Renal Neoplasms and Congenital Renal Structures

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Questions and Answers

Which gestational week does the fusion occur in a horseshoe kidney?

Week 7 (correct)

Week 11

Week 5

Week 9

Which of the following is NOT a common finding in patients with horseshoe kidneys?

Ureteropelvic junction (UPJ) obstruction

Ureteral duplication

Hydronephrosis (correct)

Vesicoureteral reflux

Which condition is a horseshoe kidney sometimes associated with?

Cystic fibrosis

Turner's syndrome (correct)

Parkinson's disease

Sickle cell anemia

What is the best diagnostic test for detecting a horseshoe kidney?

CT urography Signup and view all the answers

What is the increased risk associated with a horseshoe kidney?

Increased risk of Wilms' tumor Signup and view all the answers

What is the most common type of renal fusion abnormality?

Horseshoe kidney Signup and view all the answers

What percentage of renal fusions occur at the lower poles of the kidneys?

90% Signup and view all the answers

What is the best test for detecting a horseshoe kidney?

CT urography Signup and view all the answers

What is the best test for detecting vesicoureteral reflux in patients with a horseshoe kidney?

VCUG Signup and view all the answers

What is the most common cause of hydronephrosis?

Kidney stones larger than 10 mm Signup and view all the answers

What is the typical presentation of a patient with hydronephrosis pain?

Pain in flanks, abdomen, or groin Signup and view all the answers

What is the typical urine analysis finding in a patient with hydronephrosis?

Normal Signup and view all the answers

What is the best imaging test to detect hydronephrosis?

Ultrasound Signup and view all the answers

What is the mechanism by which hydronephrosis can cause hypertension, which is the main sign of this condition?

Activation of the RAAS system Signup and view all the answers

Which of the following statements about the urine analysis in a patient with hydronephrosis is correct?

The urine analysis will be normal Signup and view all the answers

Which imaging modality should be ordered if there is a concern for a kidney stone causing hydronephrosis?

CT scan Signup and view all the answers

What is the underlying genetic cause of Polycystic Kidney Disease (PKD)?

An autosomal dominant mutation in either the PDK1 or PDK2 gene Signup and view all the answers

Which of the following organs is NOT commonly affected in Polycystic Kidney Disease?

Gallbladder Signup and view all the answers

Which of the following is NOT a common presenting symptom or sign in patients with Polycystic Kidney Disease?

Jaundice Signup and view all the answers

Which diagnostic test is typically used to diagnose Polycystic Kidney Disease?

Ultrasound Signup and view all the answers

What is the recommended management approach for a patient with multiple cysts due to Polycystic Kidney Disease?

Increased fluid intake and hypertension control Signup and view all the answers

Which organ is most commonly affected in patients with Polycystic Kidney Disease?

Liver Signup and view all the answers

What is the recommended management approach for patients with simple cysts due to Polycystic Kidney Disease?

Observation Signup and view all the answers

Which age group tends to be affected by Polycystic Kidney Disease (PKD)?

Adults in their 30-40s Signup and view all the answers

What is the most common cause of secondary hypertension that should be suspected in a patient with continued hypertension even while taking 3 anti-hypertensive drugs (or a unexplained creatinine rise)?

Renal artery stenosis Signup and view all the answers

What is the recommended initial imaging modality for diagnosing renal artery stenosis?

CT angiography or MR angiography Signup and view all the answers

What is the definitive diagnostic test for renal artery stenosis?

Renal catheter arteriography Signup and view all the answers

Which patients with renal artery stenosis should not take ACEi or ARBs?

Patients with bilaterally renal artery stenosis or patients with only one kidney that has renal artery stenosis Signup and view all the answers

What is the definitive management for renal artery stenosis?

Revascularization Signup and view all the answers

What is a typical blood pressure reading in patients with renal artery stenosis after the age of 55?

Above 160/100 mmHg Signup and view all the answers

What size difference in kidney may indicate renal artery stenosis?

1.5+ cm Signup and view all the answers

What is the classic triad of symptoms for patients with advanced renal cell carcinoma?

Hematuria, flank or abdominal pain, and a palpable abdominal mass Signup and view all the answers

Which imaging test is typically used as the initial evaluation for renal cell carcinoma?

CT scan Signup and view all the answers

What is the main treatment approach for renal cell carcinoma?

Radical nephrectomy Signup and view all the answers

Which syndrome, involving hypercalcemia and cachexia, is common in patients with renal cell carcinoma?

Stauffer Syndrome Signup and view all the answers

What is the most common location of metastasis for renal cell carcinoma?

Lungs Signup and view all the answers

What is the definitive diagnostic test for renal cell carcinoma?

Tissue biopsy Signup and view all the answers

Renal cell carcinoma is a tumor of the

proximal convoluted renal tubules Signup and view all the answers

Smoking is NOT a major risk factor for renal cell carcinoma

False Signup and view all the answers

Which of the following syndromes is characterized by the triad of aniridia, genital malformations, and mental retardation, in addition to Wilms tumor?

WAGR syndrome Signup and view all the answers

Which imaging modality is considered the most accurate for diagnosing Wilms tumor?

Both CT scan with contrast and MRI scan Signup and view all the answers

At what age range is Wilms tumor most commonly diagnosed?

0 to 5 years Signup and view all the answers

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

Muscle wasting Signup and view all the answers

What is the recommended management for Wilms tumor?

Total nephrectomy followed by chemotherapy Signup and view all the answers

Study Notes

Horseshoe Kidney

Most common of all renal fusion abnormalities
90% of fusions happen at the lower poles of the kidneys
Fusion takes place around gestational week 7 in utero
May present with other genital abnormalities such as hypospadias, undescending tests, bicornuate uterus, or septate vagina
Common findings include issues with the urinary collecting system (ureteral duplication, UPJ obstruction, and vesicoureteral reflux)
Vesicoureteral reflux increases the risk of UTIs
May present in patients with Turner's syndrome, Trisomy 13, 18, or 21
Most patients do not have symptoms
Found at 18-20 weeks gestation during an anatomy ultrasound
CT urography is the best test for detection
VCUG is the best test for urinary reflux
Two times increased incident of Wilm's tumor in patients with a horseshoe kidney

Hydronephrosis

When urine backs up into the kidney leading to kidney swelling
Patients are commonly asymptomatic, but may present with pain in the flanks, abdomen, or groin
May have hypertension due to the kidney's activation of the RAAS
Will have a normal urine analysis
Seen on ultrasound
CT scan should be ordered if concerned about kidney stones as the cause
Most common cause is a kidney stone (especially stones larger than 10 mm)
Scar tissue and adhesions from previous urinary system surgeries can also cause hydronephrosis

Polycystic Kidney Disease (PKD)

Due to cyst formation from the nephrons that grow outward
Autosomal dominant mutation in either PDK1 or PKD2
More severe PKD is from a mutation in PDK1
Multisystem disorder that commonly impacts the liver, spleen, pancreas, heart, and brain
Liver is the most commonly impacted organ
Typically found in patients in their 30-40s with a family history
Patients complain of abdominal pain, flank pain, nephrolithiasis, UTI, and hematuria
Urine analysis shows hematuria, decreased urine concentration, and proteinuria
Diagnosed with ultrasound
Managed based on the number of cysts present
Patients should be referred to a nephrologist

Renal Vascular Disease – Renal Artery Stenosis

Most common cause of secondary hypertension
Should be suspected if a patient has uncontrolled hypertension or unexplained creatinine elevate
Can cause asymmetry in kidney size with one kidney being more than 1.5 cm larger than the other
Patients likely have blood pressures above 160/100 after the age of 55
CT angiography or MR angiography are the first-line imaging modalities
Renal catheter arteriography is the definitive diagnostic test
Definitive management is revascularization
Patients with bilateral renal artery stenosis or only one kidney that has renal artery stenosis should not take ACEi or ARBs, it is contraindicated

Renal Cell Carcinoma

Tumor of the proximal convoluted renal tubules
Around 95% of primary tumors originate in the kidney
Smoking is a major risk factor
Most common location of metastasis is the lungs, which looks like a “cannon ball” on imaging
Classic triad of symptoms: hematuria, flank or abdominal pain, and a palpable abdominal mass
Patients experience malaise and weight loss
Paraneoplastic syndrome, including hypercalcemia and cachexia, is common
Stauffer Syndrome is hepatic dysfunction in the absence of liver tumors due to cytokine release
CT scan is the initial imaging test
PET scan can be used to evaluate for potential metastases in other organs
Definitive diagnosis is tissue biopsy
Main treatment is radical nephrectomy

Wilms Tumor

Nephroblastoma
Most common renal malignancy in children and most common cause of abdominal masses in children
Presents at ages 0 to 5 years
Present with a palpable abdominal mass, abdominal swelling, and pain
Associated with WAGR syndrome (deletion on chromosome 11), Denys-Drash syndrome, and Beckwith-Wiedemann syndrome
Best initial test is an abdominal ultrasound
CT with contrast or MRI is the most accurate imaging test
Has a high cure rate when managed with a total nephrectomy followed by chemotherapy

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Description

Test your knowledge on horseshoe kidneys, the most common renal fusion abnormalities. Learn about the fusion process, associated genital abnormalities, and common issues with the urinary collecting system in patients with horseshoe kidneys.