Wilms Tumor Overview
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Questions and Answers

What is the frequency of follow-up visits for a patient with Wilms tumor after the first two years of diagnosis?

  • Every 3 months
  • Every 6 months
  • Once every 2 years (correct)
  • Once a year
  • Which of the following is NOT typically monitored immediately postoperatively in a patient with Wilms tumor?

  • Blood pressure
  • Blood glucose
  • Pulse (correct)
  • Electrolytes
  • During follow-up visits for Wilms tumor, a thorough physical examination may include which of the following?

  • Assessment of dental health
  • Assessment of lung function
  • Evaluation of the musculoskeletal system
  • Evaluation of the thyroid gland (correct)
  • Which of the following complications is associated with Vincristine therapy for Wilms tumor?

    <p>Peripheral neuropathy (B)</p> Signup and view all the answers

    What is the primary reason for examining the abdomen during follow-up visits for a patient with Wilms tumor?

    <p>To detect any local tumor recurrence (D)</p> Signup and view all the answers

    What is the initial presentation of renal disease in newborns and infants that can progress to nephrotic syndrome?

    <p>Proteinuria (B)</p> Signup and view all the answers

    Which of the following is NOT a common clinical feature associated with Beckwith-Wiedemann syndrome?

    <p>Cryptorchidism (D)</p> Signup and view all the answers

    A finding of perilobar nephrogenic rests in children less than one year old significantly increases their risk of developing which condition?

    <p>Contralateral Wilms tumor (A)</p> Signup and view all the answers

    Which histological component of a 'favorable' Wilms tumor is considered the most undifferentiated and potentially malignant?

    <p>Blastema (B)</p> Signup and view all the answers

    What percentage of individuals with the specified renal disease will eventually develop Wilms tumor?

    <p>90% (A)</p> Signup and view all the answers

    In boys with Wilms tumor, which of the listed presentations is commonly found?

    <p>Hypospadias (D)</p> Signup and view all the answers

    What is characteristic of the epithelial component in a 'favorable' Wilms tumor?

    <p>Variations from primitive to differentiated structures (A)</p> Signup and view all the answers

    Which of these is NOT a syndrome listed as being sometimes associated with wilms?

    <p>Beckwith-Wiedemann syndrome (C)</p> Signup and view all the answers

    What is the most common type of cancer found in the kidneys of children?

    <p>Wilms tumor (A)</p> Signup and view all the answers

    At what age is Wilms tumor most commonly diagnosed?

    <p>Children younger than 5 years old (B)</p> Signup and view all the answers

    What is the fourth most common cancer overall that affects children?

    <p>Wilms tumor (C)</p> Signup and view all the answers

    Which of the following genetic alterations has been least associated with the development of Wilms tumor, according to the text?

    <p>MYNC (A)</p> Signup and view all the answers

    Which physician is credited with first describing Wilms tumor?

    <p>Dr. Max Wilms (D)</p> Signup and view all the answers

    What is thought to be the primary cause of Wilms tumor?

    <p>Genetic alterations affecting embryological development (B)</p> Signup and view all the answers

    Which genetic marker, when altered, has been linked to a poorer prognosis in Wilms tumor?

    <p>TP53 (B)</p> Signup and view all the answers

    The loss of heterozygosity at which chromosome is associated with a poorer prognosis?

    <p>Chromosome 16q (B)</p> Signup and view all the answers

    What is the survival rate range for patients with favorable histology associated with Wilms tumor?

    <p>99% to 86% (B)</p> Signup and view all the answers

    Which characteristic is associated with a poorer prognosis in Wilms tumor?

    <p>Anaplastic histology in stage II to IV (C)</p> Signup and view all the answers

    What risk increases as a long-term effect of radiation therapy for treating Wilms tumor?

    <p>Secondary malignancies (D)</p> Signup and view all the answers

    Which of the following treatment complications is associated with chemotherapy for Wilms tumor?

    <p>Peripheral neuropathy (A)</p> Signup and view all the answers

    What is the initial therapy recommended for patients with Von Willebrand's disease related to Wilms tumor?

    <p>DDAVP (B)</p> Signup and view all the answers

    What aspect of survival does the report from the Children's Oncology Group primarily focus on?

    <p>Effect of surveillance imaging modality on survival after recurrence (D)</p> Signup and view all the answers

    Which type of Wilms tumor patient outcome is discussed in Iaboni et al.'s report?

    <p>Wilms tumor patients with bone metastasis (C)</p> Signup and view all the answers

    What cytogenetic abnormalities are associated with poorer prognosis in Wilms tumor?

    <p>1p and 16q deletions (B)</p> Signup and view all the answers

    Which symptom is most commonly associated with the initial presentation of Wilms tumor?

    <p>Abdominal pain (A)</p> Signup and view all the answers

    What histological feature defines anaplastic cells in Wilms tumor?

    <p>Hyperchromatic, pleomorphic nuclei (D)</p> Signup and view all the answers

    What is a common effect seen in Wilms tumor patients post-nephrectomy?

    <p>Normalization of hypertension (C)</p> Signup and view all the answers

    Which of the following evaluations is specifically indicated when Wilms tumor tissue is available?

    <p>Cytogenetic studies for 1p and 16q deletions (A)</p> Signup and view all the answers

    What is a complication in Wilms tumor patients that may arise due to the tumor's interaction with the urinary system?

    <p>Urinary tract infections (A)</p> Signup and view all the answers

    Which of the following is NOT a common symptom of Wilms tumor?

    <p>Severe headaches (A)</p> Signup and view all the answers

    What type of differentiation can be seen in neoplastic stroma of Wilms tumors after preoperative chemotherapy?

    <p>Heterologous differentiation including muscle and fat (D)</p> Signup and view all the answers

    Flashcards

    What is Wilms tumor?

    Wilms tumor, also known as nephroblastoma, is a type of cancer that develops in the kidneys of children. It is the most common kidney cancer in children and the fourth most common overall.

    What causes Wilms tumor?

    The exact cause of Wilms tumor is unknown, but it's believed to arise due to genetic alterations impacting the normal development of the urinary system.

    At what age is Wilms tumor most common?

    Wilms tumor is most commonly diagnosed in children under five years old, making it a primarily childhood cancer.

    Who is the tumor named after?

    The tumor is named after German physician Dr. Max Wilms, who first described it in 1899.

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    What are the common symptoms of Wilms tumor?

    Common symptoms include an abdominal mass, pain, and blood in the urine. These are the most frequent signs of Wilms tumor.

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    How is Wilms tumor treated?

    Treatment usually involves a combination of surgery, chemotherapy, and radiation therapy. These treatments are chosen based on the stage and characteristics of the tumor.

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    What are some genetic markers associated with Wilms tumor?

    Genetic markers linked to Wilms tumor include WT1, CTNNB1, and WTX. These genes are involved in the developing urinary system.

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    What's the prognosis for Wilms tumor?

    The prognosis of Wilms tumor is generally good, especially with early detection and treatment. However, factors like stage and specific gene mutations can influence the outcome.

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    Renal disease in newborns and infants

    A type of renal disease that starts with protein in the urine and can progress to kidney failure.

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    Postoperative Monitoring

    Immediately after surgery, patients must have their blood pressure, electrolytes, temperature, and blood sugar levels monitored.

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    Wilms tumor

    A type of cancer that affects the kidneys, often developing in children.

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    Wilms Tumor Follow-Up Schedule (Initial)

    Patients with Wilms tumor usually have follow-up visits every 3 months for the first 2 years after diagnosis.

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    Beckwith-Wiedemann syndrome

    A syndrome characterized by a range of physical abnormalities, including enlarged kidneys, a large tongue, and an extra body part.

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    Favorable Wilms tumor histology

    A specific type of Wilms tumor that has a generally better outcome.

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    Wilms Tumor Follow-Up Schedule (Later)

    After 2 years, Wilms tumor follow-up visits become less frequent, occurring every 6 months for another 2 years.

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    Wilms Tumor Follow-Up Tests

    During follow-up visits for Wilms tumor, doctors might check for signs of tumor recurrence or spread by feeling the abdomen, listening to the lungs, and examining relevant areas.

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    Blastema in Wilms tumor

    The most primitive and potentially aggressive component of a Wilms tumor.

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    Epithelial component of Wilms tumor

    The part of a Wilms tumor that contains developing kidney structures, resembling different stages of kidney formation.

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    Wilms Tumor Follow-Up Complications

    Monitoring for side effects from treatment, such as peripheral neuropathy (nerve damage) from Vincristine and liver problems from radiation therapy, is important during follow-up.

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    Stromal component of Wilms tumor

    The connective tissue part of a Wilms tumor, which can be either dense or loose.

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    Perilobar nephrogenic rests

    The presence of abnormal kidney tissue that can lead to a higher risk of developing Wilms tumor in the other kidney.

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    Unfavorable Histology Wilms Tumor

    A type of Wilms tumor with a poorer prognosis due to its increased cell abnormality.

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    Anaplasia

    The abnormal cell growth and structure found in "Unfavorable Histology" Wilms tumors.

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    Loss of Heterozygosity (LOH) at 1p and 16q

    The loss of genetic material at specific locations on chromosomes 1p and 16q, which can indicate a worse prognosis in Wilms tumors.

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    Heterologous Differentiation

    Presence of different types of tissue cells, such as muscle, fat, or cartilage, in a Wilms tumor, often seen after chemotherapy.

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    Abdominal Mass

    The most common initial symptom of Wilms tumor, often presenting as a painless swelling.

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    Gross Hematuria

    Blood in the urine, which can be a symptom of Wilms tumor.

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    Hypertension in Wilms Tumor

    Elevated blood pressure that occurs in about a third of Wilms tumor patients.

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    Renal Function Tests

    Lab tests that assess the function of kidneys, including blood chemistry and urine analysis.

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    Anaplastic Histology in Wilms Tumor

    Anaplastic histology is associated with a poorer prognosis in stage II to IV Wilms tumors.

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    Diffuse vs. Focal Anaplasia

    Diffuse anaplasia indicates a more widespread abnormal cell development, leading to a worse prognosis compared to focal anaplasia.

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    LOH and TP53 in Wilms Tumor

    Loss of heterozygosity (LOH) at specific chromosome locations and the presence of the TP53 gene mutation contribute to a poorer prognosis.

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    Radiation Therapy and Secondary Cancers

    Wilms tumor patients often have increased bone, breast, colon, and thyroid cancer risk due to radiation therapy.

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    Chemotherapy and Secondary Side Effects

    Chemotherapy for Wilms tumor may lead to secondary malignancies as well as specific toxicities like hearing loss, heart dysfunction, and nerve issues.

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    What is the COG?

    The Children's Oncology Group (COG) is a large clinical trials group focused on childhood cancers, including Wilms tumor.

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    What are 'National Wilms Tumor Studies'?

    The COG has conducted several national studies (National Wilms Tumor Studies 1-5) to improve understanding and treatment of Wilms tumor.

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    Why are COG studies important?

    The COG studies on Wilms tumor have enrolled a large number of patients, allowing for analysis of various outcomes, including survival rates based on different factors like tumor stage or metastasis.

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    How does imaging impact Wilms tumor treatment?

    A focus of COG research is investigating the impact of different medical imaging techniques on survival rates in patients with Wilms tumor.

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    Why are bone metastasis in Wilms tumor cases studied?

    Wilms tumor patients with bone metastasis have a specific outcome which is studied by the COG.

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    Study Notes

    Wilms Tumor

    • Wilms tumor, also known as nephroblastoma, is the most common pediatric renal cancer, the most common pediatric abdominal cancer, and the fourth most common pediatric cancer overall
    • Typically diagnosed in children under five years old
    • Named after Dr. Max Wilms, who first described it in 1899
    • Exact cause unknown, but believed to be related to genetic alterations during genitourinary tract development.
    • Associated with genetic markers like WT1, CTNNB1, WTX, TP53, and MYNC
    • Only about 1% of Wilms patients have a relative with the disease

    Epidemiology

    • Approximately 650 new cases in the US annually
    • Girls slightly more likely to be affected than boys
    • More common in African and African American children, less common in East Asians
    • 90% diagnosed before age six, median age of diagnosis is 3.5 years
    • 5-year survival rate in the US is 92%

    Clinical Presentation

    • Often presents as an asymptomatic abdominal mass
    • Other symptoms may include abdominal pain, gross hematuria, urinary tract infections, varicocele, hypertension or hypotension (which may normalize after surgery), fever, anemia, dyspnea or tachypnea (if lung metastasis)
    • Symptoms may vary depending on the stage and histology of the tumor

    Differential Diagnosis

    • Clear cell renal sarcoma is the second most common childhood renal tumor
    • Rhabdoid renal tumors are highly malignant and often metastatic at diagnosis; has a poor prognosis
    • Congenital mesoblastic nephroma, usually detected by ultrasound in the first year of life, often associated with hypertension and elevated renin levels
    • Renal cell carcinoma is rare in the pediatric population
    • Neuroblastoma, a risk in post-radiation/chemotherapy patients

    Histopathology

    • Wilms tumors can be categorized as "favorable" or "unfavorable" based on histology
    • "Favorable" histology shows a triphasic pattern of blastema, epithelial, and stromal tissues, typically has better prognosis
    • "Unfavorable" histology shows more anaplasia and has a worse prognosis; characterized by large, hyperchromatic nuclei

    Staging

    • Stage I: Tumor completely contained within the kidney, no spillage outside the renal capsule, no vascular invasion
    • Stage II: Tumor has grown outside the kidney but still removable by surgery and no regional lymph node involvement
    • Stage III: Tumor that cannot be completely removed surgically; may involve regional lymph nodes but not distant lymph nodes; involvement of vital organs
    • Stage IV: Distant metastases (e.g. to lungs, brain, liver, bones or lymph nodes)
    • Stage V: Bilateral involvement of both kidneys

    Treatment and Management

    • Typically involves nephrectomy followed by chemotherapy
    • Chemotherapy is used in cases with larger tumors/extensive spread
    • Combination chemotherapy with vincristine and dactinomycin
    • Postoperative radiation may be used, depending on histology and extent of spread.
    • Monitoring of blood pressure, electrolytes, temperature and blood glucose post-operatively

    Follow-up Care

    • Regular visits with oncologist every 3-24 months depending on stage and histology
    • Includes physical exams, imaging, blood tests (including renal function blood tests, liver function tests), potentially thyroid testing, hearing tests (in patients who received carboplatin)
    • This care continues for several years after diagnosis.

    Complications

    • Recurrence is possible
    • Secondary malignancies from radiation/chemotherapy are a risk
    • Hepatic veno-occlusive disease is possible in patients who receive therapy

    Prognosis

    • Survival rates depend on stage and histology, with better outcomes for early detection, "favorable" histology and early treatment
    • Females with Wilms tumor-aniridia syndrome (WAGR) may have an increased risk of developing gonadoblastoma
    • Factors such as older age, large tumor size or recurrence contribute to a poorer prognosis.

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    Description

    This quiz explores Wilms tumor, the most common form of pediatric renal cancer. It covers its epidemiology, clinical presentation, and genetic associations. Test your knowledge about this important topic in pediatric oncology.

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