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    Tamana Ishan
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    Wilms tumor, also known as nephroblastoma, is the most common primary malignant renal tumor in children. It typically occurs in children between ages 2-3 years old. The vast majority of cases are sporadic, though 1-2% are familial and occur at an earlier age. Genetic syndromes like WAGR, Denys Drash, and Beck Wiedemann increase the risk of developing Wilms tumor. Clinical findings may include an abdominal mass, hematuria, hypertension, abdominal pain, or fever. Diagnosis involves abdominal ultrasound, CT scan, chest x-ray, and biopsy. Treatment involves surgery, chemotherapy, and sometimes radiation therapy. Prognosis is generally good, though anap

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    Wilms tumor, also known as nephroblastoma, is the most common primary malignant renal tumor in children. It typically occurs in children between ages 2-3 years old. The vast majority of cases are sporadic, though 1-2% are familial and occur at an earlier age. Genetic syndromes like WAGR, Denys Drash, and Beck Wiedemann increase the risk of developing Wilms tumor. Clinical findings may include an abdominal mass, hematuria, hypertension, abdominal pain, or fever. Diagnosis involves abdominal ultrasound, CT scan, chest x-ray, and biopsy. Treatment involves surgery, chemotherapy, and sometimes radiation therapy. Prognosis is generally good, though anap

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    1 views15 pages

    Wilms Tomor

    Uploaded by

    Tamana Ishan
    Wilms tumor, also known as nephroblastoma, is the most common primary malignant renal tumor in children. It typically occurs in children between ages 2-3 years old. The vast majority of cases are sporadic, though 1-2% are familial and occur at an earlier age. Genetic syndromes like WAGR, Denys Drash, and Beck Wiedemann increase the risk of developing Wilms tumor. Clinical findings may include an abdominal mass, hematuria, hypertension, abdominal pain, or fever. Diagnosis involves abdominal ultrasound, CT scan, chest x-ray, and biopsy. Treatment involves surgery, chemotherapy, and sometimes radiation therapy. Prognosis is generally good, though anap

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    of 15

    ‫بسم اهلل الرحمن‬

    ‫الرحیم‬
    ‫‪WILMS TUMOR‬‬

    ‫ترتیب کننده‪:‬داکتر حفیظ الله (فرزین) ترینی سال سوم سرویس داخله اطفال‬
    ‫رهنمارنحورمل دوکتور حبیب الرحمن(رحمانی) ترینر متخصص سرویس داخله اطفال‬‫‪:‬‬ ‫استاد‬
    Contents:

    • Wilms tumor
    • Genetic
    • Clinical finding
    • Deferential Diagnosis
    • Diagnose
    • treatment
    WILMS TUMOR or Nephroblastoma

    • Most common primary malignant renal tumor of childhood 6%


    • Peak of incidence at 2-3years of age
    • Most common sites of metastases are the lungs, regional lymph
    node and liver
    Genetic

    Vast majority of wilms tumor sporadic


    1%-2% is familial which occurs at earlier age
    Develop in foci of embryonal kidney tissue called nephrogenic
    rest
    WT1 the most characterized Wilms tumor gene
    Cont..
    • Children with some genetic syndromes are predisposing to
    wilms tumor:
    1. WAGR (wilms tumor, aniridia, genitourinary abnormalities,
    MR)
    2. Denys Drash syndrome ( Renal failure, Mesangeal sclerosis,
    Male hermaphroditism)
    3. Beck wiedemann syndrome( hemi hypertrophy, macroglossia,
    omphalocele, organomegaly)
    Clinical finding
    Asymptomatic
    Abdominal mass
    Hematuria 10-25%
    Hypertension 25%
    Abdominal pain 30%
    Fever 20%
    Anorexia and vomiting
    Anemia
    Thrombocytosis
    Von willebrand factor and factor V11 deficiency
    polycythemia
    Staging for wilms tumor
    Deferential Diagnosis
    • Neuroblastoma
    • Hydronephrosis
    • multicystic kidney disease
    • abdominal lymphoma
    • retroperitoneal rhabdomyosarcoma
    Diagnosis
    Abdominal ultrasound
    CT scan
    Chest x ray
    Bone imaging
    Fine needle aspiration cytology
    Treatment
    • Surgery
    • Radiotherapy
    • Chemotherapy
    Con..
    • <2yr old with tumor <550gr Nephrectomy
    • Vincristine and Actinomycin for 18week at stage 1 and 2
    • Doxorubicin and abdominal radiation for stage 3
    • Cyclophosphamide, Carboplatin and etopside for anaplasia and
    metastatic disease
    • Pulmonary radiation for pulmonary metastasis
    •
    Prognosis
    • Young age, low stage and low weight <550gr are favorable
    prognosis
    • Anaplasia and loss of heterozygosity of 1p or 16q increase the
    risk of recurrence
    Reference
    • GHAI essential pediatric 9th edition
    • Basis of pediatric 9th edition (Pervez Akbar khan)
    • Nelson Textbook of Pediatrics 21st edition
    Thank you

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