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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?
What is the frequency of follow-up visits for a patient with Wilms tumor after the first two years of diagnosis?
Which of the following is NOT typically monitored immediately postoperatively in a patient with Wilms tumor?
Which of the following is NOT typically monitored immediately postoperatively in a patient with Wilms tumor?
During follow-up visits for Wilms tumor, a thorough physical examination may include which of the following?
During follow-up visits for Wilms tumor, a thorough physical examination may include which of the following?
Which of the following complications is associated with Vincristine therapy for Wilms tumor?
Which of the following complications is associated with Vincristine therapy for Wilms tumor?
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What is the primary reason for examining the abdomen during follow-up visits for a patient with Wilms tumor?
What is the primary reason for examining the abdomen during follow-up visits for a patient with Wilms tumor?
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What is the initial presentation of renal disease in newborns and infants that can progress to nephrotic syndrome?
What is the initial presentation of renal disease in newborns and infants that can progress to nephrotic syndrome?
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Which of the following is NOT a common clinical feature associated with Beckwith-Wiedemann syndrome?
Which of the following is NOT a common clinical feature associated with Beckwith-Wiedemann syndrome?
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A finding of perilobar nephrogenic rests in children less than one year old significantly increases their risk of developing which condition?
A finding of perilobar nephrogenic rests in children less than one year old significantly increases their risk of developing which condition?
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Which histological component of a 'favorable' Wilms tumor is considered the most undifferentiated and potentially malignant?
Which histological component of a 'favorable' Wilms tumor is considered the most undifferentiated and potentially malignant?
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What percentage of individuals with the specified renal disease will eventually develop Wilms tumor?
What percentage of individuals with the specified renal disease will eventually develop Wilms tumor?
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In boys with Wilms tumor, which of the listed presentations is commonly found?
In boys with Wilms tumor, which of the listed presentations is commonly found?
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What is characteristic of the epithelial component in a 'favorable' Wilms tumor?
What is characteristic of the epithelial component in a 'favorable' Wilms tumor?
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Which of these is NOT a syndrome listed as being sometimes associated with wilms?
Which of these is NOT a syndrome listed as being sometimes associated with wilms?
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What is the most common type of cancer found in the kidneys of children?
What is the most common type of cancer found in the kidneys of children?
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At what age is Wilms tumor most commonly diagnosed?
At what age is Wilms tumor most commonly diagnosed?
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What is the fourth most common cancer overall that affects children?
What is the fourth most common cancer overall that affects children?
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Which of the following genetic alterations has been least associated with the development of Wilms tumor, according to the text?
Which of the following genetic alterations has been least associated with the development of Wilms tumor, according to the text?
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Which physician is credited with first describing Wilms tumor?
Which physician is credited with first describing Wilms tumor?
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What is thought to be the primary cause of Wilms tumor?
What is thought to be the primary cause of Wilms tumor?
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Which genetic marker, when altered, has been linked to a poorer prognosis in Wilms tumor?
Which genetic marker, when altered, has been linked to a poorer prognosis in Wilms tumor?
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The loss of heterozygosity at which chromosome is associated with a poorer prognosis?
The loss of heterozygosity at which chromosome is associated with a poorer prognosis?
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What is the survival rate range for patients with favorable histology associated with Wilms tumor?
What is the survival rate range for patients with favorable histology associated with Wilms tumor?
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Which characteristic is associated with a poorer prognosis in Wilms tumor?
Which characteristic is associated with a poorer prognosis in Wilms tumor?
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What risk increases as a long-term effect of radiation therapy for treating Wilms tumor?
What risk increases as a long-term effect of radiation therapy for treating Wilms tumor?
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Which of the following treatment complications is associated with chemotherapy for Wilms tumor?
Which of the following treatment complications is associated with chemotherapy for Wilms tumor?
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What is the initial therapy recommended for patients with Von Willebrand's disease related to Wilms tumor?
What is the initial therapy recommended for patients with Von Willebrand's disease related to Wilms tumor?
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What aspect of survival does the report from the Children's Oncology Group primarily focus on?
What aspect of survival does the report from the Children's Oncology Group primarily focus on?
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Which type of Wilms tumor patient outcome is discussed in Iaboni et al.'s report?
Which type of Wilms tumor patient outcome is discussed in Iaboni et al.'s report?
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What cytogenetic abnormalities are associated with poorer prognosis in Wilms tumor?
What cytogenetic abnormalities are associated with poorer prognosis in Wilms tumor?
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Which symptom is most commonly associated with the initial presentation of Wilms tumor?
Which symptom is most commonly associated with the initial presentation of Wilms tumor?
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What histological feature defines anaplastic cells in Wilms tumor?
What histological feature defines anaplastic cells in Wilms tumor?
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What is a common effect seen in Wilms tumor patients post-nephrectomy?
What is a common effect seen in Wilms tumor patients post-nephrectomy?
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Which of the following evaluations is specifically indicated when Wilms tumor tissue is available?
Which of the following evaluations is specifically indicated when Wilms tumor tissue is available?
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What is a complication in Wilms tumor patients that may arise due to the tumor's interaction with the urinary system?
What is a complication in Wilms tumor patients that may arise due to the tumor's interaction with the urinary system?
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Which of the following is NOT a common symptom of Wilms tumor?
Which of the following is NOT a common symptom of Wilms tumor?
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What type of differentiation can be seen in neoplastic stroma of Wilms tumors after preoperative chemotherapy?
What type of differentiation can be seen in neoplastic stroma of Wilms tumors after preoperative chemotherapy?
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Flashcards
What is Wilms tumor?
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?
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?
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?
Who is the tumor named after?
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What are the common symptoms of Wilms tumor?
What are the common symptoms of Wilms tumor?
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How is Wilms tumor treated?
How is Wilms tumor treated?
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What are some genetic markers associated with Wilms tumor?
What are some genetic markers associated with Wilms tumor?
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What's the prognosis for Wilms tumor?
What's the prognosis for Wilms tumor?
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Renal disease in newborns and infants
Renal disease in newborns and infants
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Postoperative Monitoring
Postoperative Monitoring
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Wilms tumor
Wilms tumor
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Wilms Tumor Follow-Up Schedule (Initial)
Wilms Tumor Follow-Up Schedule (Initial)
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Beckwith-Wiedemann syndrome
Beckwith-Wiedemann syndrome
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Favorable Wilms tumor histology
Favorable Wilms tumor histology
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Wilms Tumor Follow-Up Schedule (Later)
Wilms Tumor Follow-Up Schedule (Later)
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Wilms Tumor Follow-Up Tests
Wilms Tumor Follow-Up Tests
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Blastema in Wilms tumor
Blastema in Wilms tumor
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Epithelial component of Wilms tumor
Epithelial component of Wilms tumor
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Wilms Tumor Follow-Up Complications
Wilms Tumor Follow-Up Complications
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Stromal component of Wilms tumor
Stromal component of Wilms tumor
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Perilobar nephrogenic rests
Perilobar nephrogenic rests
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Unfavorable Histology Wilms Tumor
Unfavorable Histology Wilms Tumor
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Anaplasia
Anaplasia
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Loss of Heterozygosity (LOH) at 1p and 16q
Loss of Heterozygosity (LOH) at 1p and 16q
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Heterologous Differentiation
Heterologous Differentiation
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Abdominal Mass
Abdominal Mass
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Gross Hematuria
Gross Hematuria
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Hypertension in Wilms Tumor
Hypertension in Wilms Tumor
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Renal Function Tests
Renal Function Tests
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Anaplastic Histology in Wilms Tumor
Anaplastic Histology in Wilms Tumor
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Diffuse vs. Focal Anaplasia
Diffuse vs. Focal Anaplasia
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LOH and TP53 in Wilms Tumor
LOH and TP53 in Wilms Tumor
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Radiation Therapy and Secondary Cancers
Radiation Therapy and Secondary Cancers
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Chemotherapy and Secondary Side Effects
Chemotherapy and Secondary Side Effects
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What is the COG?
What is the COG?
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What are 'National Wilms Tumor Studies'?
What are 'National Wilms Tumor Studies'?
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Why are COG studies important?
Why are COG studies important?
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How does imaging impact Wilms tumor treatment?
How does imaging impact Wilms tumor treatment?
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Why are bone metastasis in Wilms tumor cases studied?
Why are bone metastasis in Wilms tumor cases studied?
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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.