Question 1 of 10

    Nephroblastoma (Wilms Tumor)

    Wilms tumor[1]

    Nephroblastoma, also known as Wilms tumor, is the most common renal malignancy in children. Patients will typically have an asymptomatic abdominal mass although some will have other features as well. The prognosis is excellent due to advances in therapy.

    The disease is caused by the following process: neoplastic.

    Presentation

    Nephroblastoma, also referred to as Wilms tumor, is the fifth most common malignancy in childhood and the most frequent renal tumor in the pediatric population [1] [2]. Additionally, its peak incidence is in early childhood between the ages of 3 to 4 [3]. Patients with trisomies 13 and 18, Beckwith-Wiedemann syndrome, and Drash syndrome are at risk for developing this tumor [3]. Due to advances in treatment, the survival rate is 80% to 90%.

    Regarding the presentation, the majority of patients will have an asymptomatic abdominal mass [4] [5] while some will also develop manifestations such as malaise, abdominal pain, hematuria, and hypertension [5]. The mass may be discovered incidentally or during a workup following trauma, hematuria, or hypertension [4]. In rare cases, the tumor ruptures and causes fever, pain, and anemia [5]. Furthermore, respiratory symptoms are present in those with metastasis to the lungs.

    Nephroblastoma adheres to the "rules of 10s," which relates that 10% of these cases are bilateral, 10% develop vascular invasion, 10% exhibit lung metastasis at initial presentation, 10% have histology suggestive of poor outcome, and 10% demonstrate calcifications on imaging [6].

    Complications

    There are adverse effects associated with various types of treatment. For example, radiation therapy poses danger to the remaining kidney while chemotherapy agents are harmful to the liver and other organs. Also, removal of one kidney will lead t0 hypertrophy of the remaining kidney.

    Physical exam

    Remarkable findings include an abdominal mass, which should be palpated carefully to avoid rupture. Other notable features will be those of associated syndromes.

    Liver, Gall & Pancreas
    Hepatomegaly
    • VOD is primarily a clinical diagnosis characterized by hepatomegaly or pain in the right upper quadrant, jaundice, ascites, and unexplained weight gain.[emedicine.medscape.com]
    • Beckwith-Wiedemann syndrome: 4–10% risk for Wilms tumor, excessive growth at the cellular level, organ level (macroglossia, nephromegaly, hepatomegaly) or body segment level (hemihypertrophy), WT2 mutations (see below).[urology-textbook.com]
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  • Entire body system
    Anemia
    • Patients sometimes experience abdominal pain (around 10% of cases), hypertension, fever (20% of cases), hematuria and anemia.[orpha.net]
    • Urinalysis of renal nephroblastoma usually reveals varying degrees of nonspecific hematuria, pyuria and proteinuria, and hematological analysis may show varying degrees of neutrophilia, anemia and thrombocytopenia.[vetbook.org]
    • […] common finding with renal tumors hematuria is uncommon urine sediment cytology is rarely diagnostic for renal tumors Blood Tests hematology and serum biochemistry findings are usually normal or non-specific mild-to-moderate normochromic, normocytic anemia[vsso.org]
    • […] physician, but may include the following: Urinalysis: a sample of urine will be examined for blood, sugar, protein and bacteria Blood tests: a sample of blood will be drawn and the following tests will be ordered from it: Complete blood count to look for anemia[surgery4children.com]
    • The patients were followed during the period of this study, and readmitted several times in the Pediatric Oncology Unit (POU)according to their chemotherapy schedule or if there were other medical indications (infections, anemia or thrombocytopenia which[panafrican-med-journal.com]
    Fever
    • Your child is being treated for this condition and symptoms get worse or new symptoms develop, mainly cough, chest pain, weight loss, or persistent fevers.[nlm.nih.gov]
    • Later, symptoms may indicate fever, distortion of the kidney mass, evidence of secondary tumours elsewhere in the body, abdominal and flank pain, weight loss, nausea, loss of appetite, and vomiting.[britannica.com]
    • Patients sometimes experience abdominal pain (around 10% of cases), hypertension, fever (20% of cases), hematuria and anemia.[orpha.net]
    • Typical signs and symptoms of Wilms tumor include the following: a painless, palpable abdominal mass loss of appetite abdominal pain fever nausea and vomiting blood in the urine (in about 20% of cases) high blood pressure in some cases (especially if[en.wikipedia.org]
    • Cancer Cancer Kidney conditions Urogenital tumors Abdominal conditions Urinary system conditions Children's health conditions Symptoms of Nephroblastoma (Wilms' tumor) Some of the symptoms of Nephroblastoma incude: Abdominal mass Abdominal swelling Fever[rightdiagnosis.com]
    Weight Loss
    • Later, symptoms may indicate fever, distortion of the kidney mass, evidence of secondary tumours elsewhere in the body, abdominal and flank pain, weight loss, nausea, loss of appetite, and vomiting.[britannica.com]
    • Your child is being treated for this condition and symptoms get worse or new symptoms develop, mainly cough, chest pain, weight loss, or persistent fevers.[nlm.nih.gov]
    • Malaise, fever, weight loss, and a varicocele may be present.[oncologynurseadvisor.com]
    • In dogs, diagnosis is usually established later in life, with patients presenting with weight loss, lumbar pain.[vetbook.org]
    • loss Bone pain   history and physical Urinalysis: blood, sugar, protein and bacteria infection  Blood tests:-  Radiology studies    Ultrasound CT scan X-ray of the abdomen  Biopsy Treatment  In general, this type of cancer is curable.[scribd.com]
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  • Immune System
    Splenomegaly
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  • gastrointestinal
    Abdominal Distension
    Abdominal Mass
    • Clinical presentation is typically with a painless upper quadrant abdominal mass.[radiopaedia.org]
    • ) Rarely, anaemia or polycythaemia (secondary to production of erythropoeitin by the tumour) On examination, the most common finding is an abdominal mass.[ozradonc.wikidot.com]
    • Most common abdominal malignancy of children Both kidneys affected synchronously or metachronously in 5-10% Peak incidence at 2.5-3 years More common in blacks than whites with an approximately equal gender distribution Clinical Findings Asymptomatic abdominal[learningradiology.com]
    • Nephroblastoma, or Wilms’ Tumor, is the most common solid abdominal tumor in childhood and usually appears as a painless abdominal mass in an otherwise well child.[childrens.memorialhermann.org]
    Abdominal Pain
    • pain Nausea See full list of 23 symptoms of Nephroblastoma (Wilms' tumor) Treatments for Nephroblastoma (Wilms' tumor) Treatments for Nephroblastoma (Wilms' tumor) include: Hospitalization Surgery Surgical kidney removal - especially if only one kidney[rightdiagnosis.com]
    • pain Hematuria Hypertension Urinary tract infection Varicocele Aniridia Hemihypertrophy Beckwith-Wiedemann Syndrome Imaging Findings Ultrasound is frequently the first study Echogenic mass CT is useful in determining extent, lymph nodes and liver metastases[learningradiology.com]
    • Patients sometimes experience abdominal pain (around 10% of cases), hypertension, fever (20% of cases), hematuria and anemia.[orpha.net]
    • Symptoms may include any of the following: Abdominal pain Abnormal urine color Constipation Fever General discomfort or uneasiness ( malaise ) High blood pressure Increased growth on only one side of the body Loss of appetite Nausea and vomiting Swelling[nlm.nih.gov]
    • Severe acute abdominal pain is sometimes caused by spontaneous or traumatic rupture of the tumor.[link.springer.com]
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  • cardiovascular
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  • urogenital
    Hematuria
    • […] is uncommon urine sediment cytology is rarely diagnostic for renal tumors Blood Tests hematology and serum biochemistry findings are usually normal or non-specific mild-to-moderate normochromic, normocytic anemia can be caused by either hematuria or[vsso.org]
    • […] children Both kidneys affected synchronously or metachronously in 5-10% Peak incidence at 2.5-3 years More common in blacks than whites with an approximately equal gender distribution Clinical Findings Asymptomatic abdominal mass most common Abdominal pain Hematuria[learningradiology.com]
    • Patients sometimes experience abdominal pain (around 10% of cases), hypertension, fever (20% of cases), hematuria and anemia.[orpha.net]
    • Constipation Fever General discomfort or uneasiness ( malaise ) High blood pressure Increased growth on only one side of the body Loss of appetite Nausea and vomiting Swelling in the abdomen ( abdominal hernia or mass ) Sweating (at night) Blood in urine (hematuria[nlm.nih.gov]
    • […] for Nephroblastoma Treatment of Nephroblastoma: For more treatment information about Nephroblastoma, see treatment of Wilms' tumor (Nephroblastoma) Nephroblastoma: Related Disease Topics These medical disease topics may be related to Nephroblastoma: hematuria[rightdiagnosis.com]
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  • Eyes
    Aniridia
    • Sporadic aniridia mutations may affect the WT1 region adjacent to the AN2 aniridia region, causing a kidney cancer called nephroblastoma (Wilms tumor).[diki.pl]
    • The association of nephroblastoma, aniridia, genitourinary malformation, and mental retardation is called WAGR syndrome.[link.springer.com]
    • 5-10% Peak incidence at 2.5-3 years More common in blacks than whites with an approximately equal gender distribution Clinical Findings Asymptomatic abdominal mass most common Abdominal pain Hematuria Hypertension Urinary tract infection Varicocele Aniridia[learningradiology.com]
    • Etiology Nephroblastoma is sporadic in 99% of cases and, among these cases, 10% are associated with congenital anomalies (aniridia, hemihypertrophy, genitourinary defects) or form part of specific syndromes (Beckwith-Wiedemann, Denys-Drash, WAGR or Perlman[orpha.net]
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  • Workup

    Any child with an abdominal mass warrants a full workup consisting of a patient and family history, complete physical exam, and the appropriate studies.

    Laboratory tests

    The clinician must obtain a complete blood count (CBC), complete metabolic panel (CMP) including renal function tests, and a coagulation panel. Urinalysis is also important.

    Cytogenetic testing

    Chromosomal analysis for associated syndromes is indicated.

    Imaging

    Ultrasonography of the kidneys is the initial diagnostic modality since it is widely available, inexpensive, and safe. Renal ultrasonography determines the location and characteristic of the mass as it differentiates solid from cystic lesions [5]. This study also detects thrombi in the inferior vena cava (IVC).

    Abdominal computed tomography (CT) scan provides critical details regarding the tumor's origin, vascular and lymph nodal invasion, bilateral renal involvement, and metastatic disease [7]. Moreover, CT with contrast enhancement demonstrates a greater sensitivity for vascular involvement [8].

    Experts advocate that contrast-enhanced magnetic resonance imaging (MRI) is the preferred diagnostic study [3]. This modality exhibits the most sensitivity for ascertaining caval patency and invasion. Generally, a nephroblastoma has low signal intensity on a T1 weighted image and high intensity on T2 [9].

    Chest radiography and CT scan are performed to investigate lung metastasis.

    Histology

    The diagnosis is confirmed by histologic analysis of the tumor, which is characterized by epithelial, stromal, and blastemal embryonic elements [4]. Very importantly, histology also determines the prognosis [10].

    Laboratory

    Serum
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  • Imaging

    X-ray
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  • Treatment

    Prognosis

    Complications

    Ambiguous Genitalia
    • Wilms tumor has been associated with a number of genetic syndromes or birth defects which include : WAGR (a constellation of syndromes including Wilms tumor, Aniridia, ambiguous Genitalia, and mental Retardation); Beckwith-Wiedemann Syndrome (a condition[surgery4children.com]
    • genitalia and progressive renal failure in genotypic males, associated with an abnormal WT1 gene on 11p13).[cancerimagingjournal.biomedcentral.com]
    Ascites
    • VOD is primarily a clinical diagnosis characterized by hepatomegaly or pain in the right upper quadrant, jaundice, ascites, and unexplained weight gain.[emedicine.medscape.com]
    • Infiltration of adjacent organs/structures •Pericardium •Diaphragm •Kidney •Liver •Duodeno-pancreatic block •Mesentery Conditions to be recorded, but not considered IDRFs •Multifocal primary tumours •Pleural effusion, with or without malignant cells •Ascites[cancerimagingjournal.biomedcentral.com]
    • Ascites beyond the cul-de-sac is most predictive of preoperative Wilms tumor rupture, irrespective of attenuation.[dana-farber.org]
    Hypertension
    • In addition to this paediatric renal tumour, the coexistence of juvenile arterial hypertension suggests an associated vascular defect.[medicaljournals.se]
    • […] vacillate tabernaemontana divaricate petty bourgeoisie mail telugu oblivious hessian balkans nephroblastoma nephroblastoma - Dictionary definition and meaning for word nephroblastoma Definition (noun) malignant renal tumor of young children characterized by hypertension[mnemonicdictionary.com]
    • Wilms' ... more Nephroblastoma: See also: Wilms' tumor : » Introduction: Wilms' tumor » Symptoms of Wilms' tumor » Treatments for Wilms' tumor Nephroblastoma: malignant renal tumor of young children characterized by hypertension and blood in the urine[rightdiagnosis.com]
    • […] kidneys affected synchronously or metachronously in 5-10% Peak incidence at 2.5-3 years More common in blacks than whites with an approximately equal gender distribution Clinical Findings Asymptomatic abdominal mass most common Abdominal pain Hematuria Hypertension[learningradiology.com]
    • Patients sometimes experience abdominal pain (around 10% of cases), hypertension, fever (20% of cases), hematuria and anemia.[orpha.net]
    Neuroblastoma
    • Signs & Symptoms  Signs and symptoms of neuroblastoma vary depending on what part of the body is affected.   Neuroblastoma in the abdomen Neuroblastoma in the chest Cont…  Neuroblastoma in the abdomen :  • Neuroblastoma in the chest :• Wheezing[scribd.com]
    • Tumor composed predominantly of blastema may be difficult to differentiate from neuroblastoma.[pathpedia.com]
    • : see neuroblastoma vs.[radiopaedia.org]
    • MIBG-Scintigraphy: if neuroblastoma is possible.[urology-textbook.com]
    Polycythemia
    • […] humans, mutation of the tumor suppressor gene WT1 (Wilm’s tumor gene located on chromosome 11p13) is a causative factor TYPICAL CLINICAL FINDINGS: · Signs may be absent · Abdominal enlargement and hematuria have been reported · Paraneoplastic syndrome : Polycythemia[askjpc.org]
    Varicocele
    • […] metachronously in 5-10% Peak incidence at 2.5-3 years More common in blacks than whites with an approximately equal gender distribution Clinical Findings Asymptomatic abdominal mass most common Abdominal pain Hematuria Hypertension Urinary tract infection Varicocele[learningradiology.com]
    • Malaise, fever, weight loss, and a varicocele may be present.[oncologynurseadvisor.com]
    • Vomiting, fever or varicoceles are rarely present.[urology-textbook.com]
    • Abdominal pain due to liver metastasis, prominent abdominal wall vessels, or varicocele due to inferior vena cava obstruction.[dana-farber.org]
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  • Etiology

    Epidemiology

    Sex distribution
    Age distribution

    Pathophysiology

    Prevention

    Summary

    Patient Information

    Self-assessment

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    References

    1. Breslow N, Olshan A, Beckwith JB, et al. Epidemiology of Wilms tumor. Med Pediatr Oncol. 1993;21(3):172–81.
    2. Pastore G, Znaor A, Spreafico F, et al. Malignant renal tumours incidence and survival in European children (1978–1997): report from the Automated Childhood Cancer Information System project. Eur J Cancer. 2006;42(13):2103–2114.
    3. Dumba M, Jawad N, McHugh K. Neuroblastoma and nephroblastoma: a radiological review. Cancer Imaging. 2015;15(1):5.
    4. Lonergan GJ, Martinez-Leon MI, Agrons GA, Montemarano H, Suarez ES. Nephrogenic rests, nephroblastomatosis, and associated lesions of the kidney. Radiographics. 1998;18(4):947–68.
    5. Davidoff AM. Wilms tumor. Adv Pediatr. 2012;59(1):247–67.
    6. Dahnert W. Urogenital tract. In: Radiology Review Manual. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2011: 895-1012.
    7. Refaie HD, Sarhan M, Hafez A. Role of CT in assessment of unresectable Wilms' tumor response after preoperative chemotherapy in pediatrics. ScientificWorldJournal. 2008; 8:661-69.
    8. Khanna G, Rosen N, Anderson JR, et al. Evaluation of diagnostic performance of CT for detection of tumour thrombus in children with Wilms tumour: a report from the Children’s Oncology Group. Pediatr Blood Cancer. 2012;58(4):551–55.
    9. Lowe HL, Isuani BH, Heller RM, Stein SM, Johnson JE, Navarro OM, et al. Pediatric Renal Masses: Wilms Tumor and Beyond. Radiographics. 2000;20(6):1585–1603.
    10. Beckwith JB, Palmer NF. Histopathology and prognosis of Wilms tumor results from the first National Wilms’ Tumor Study. Cancer. 1978;41(5):1937–48.

    • A 32 Days Old Baby with Nephroblastoma-A Case Report - MM Uddin, F Bhuiya, S Sharmin - Bangladesh Oncology , 2009 - banglajol.info
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    • Adult nephroblastoma induced erythrocytosis - P Rigatti, F Montorsi, G Guazzoni - journal of urology , 1990 - informahealthcare.com
    • Blood Precipitate Associated With Intra‐Abdominal Carboxymethylcellulose Administration - MJ Burkhard, G Baxter, MA Thrall - Veterinary Clinical , 2009 - Wiley Online Library
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    • Beckwith-Wiedemann syndrome: development of nephroblastoma during the surveillance period - EM Azouz, EJ Larson, J Patel, MT Gyepes - Pediatric radiology, 1990 - Springer
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    • A mouse model of the aniridia-Wilms tumor deletion syndrome. - T Glaser, J Lane, D Housman - Science (New York, NY), 1990 - ukpmc.ac.uk
    • Asymptomatic inferior vena cava abnormalities in three children withend‐stage renal disease: Risk factors and screening guidelines for pretransplant diagnosis - SE Thomas, RO Hickman, D Tapper - Pediatric , 2001 - Wiley Online Library
    • Clinical presentation and treatment outcome in children with nephroblastoma in Kenya - FK Abdallah, WM Macharia - East African medical journal, 2004 - ajol.info
    • Childhood renal tumours with intravascular extension - I Mushtaq, R Carachi, G Roy - British journal of urology, 1996 - Wiley Online Library
    • Familial occurrence of Wilms' tumor: nephroblastoma in one of monozygous twins and in another sibling. - RC Juberg, EC St Martin - American journal of human , 1975 - ncbi.nlm.nih.gov
    • ARKADI M RYWLIN INTERNATIONAL PATHOLOGY SLIDE SEMINAR CLUB - C DIRECTOR, DRS SUSTER, DRDV SPAGNOLO - amr-seminar.org
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    • Bipolaris spicifera Dual Systemic Mycosis Caused by - BJ Waurzyniak, JP Hoover, KD Clinkenbeard - Vet , 1992 - researchgate.net
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    • Carcinogenicity bioassays of vinyl chloride: I. Research plan and early results - C Maltoni, G Lefemine - Environmental Research, 1974 - Elsevier
    • Aniridia, male pseudohermaphroditism, gonadoblastoma, mental retardation, and del 11p13 - C Turleau, J Grouchy, JL Dufier, P Hoang - Human genetics, 1981 - Springer
    • Adult nephroblastoma with glomerular intracapillary deposits of IgM in the contralateral kidney. - S Banik, CK Heffernan, M Rossi - The Journal of urology, 1985 - ukpmc.ac.uk
    • BILATERAL NEPHROBLASTOMA (WILMS'TUMOR) - LW Martin, RJ Kloecker - Pediatrics, 1961 - Am Acad Pediatrics
    • Botryoid Wilms tumor: case report and review of literature - A Honda, M Shima, S Onoe, M Hanada, T Nagai - Pediatric , 2000 - Springer
    • A syndrome of pseudohermaphroditism, Wilms' tumor, hypertension, and degenerative renal disease - A Drash, F Sherman, WH Hartmann - The Journal of pediatrics, 1970 - Elsevier
    • Anemia in children with cancer is associated with decreased erythropoietic activity and not with inadequate erythropoietin production - F Corazza, Y Beguin, P Bergmann - , 1998 - bloodjournal.hematologylibrary.org
    • Aniridia and wilms's tumour (nephroblastoma). - TF Mackintosh, TG Girdwood, DJ Parker - British Journal of , 1968 - bjo.bmj.com
    • Alagille syndrome and nephroblastoma: Unusual coincidence of two rare disorders - F Bourdeaut, A Guiochon‐Mantel - Pediatric Blood & , 2008 - Wiley Online Library
    • Chylous ascites following surgical treatment for Wilms tumor - AC Weiser, BW Lindgren, ML Ritchey, I Franco - The Journal of urology, 2003 - Elsevier
    • Adult Wilms' Tumor Associated with Polycythemia: a Case Report - CW Chen, SP Huang, YC Li, YH Chou - JOURNAL OF MEDICAL , 2001 - ukpmc.ac.uk
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    Media References

    1. Wilms tumor, Public Domain

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