’s transl)]”. Z Erkr Atmungsorgane (in German). 146 (2): 120–7. PMID 65842.
  2. ↑ Morimoto T (January 1970). “[Circadian rhythm of urine composition and kidney function]”. Nippon Rinsho (in Japanese). 28 (1): 193–5. PMID 5461897.
  3. ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq
  4. ↑ Wilms tumour. Dr Tim Luijkx and Dr Frank Gaillard et al. Radiopaedia.org 2015.http://radiopaedia.org/articles/wilms-tumour

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Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sargun Singh Walia M.B.B.S.[2],Shanshan Cen, M.D. [3]

Overview

Common risk factors in the development of wilms tumor include familial wilms tumor, congenital anomalies, WT-1 related syndromes and WT2-related syndromes. Less common risk factors in the development of wilms tumor include perlman syndrome, simpson-golabi-ehemel syndrome, Sotos syndrome, 9q22.3 microdeletion syndrome, Bloom syndrome, li-fraumeni syndrome and Alagille syndrome.

Risk factors

Common Risk Factors

Less Common Risk Factors

References

  1. ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq
  2. ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq
  3. ↑ Crider-Miller SJ, Reid LH, Higgins MJ, Nowak NJ, Shows TB, Futreal PA, Weissman BE (December 1997). “Novel transcribed sequences within the BWS/WT2 region in 11p15.5: tissue-specific expression correlates with cancer type”. Genomics. 46 (3): 355–63. doi:10.1006/geno.1997.5061. PMID 9441738.
  4. ↑ Morris MR, Astuti D, Maher ER (May 2013). “Perlman syndrome: overgrowth, Wilms tumor predisposition and DIS3L2”. Am J Med Genet C Semin Med Genet. 163C (2): 106–13. doi:10.1002/ajmg.c.31358. PMID 23613427.
  5. ↑ Alton KB, Patrick JE, Shaw C, McGuire JL (1975). “Comparative biotransformation of triflubazam in rats, dogs, and monkeys”. Drug Metab. Dispos. 3 (6): 445–52. PMID 1219.
  6. ↑ Heller BR, Walsh FJ (January 1976). “Changing nursing students’ attitudes toward the aged: an experimental study”. J Nurs Educ. 15 (1): 9–17. PMID 1479.
  7. ↑ Berger C, Frappaz D, Leroux D, Blez F, Vercherat M, Bouffet E, Jalbert P, Brunat-Mentigny M (August 1996). “[Wilms tumor and Bloom syndrome]”. Arch Pediatr (in French). 3 (8): 802–5. PMID 8998536.
  8. ↑ Hartley AL, Birch JM, Tricker K, Wallace SA, Kelsey AM, Harris M, Jones PH (June 1993). “Wilms’ tumor in the Li-Fraumeni cancer family syndrome”. Cancer Genet. Cytogenet. 67 (2): 133–5. PMID 8392435.
  9. ↑ Bourdeaut F, Guiochon-Mantel A, Fabre M, Martelli H, Patte C, Porta G, Bernard O, Delattre O, Jacquemin E (April 2008). “Alagille syndrome and nephroblastoma: Unusual coincidence of two rare disorders”. Pediatr Blood Cancer. 50 (4): 908–11. doi:10.1002/pbc.21255. PMID 17584876.
Screening

Screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sargun Singh Walia M.B.B.S.[2]

Overview

Screening can be very helpful to diagnose wilms tumor in high risk patients.Screening is done with serial abdominal ultrasonography.

Screening

  • Screening can be very helpful to diagnose wilms tumor in high risk patients.[1]
  • High risk patients include:
  • Screening is done with serial abdominal ultrasonography.[2]
  • Serial abdominal ultrasonography is done as follows:
    • Children suffering from beckwith-wiedemann syndrome:
      • Ultrasonography every three months until age seven years.
    • Children suffering with WAGR and WT1-related syndromes:
      • Ultrasonography every three months until age five years
    • Siblings of an individual with familial wilms tumor:
      • Ultrasonography every three months until age eight years.
    • Offspring of survivors of bilateral wilms tumor:
      • Ultrasonography every three months until age eight years.

References

  1. ↑ Green DM, Breslow NE, Beckwith JB, Norkool P (1993). “Screening of children with hemihypertrophy, aniridia, and Beckwith-Wiedemann syndrome in patients with Wilms tumor: a report from the National Wilms Tumor Study”. Med. Pediatr. Oncol. 21 (3): 188–92. PMID 8095320.
  2. ↑ Kalish JM, Doros L, Helman LJ, Hennekam RC, Kuiper RP, Maas SM, Maher ER, Nichols KE, Plon SE, Porter CC, Rednam S, Schultz K, States LJ, Tomlinson GE, Zelley K, Druley TE (July 2017). “Surveillance Recommendations for Children with Overgrowth Syndromes and Predisposition to Wilms Tumors and Hepatoblastoma”. Clin. Cancer Res. 23 (13): e115–e122. doi:10.1158/1078-0432.CCR-17-0710. PMID 28674120. Vancouver style error: initials (help)

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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sargun Singh Walia M.B.B.S.[2]

Overview

The symptoms of wilms tumor usually develop in the first decade of life, and start with symptoms such as an abdominal/flank mass. If left untreated, patients with wilms tumor may progress to develop hypertension, anemia, weight loss, renal failure and metastasis. The overall 5-year survival rate is approximately 63% for patients aged 10 to 16 years. Depending on various factors at the time of diagnosis, the prognosis may vary. These factors are histology of tumor, stage of tumor, genetic and molecular markers and age of the patient.

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of wilms tumor usually develop in the first decade of life, and start with symptoms such as an abdominal/flank mass.
  • If left untreated, patients with wilms tumor may progress to develop hypertension, anemia, weight loss, renal failure and metastasis.
  • The overall 5-year survival rate is approximately 63% for patients aged 10 to 16 years.

Complications

  • Common complications of wilms tumor include:
    • Hypertension
    • Anu
    • Anemia
    • Weight loss
    • Renal failure
    • Metastasis to:[1]
      • Lungs
      • Liver
      • Bone
      • Brain

Prognosis

  • Depending on various factors at the time of diagnosis, the prognosis may vary.[2]
  • However, the prognosis can be tumor recurrence or death.
  • These factors are:
    • Histology of tumor
    • Stage of the tumor
    • Genetic and molecular markers
    • Age of the patient
Histology of tumor
  • 2 research groups have classified wilms tumor based on histology. These are:
    •  National Wilms Tumor Study / Children’s Oncology Group (NWTS/COG) .
    • International Society of Pediatric Oncology (SIOP).
  •  Prognosis is dependant on presence or absence of anaplasia.
  •  If anaplasia is positive then it signifies a poor prognosis in children.[3][4]

Stage of the tumor

  • Staging of wilms tumor is done on the basis of:[5]
    • Genetics
    • Histology
    • Molecular markers
  • Stage I (43% of patients)
    • Outcome: 98% 4-year survival; 85% 4-year survival if anaplastic
  • Stage II (23% of patients)
    • Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic
  • Stage III (23% of patients)
    • Outcome: 95% 4-year survival; 56% 4-year survival if anaplastic
  • Stage IV (10% of patients)
    • Outcome: 90% 4-year survival; 17% 4-year survival if anaplastic

Genetic and molecular markers

  • Multiple genetic and molecular markers can predict prognosis.[6] [7]
  • These markers are:
    • Loss of heterozygosity (LOH) at chromosomes:
      • 1p
      • 11p15
      • 16q
    • Gain in :
      • 1q
  • These markers are associated with increased risk of relapse and mortality.

Age of the patient

  • Patient with age lass than 2 years have a better prognosis.[8]
  • As the patient gets old the treatment related complication rate also increases.[9]

References

  1. ↑ Termuhlen AM, Tersak JM, Liu Q, Yasui Y, Stovall M, Weathers R, Deutsch M, Sklar CA, Oeffinger KC, Armstrong G, Robison LL, Green DM (December 2011). “Twenty-five year follow-up of childhood Wilms tumor: a report from the Childhood Cancer Survivor Study”. Pediatr Blood Cancer. 57 (7): 1210–6. doi:10.1002/pbc.23090. PMC 4634648. PMID 21384541.
  2. ↑ Dome JS, Graf N, Geller JI, Fernandez CV, Mullen EA, Spreafico F, Van den Heuvel-Eibrink M, Pritchard-Jones K (September 2015). “Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration”. J. Clin. Oncol. 33 (27): 2999–3007. doi:10.1200/JCO.2015.62.1888. PMC 4567702. PMID 26304882.
  3. ↑ Zuppan CW, Beckwith JB, Luckey DW (October 1988). “Anaplasia in unilateral Wilms’ tumor: a report from the National Wilms’ Tumor Study Pathology Center”. Hum. Pathol. 19 (10): 1199–209. PMID 2844645.
  4. ↑ D’Angio GJ, Evans A, Breslow N, Beckwith B, Bishop H, Farewell V, Goodwin W, Leape L, Palmer N, Sinks L, Sutow W, Tefft M, Wolff J (May 1981). “The treatment of Wilms’ tumor: results of the Second National Wilms’ Tumor Study”. Cancer. 47 (9): 2302–11. PMID 6164480.
  5. ↑ Metzger ML, Dome JS (2005). “Current therapy for Wilms’ tumor”. Oncologist. 10 (10): 815–26. doi:10.1634/theoncologist.10-10-815. PMID 16314292.
  6. ↑ Perlman EJ, Grundy PE, Anderson JR, Jennings LJ, Green DM, Dome JS, Shamberger RC, Ruteshouser EC, Huff V (February 2011). “WT1 mutation and 11P15 loss of heterozygosity predict relapse in very low-risk wilms tumors treated with surgery alone: a children’s oncology group study”. J. Clin. Oncol. 29 (6): 698–703. doi:10.1200/JCO.2010.31.5192. PMC 3056654. PMID 21189373.
  7. ↑ D’Angio GJ (September 2008). “Pre- or postoperative therapy for Wilms’ tumor?”. J. Clin. Oncol. 26 (25): 4055–7. doi:10.1200/JCO.2008.16.5316. PMID 18757319.
  8. ↑ Breslow NE, Palmer NF, Hill LR, Buring J, D’Angio GJ (April 1978). “Wilms’ tumor: prognostic factors for patients without metastases at diagnosis: results of the National Wilms’ Tumor Study”. Cancer. 41 (4): 1577–89. PMID 205340.
  9. ↑ Reinhard H, Aliani S, Ruebe C, Stöckle M, Leuschner I, Graf N (November 2004). “Wilms’ tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study”. J. Clin. Oncol. 22 (22): 4500–6. doi:10.1200/JCO.2004.12.099. PMID 15542800.

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Diagnosis

Diagnosis

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