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What is the first step in the management of Wilms Tumor according to the Society of International Pediatric Oncology (SIOP)?
Blastemal histological findings indicate a better prognosis for Wilms Tumor.
False
What type of chemotherapy is used for Wilms Tumor?
Dactinomycin based chemotherapy
The initial imaging investigation typically employed is ___.
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Match the following terms related to Wilms Tumor management and their descriptions:
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What is the size in mm of a 12 French Foley catheter?
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Silicone Foley catheters can be used for a maximum duration of 2 months.
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What is the best method to retract a stuck Foley catheter balloon during deflation?
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Chronic urinary retention is commonly associated with _______ obstruction.
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Match the following types of Foley catheters with their duration of use:
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Which clinical feature is associated with secondary bladder diverticulum?
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A primary congenital bladder diverticulum is caused by a muscular defect between the intravesical ureter and the root of the ureteral hiatus.
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What procedure is performed to repair a bladder injury in a stable patient?
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A Grade IV bladder injury involves a laceration that is __________ bladder wall laceration ≥ 2 cm.
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Match the type of bladder diverticulum with its characteristics:
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Which type of renal cancer has the best prognosis?
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Distant metastasis of renal cell carcinoma is most commonly seen in the liver.
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What is the key feature of clear cell renal cell carcinoma?
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The classical triad of symptoms associated with renal cell carcinoma includes hematuria, flank pain, and ______.
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Match the following renal conditions with their features:
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What is a common indication for performing a radical nephrectomy?
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Cryoablation is indicated for elderly patients with renal cell carcinoma (RCC) where surgery cannot be performed.
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What is the most common pediatric renal tumor?
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Wilms Tumor can often present as __________.
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Match the associated conditions with their descriptions:
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Which of the following is a common complication during the spread of Wilms Tumor?
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Histological findings showing epithelial cells in Wilms Tumor are indicative of poor prognosis.
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What treatment option can be used to decrease tumor burden in metastatic tumors?
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Study Notes
Urology 352
- Peripheral calcification can indicate neuroblastoma, which is an intra-tumoral calcification.
- Stage V in Wilms Tumor (WT) involves bilateral involvement.
- WT is sensitive to chemotherapy and radiation.
- The standard chemotherapy treatment for WT is based on dactinomycin.
- The surgical principles for WT are similar to those for renal cell carcinoma (RCC).
- The recommended approach for managing WT is surgery followed by chemotherapy and potentially radiation.
- The most important prognostic factors for WT are histological findings, especially the presence of blastemal cells, which indicate a poor prognosis.
- The Society of International Pediatric Oncology (SIOP) recommends chemotherapy, followed by surgery, and then radiation therapy for WT.
Bladder Diverticulum
- Bladder diverticula can lead to clinical features such as syncopal attack, peritonitis, and sudden urge to pass urine while changing posture.
- In stable patients, a CT urography can be used to visualize the bladder diverticulum.
- Other diagnostic tools for bladder diverticula include MCU (Micturition Cystourethrography) and RGU (Retrograde Urethrography).
- If necessary, explorative laparotomy with bladder repair in two layers and Foley's catheterization/SPC (Suprapubic Catheterization) can be performed.
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Grading
- Grade 1: Contusion or intramural hematoma.
- Grade 2: Partial-thickness laceration.
- Grade 3: Extraperitoneal laceration less than 2 cm, extraperitoneal laceration greater than 2 cm, or intraperitoneal laceration less than 2 cm.
- Grade 4: Intraperitoneal laceration greater than 2 cm.
- Grade 5: Laceration extending to the bladder neck or ureteral orifice.
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Types:
- Primary Congenital: This type is characterized by a herniation of the mucosa and is caused by a congenital muscular defect between the intravesical ureter and the root of the ureteral hiatus (Hutch diverticulum). It can be associated with VUR (vesico-ureteral reflux).
- Secondary (Pulsion): This type is caused by conditions like bladder outlet obstruction, BPH, urethral stricture, and bladder neck stenosis. Clinical features include recurrent UTI, VUR and associated upper urinary tract infections, and recurrent urge to pass urine when changing posture.
Foley's Catheter & Urinary Retention
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Foley's Catheter:
- French Grading: Used to measure the outer circumference/diameter of the catheter. Each French unit corresponds to 0.33 mm.
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Color Coding:
- White: 12 French (4mm)
- Green: 14 French (4.7 mm)
- Orange: 16 French (5.3 mm)
- Red: 18 French (6 mm)
- Yellow: 20 French (6.7 mm)
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Types:
- Rubber Foley's: Lasts approximately 25-30 days.
- Silicone Foley's: Lasts approximately 3 months. Offers reduced colonization and crusting.
- Stuck Foley's: Ultrasound-guided balloon puncture is the best method to retract the balloon during deflation.
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Urinary Retention:
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Acute Urinary Retention:
- Clinical Features: Pain and abdominal swelling.
- Dehydration: Usually absent.
- Investigation: KFT (Kidney Function Test) usually normal.
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Chronic Urinary Retention:
- Predisposing factors: Bladder outlet obstruction, BPH.
- Management: Foley catheterization/SPC (Suprapubic catheterization), addressing the underlying cause (e.g., bladder neck stenosis or spinal injury).
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Acute Urinary Retention:
Kidney: Part 2
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Renal Cell Carcinoma (RCC) Types:
- Clear Cell: Most common, prognosis varies. Arises from proximal convoluted tubule (PCT) and is characterized by clear cells.
- Papillary: Prognosis varies. Arises from PCT or distal convoluted tubule (DCT).
- Chromophobe: Best prognosis. Arises from unknown origin and features plant-like cells/bodies.
- Collecting Duct: Worst prognosis, arises from collecting duct and only occurs in 15-20% of patients.
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Other RCC Characteristics:
- Clear Cell: Hematuria is the most common presentation.
- Papillary: Characterized by the classical triad: hematuria, flank pain, and an abdominal mass.
- Chromophobe: Known for resin-like nuclei.
- Collecting Duct: Doesn't have any distinctive features.
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Spread of RCC:
- Along renal veins: Tumor thrombi can spread along the renal veins.
- Left RCC: Can block the testicular vein leading to secondary varicocele.
- Distant Metastases: Most commonly in the lungs, presenting as cannonball metastases.
- Paraneoplastic syndromes: Elevated ESR is the most common
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Endocrine Paraneoplastic Syndromes:
- Hypercalcemia: Due to parathyroid-related peptide.
- Hypertension.
- Polycythemia: Due to erythropoietin release.
- Nonmetastatic hepatic dysfunction (Stauffer syndrome): Mediated by interleukin-6, causing elevated bilirubin and liver enzymes.
- Galactorrhea.
- Cushing's syndrome: Due to ACTH release.
- Alterations in glucose metabolism.
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Nonendocrine Paraneoplastic Syndromes:
- Amyloidosis.
- Anemia.
- Neuromyopathies: Lambert-Eaton syndrome.
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Investigations:
- CECT (Contrast-enhanced computed tomography).
- Biopsy: Indicated for metastasis, unclear diagnosis, or if a diagnosis is unclear.
- CT angiography: To assess the spread of the tumor along the renal vein.
- Image Description: Three grayscale CT images of a torso. A lighter, less dense area is visible at the center of one of the images. This could indicate a tumor or another abnormality within the abdominal region.
Radical Nephrectomy
- Performed if partial nephrectomy is contraindicated.
- Involves removing the kidney, Gerota's fascia, para-aortic lymph nodes, and the ureter up to the pelvic brim.
Cryoablation
- Rapid freezing and gradual thawing of the tumor tissue at -20°C.
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Indications:
- For small RCC where surgery is not possible (e.g., elderly patients).
- As palliative surgery for advanced or metastatic tumors.
- Debulking surgery for metastatic tumors to reduce tumor burden.
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Used in conjunction with:
- mTOR (mammalian target of rapamycin) inhibitors (e.g., sirolimus).
- Tyrosine kinase inhibitors (e.g., sorafenib, sunitinib).
- IL-2 (interleukin-2).
Wilms Tumor (WT)
- Most common pediatric renal tumor.
- Typically occurs in children between the ages of 2 and 5 years.
- Often associated with abdominal malignancies and presents similarly to neuroblastoma.
Clinical Features
- Presence of a mass in the abdomen that rarely crosses the midline (neuroblastoma commonly crosses the midline).
- Hematuria.
Spread
- Along renal veins as tumor thrombi.
- Distant metastases: Lungs are the most common site.
- Relatively easy to resect.
Etiology
- Familial (locus: chromosome II).
Associated Conditions
- Sporadic.
- Can be associated with:
- Beckwith-Wiedemann syndrome: Characterized by macroglossia, hypoglycemia, and WT.
- Denys-Drash syndrome: Includes WT, GU malformations, and nephropathy.
- WAGR syndrome: Features WT, aniridia (absence of the iris), genitourinary abnormalities, and mental retardation.
Prognostic Factors
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Histological findings:
- Epithelial cells.
- Blastemal cells: Highly associated with poor prognosis.
- Additional Notes:* The image contains a colored box labeled "Active space" (purple). The image also includes a table/flowchart showing the relationship between different types of cancer (HCC, GIST). However, the image is too blurry to properly understand this diagram/table.
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Description
This quiz covers key topics in Urology 352, focusing on Wilms Tumor, its treatment protocols, and the implications of bladder diverticulum. Learn about the standard treatments, surgical principles, and important prognostic factors related to these conditions.
