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.


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.

urogenital
Hematuria
  • […] 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]
  • Common manifestations of nephroblastoma include an abdominal mass, abdominal pain, macroscopic or microscopic hematuria, fever, and anorexia.[link.springer.com]
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  • Eyes
    Aniridia
    • In aniridia (an-ih-RID-e-uh), the iris — the colored portion of the eye — forms only partially or not at all.[mayoclinic.org]
    • In some children the tumour is associated with an abnormality of chromosome number 13; in these cases other features, such as absence of the iris in the eye ( see aniridia) and hemihypertrophy, are present.[oxfordreference.com]
    • 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]
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  • cardiovascular
    Hypertension
    • In addition to this paediatric renal tumour, the coexistence of juvenile arterial hypertension suggests an associated vascular defect.[medicaljournals.se]
    • […] vigorous isopropyl alcohol irredeemable uncomparable autogenous dasht-e-kavir hysterically 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]
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  • gastrointestinal
    Abdominal Mass
    • ) 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]
    • 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]
    • 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]
    • An abdominal mass (unilateral in most cases) is frequently present.[orpha.net]
    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]
    Abdominal Distension
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  • Entire body system
    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]
    • Malaise, fever, weight loss, and a varicocele may be present.[oncologynurseadvisor.com]
    • 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]
    Anemia
    • Patients sometimes experience abdominal pain (around 10% of cases), hypertension, fever (20% of cases), hematuria and anemia.[orpha.net]
    • […] 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]
    • Fanconi anemia D1: generally increased risk of tumors, 20% risk of Wilms tumor.[urology-textbook.com]
    • Rarely, a Wilms' tumor is diagnosed after there has been bleeding into the tumor, resulting in sudden swelling of the abdomen and a low red blood cell count (anemia).[encyclopedia.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]
    • Systemic symptoms such as weight loss, malaise, weakness, and fever are indicative of advanced disease [5] [6] .[ijcasereportsandimages.com]
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  • 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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  • Immune System
    Splenomegaly
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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

    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]
    • Malaise, fever, weight loss, and a varicocele may be present.[oncologynurseadvisor.com]
    • 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]
    Hypertension
    • In addition to this paediatric renal tumour, the coexistence of juvenile arterial hypertension suggests an associated vascular defect.[medicaljournals.se]
    • […] vigorous isopropyl alcohol irredeemable uncomparable autogenous dasht-e-kavir hysterically 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]
    Anemia
    • Patients sometimes experience abdominal pain (around 10% of cases), hypertension, fever (20% of cases), hematuria and anemia.[orpha.net]
    • […] 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]
    • Fanconi anemia D1: generally increased risk of tumors, 20% risk of Wilms tumor.[urology-textbook.com]
    • Rarely, a Wilms' tumor is diagnosed after there has been bleeding into the tumor, resulting in sudden swelling of the abdomen and a low red blood cell count (anemia).[encyclopedia.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]
    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]
    Neuroblastoma
    • Tumor composed predominantly of blastema may be difficult to differentiate from neuroblastoma.[pathpedia.com]
    • MIBG-Scintigraphy: if neuroblastoma is possible.[urology-textbook.com]
    • […] with compressed normal renal tissue May be partially cystic from necrosis Curvilinear or punctate calcifications Invasion of IVC by tumor Hypervascular tumors On MRI, tumor tends to have decreased signal on T1 and increased on T2 Differential Diagnosis Neuroblastoma[learningradiology.com]
    • Differential diagnosis Differential diagnoses include other renal tumors in children such as mesoblastic nephroma (especially in infants), clear cell sarcoma, neuroblastoma (extremely rare in the kidney but may invade the kidney by contiguity), rhabdoid[orpha.net]
    • […] which are a small part pediatric solid tumours, include clear cell sarcoma of the kidney, mesoblastic nephroma, cystic partially differentiated nephroblastoma , malignant rhabdoid tumour, renal cell carcinoma (RCC), renal medullary carcinoma, intrarenal neuroblastoma[thefreedictionary.com]
    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]

    Etiology

    Epidemiology

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    References

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    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.

    1. 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
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    5. A syndrome of pseudohermaphroditism, Wilms' tumor, hypertension, and degenerative renal disease - A Drash, F Sherman, WH Hartmann - The Journal of pediatrics, 1970 - Elsevier
    6. 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
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    8. Carcinogenicity bioassays of vinyl chloride: I. Research plan and early results - C Maltoni, G Lefemine - Environmental Research, 1974 - Elsevier
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    12. A renal tumour of the nephroblastoma type inPraomys (Mastomys) natalensis - P Jobard, G Delbarre, B Delbarre, E Aron - Cellular and Molecular , 1974 - Springer
    13. 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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    24. Blood Precipitate Associated With Intra‐Abdominal Carboxymethylcellulose Administration - MJ Burkhard, G Baxter, MA Thrall - Veterinary Clinical , 2009 - Wiley Online Library
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    Media References

    1. Wilms tumor, Public Domain

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