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    Wilms Tumor

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    Prasetya Ismail Permadi
    Wilms Tumor (WT) is the most common intra-abdominal malignant tumor in children, with a mean age of onset of 3.5 years. WT typically presents as a large abdominal or flank mass with abdominal pain, hematuria, and fever. Initial evaluation includes urinalysis, imaging tests like ultrasound and CT scans to determine if the tumor is confined to the kidney or has metastasized to other organs like the lungs or liver. Treatment involves primary nephrectomy followed by chemotherapy or radiation depending on the tumor stage and presence of poor prognostic features like anaplasia or sarcomatous degeneration. WT can be sporadic, familial, or associated with genetic syndromes like WAGR

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    Wilms Tumor

    Uploaded by

    Prasetya Ismail Permadi
    46 views3 pages
    Wilms Tumor (WT) is the most common intra-abdominal malignant tumor in children, with a mean age of onset of 3.5 years. WT typically presents as a large abdominal or flank mass with abdominal pain, hematuria, and fever. Initial evaluation includes urinalysis, imaging tests like ultrasound and CT scans to determine if the tumor is confined to the kidney or has metastasized to other organs like the lungs or liver. Treatment involves primary nephrectomy followed by chemotherapy or radiation depending on the tumor stage and presence of poor prognostic features like anaplasia or sarcomatous degeneration. WT can be sporadic, familial, or associated with genetic syndromes like WAGR

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    Wilms Tumor (WT) is the most common intra-abdominal malignant tumor in children, with a mean age of onset of 3.5 years. WT typically presents as a large abdominal or flank mass with abdominal pain, hematuria, and fever. Initial evaluation includes urinalysis, imaging tests like ultrasound and CT scans to determine if the tumor is confined to the kidney or has metastasized to other organs like the lungs or liver. Treatment involves primary nephrectomy followed by chemotherapy or radiation depending on the tumor stage and presence of poor prognostic features like anaplasia or sarcomatous degeneration. WT can be sporadic, familial, or associated with genetic syndromes like WAGR

    Copyright:

    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    46 views3 pages

    Wilms Tumor

    Uploaded by

    Prasetya Ismail Permadi
    Wilms Tumor (WT) is the most common intra-abdominal malignant tumor in children, with a mean age of onset of 3.5 years. WT typically presents as a large abdominal or flank mass with abdominal pain, hematuria, and fever. Initial evaluation includes urinalysis, imaging tests like ultrasound and CT scans to determine if the tumor is confined to the kidney or has metastasized to other organs like the lungs or liver. Treatment involves primary nephrectomy followed by chemotherapy or radiation depending on the tumor stage and presence of poor prognostic features like anaplasia or sarcomatous degeneration. WT can be sporadic, familial, or associated with genetic syndromes like WAGR

    Copyright:

    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
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    Wilms Tumor (WT)

    the most common intra-abdominal malignant tumor in children


    incidence mean age = 3.5 years.
    WT present as a large abdominal or flank mass with abdominal pain, hematuria, and
    fever.
    Other presentations =malaise, weight loss, anemia, left varicocele (obstructed left renal
    vein), and hypertension.
    Initial evaluation :
    urinalysis
    Chest-X-rays
    Abdominal x ray
    IVP = The presence of a solid, intrarenal mass causing intrinsic distortion of the calyceal
    collecting system is diagnostic of Wilms tumor.
    Ultrasound
    Computed Tomography.
    Doppler sonography of the renal vein and inferior vena cava can exclude venous tumor
    involvement.
    Metastasis occurs commonly to lungs and liver.
    Operation is both for treatment and staging
    primary nephrectomy is done for all
    further therapy is based on the surgical and pathological findings.
    transverse incision,
    radical nephrectomy, explore the contralateral kidney
    avoide tumor spillage,
    take biopsy of lymph nodes. Nodes are taken to determine extent of disease.

    WT staging consists of:


    Stage I- tumor limited to kidney and completely resected.
    Stage II- tumor extends beyond the kidney but is completely excised.
    Stage III- residual non-hematogenous tumor confined to the abdomen.
    Stage IV- hematogenous metastasis.
    Stage V- bilateral tumors.

    chemotherapy or radiotherapy depends on staging and histology


    Non-favorable histologic characteristics are:
    anaplasia (three times enlarged nucleus, hyperchromatism, mitosis),
    sarcomatous degeneration.
    Disease-free survival is 95% for Stage I and 80% for others
    Prognosis is poor for those children with lymph nodes, lung and liver metastasis.

    Occurance
    sporadic (95%),
    familial (1-2%)
    associated with a syndrome (2%).
    syndromes predisposing to WT are
    WAGR (Wilms, aniridia, genitourinary malformation and mental retardation),
    Beckwith-Wiedemann Syndrome (gigantism, macroglossia, pancreas cell hyperplasia,
    BWS), and Denys-Drash Syndrome (male pseudohermaphrodite, nephropathy and
    Wilms tumor, DDS).
    Sporadic WT may be associated with hemihypertrophy or genitourinary malformations
    such as hypospadia, cryptorchidism and renal fusion.
    Bilateral kidney tumors are seen in 5-10% of cases.
    Routine abdominal ultrasound screening every six months up to the age of eight years is
    recommended for children at high risk for developing WT such as the above-mentioned
    syndromes.

    It was originally thought that WT developed after the two-hit mutational model
    developed for retinoblastoma: When the first mutation occurs before the union the sperm
    and egg (constitutional or germline mutation) the tumor is heritable and individuals are at
    risk for multiple tumors.
    Nonhereditary WT develops as the result of two-postzygotic mutations (somatic) in a
    single cell.
    several genes' mutations are involved in the WT pathogenesis.

    Patients with WT and a diploid DNA content (indicating low proliferation) have
    excellent prognosis.
    Hyperdiploidy (high mitotic activity) is a poor prognostic feature
    advantage of genetic testing is that children with syndromes known to be at high risk
    for developing WT can undergo screening

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