Renal Pathology Practice Questions

Q: Sickle cell disease is associated with which type of renal cell carcinoma?

Medullary. **Explanation:** **Renal Medullary Carcinoma (RMC)** is a highly aggressive, rare malignancy that occurs almost exclusively in young patients (mean age ~20 years) who carry the **Sickle Cell Trait (HbAS)** or, less commonly, Sickle Cell Disease (HbSS). **Why Medullary is correct:** The pathogenesis is linked to the hypoxic and hypertonic environment of the renal medulla [1]. These conditions promote the sickling of red blood cells in the vasa recta, leading to chronic ischemia and micro-infarctions [1]. This chronic injury, combined with the loss of the **SMARCB1 (INI1)** tumor suppressor gene expression (a hallmark of RMC), triggers malignant transformation. It typically presents as a central, infiltrative mass involving the renal pelvis and medulla. **Why other options are incorrect:** * **Papillary RCC:** Associated with trisomy 7 and 17 or MET gene mutations; it is the second most common RCC but has no specific link to hemoglobinopathies. * **Chromophobe RCC:** Originates from intercalated cells of collecting ducts; characterized by "halos" around nuclei and associated with Birt-Hogg-Dubé syndrome. * **Colloid (Mucinous) Carcinoma:** Extremely rare in the kidney; usually refers to a variant of adenocarcinoma often seen in the GI tract or breast, not associated with sickle cell. **High-Yield Clinical Pearls for NEET-PG:** * **Hallmark Marker:** Loss of **INI1 (SMARCB1)** protein expression on immunohistochemistry. * **Demographics:** Most common in young African-American males with sickle cell trait [1]. * **Prognosis:** Extremely poor; most patients present with metastatic disease at the time of diagnosis. * **Location:** Predominantly involves the **right kidney**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 948-949.

Q: What is the most common renal tumour?

Renal cell carcinoma. **Explanation:** **Renal Cell Carcinoma (RCC)** is the most common primary malignancy of the kidney, accounting for approximately **80-85%** of all malignant renal tumors in adults [1]. It originates from the renal tubular epithelium, most commonly the proximal convoluted tubule (in the case of the Clear Cell subtype). **Analysis of Options:** * **A. Renal Cell Carcinoma (Correct):** It is the most frequent renal tumor in adults, typically occurring in the 6th to 7th decades of life. Risk factors include smoking, obesity, hypertension, and acquired cystic kidney disease (ACKD) in dialysis patients [1]. * **B. Wilm’s Tumour (Nephroblastoma):** While it is the most common renal tumor in **children** (typically aged 2–5 years), it is rare in adults. * **C & D. Squamous and Transitional Cell Carcinoma:** These arise from the **urothelium** of the renal pelvis, not the renal parenchyma. Transitional Cell Carcinoma (TCC) is the most common tumor of the renal pelvis, but it is significantly less common than RCC overall [1]. Squamous cell carcinoma is rare and usually associated with chronic irritation (e.g., staghorn calculi). **High-Yield Clinical Pearls for NEET-PG:** * **Classic Triad:** Hematuria (most common), flank pain, and a palpable mass (seen in only 10% of cases). * **Most Common Subtype:** Clear Cell RCC (associated with **VHL gene** deletion on Chromosome 3p) [1]. * **Paraneoplastic Syndromes:** RCC is known as the "Internist's Tumor" because it can secrete hormones leading to Polycythemia (EPO), Hypercalcemia (PTHrP), and Hypertension (Renin). * **Staging:** The most important prognostic factor is the pathologic stage (TNM), specifically invasion of the renal vein or perinephric fat [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 958-961.

Q: Bilateral renal cell carcinoma is seen in which of the following conditions?

Von Hippel-Lindau disease. ### **Explanation** **Correct Option: C. Von Hippel-Lindau (VHL) disease** Von Hippel-Lindau disease is an autosomal dominant hereditary cancer syndrome caused by a mutation in the **VHL gene on chromosome 3p25**. The VHL protein normally degrades hypoxia-inducible factor (HIF); its loss leads to increased angiogenesis and cell proliferation. Approximately **25–45%** of patients with VHL develop **Clear Cell Renal Cell Carcinoma (RCC)**. A hallmark of hereditary RCC (like VHL) is that the tumors are frequently **bilateral and multicentric**, occurring at a younger age compared to sporadic cases [1]. **Analysis of Incorrect Options:** * **A. Eagle-Barrett Syndrome (Prune Belly Syndrome):** Characterized by a triad of abdominal muscle deficiency, undescended testes, and urinary tract abnormalities (e.g., megaureter). It is not associated with a predisposition to RCC. * **B. Beckwith-Wiedemann Syndrome:** A pediatric overgrowth disorder (WT2 mutation on Ch 11p15) associated with macroglossia, omphalocele, and hemihypertrophy. It significantly increases the risk of **Wilms tumor (Nephroblastoma)**, not RCC. * **D. Bilateral Angiomyolipoma:** While these are benign mesenchymal tumors frequently seen in **Tuberous Sclerosis** [1], they are not carcinomas. Although they can be bilateral, they do not represent Renal Cell Carcinoma. **High-Yield Clinical Pearls for NEET-PG:** * **VHL Syndrome Triad:** Hemangioblastomas (cerebellum/retina), Pheochromocytoma, and Clear Cell RCC. * **Most common type of RCC in VHL:** Clear Cell variant [1]. * **Other syndromes with RCC:** Hereditary Papillary Renal Carcinoma (MET gene) and Birt-Hogg-Dubé syndrome (FLCN gene - associated with chromophobe RCC) [1]. * **Staining:** RCC is typically positive for **Vimentin and CD10**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 957-961.

Q: All of the following are true about thin basement membrane defect, except:

Impaired renal function. **Explanation:** **Thin Basement Membrane Disease (TBMD)**, also known as **Benign Familial Hematuria**, is a common hereditary glomerular disease. The correct answer is **D** because TBMD is characterized by an excellent prognosis with **preserved renal function**; progression to chronic kidney disease or renal failure is extremely rare. * **Why Option D is correct:** Unlike Alport Syndrome, TBMD does not typically lead to hypertension, significant proteinuria, or impaired renal function. The glomerular filtration rate (GFR) remains normal throughout life. * **Why Option A is wrong:** TBMD is synonymous with "Benign Familial Hematuria" because of its non-progressive nature and strong familial tendency. * **Why Option B is wrong:** The inheritance pattern is typically **Autosomal Dominant**. It most commonly results from heterozygous mutations in the *COL4A3* or *COL4A4* genes (the same genes involved in Alport Syndrome). * **Why Option C is wrong:** The hallmark clinical presentation is persistent or recurrent **microscopic hematuria**, often discovered incidentally during routine urinalysis in childhood or young adulthood. **High-Yield Clinical Pearls for NEET-PG:** * **Electron Microscopy (EM):** This is the gold standard for diagnosis. It shows diffuse thinning of the Glomerular Basement Membrane (GBM), often measuring **150–250 nm** (Normal is ~300–400 nm). * **Light Microscopy & Immunofluorescence:** Usually appear completely **normal**. * **Differential Diagnosis:** It must be distinguished from **Alport Syndrome**, which presents with a "basket-weave" appearance on EM, sensorineural deafness, ocular defects, and progressive renal failure. * **Key Distinction:** TBMD = Heterozygous mutation; Alport Syndrome = X-linked (most common) or Autosomal Recessive (homozygous/compound heterozygous).

Q: Subepithelial deposits are characteristic findings in which of the following conditions?

Membranous glomerulonephritis (MGN). ### Explanation **Correct Answer: C. Membranous glomerulonephritis (MGN)** In **Membranous Glomerulonephritis (MGN)**, the hallmark finding on electron microscopy (EM) is the presence of **subepithelial deposits** [1] (located between the glomerular basement membrane and podocytes). These immune complexes trigger the formation of new basement membrane material around them, leading to the characteristic **"Spikes and Dome"** appearance on Silver stain [1]. This process causes diffuse thickening of the capillary wall without significant cellular proliferation [1]. **Analysis of Incorrect Options:** * **A. Minimal Change Disease:** Characterized by the **absence** of immune deposits. The primary finding is the **effacement (fusion) of podocyte foot processes** on EM. * **B. Membranoproliferative Glomerulonephritis (MPGN):** * **Type I** features **subendothelial** deposits [1]. * **Type II** (Dense Deposit Disease) features **intramembranous** deposits [1]. Both show a "tram-track" appearance due to mesangial interposition. * **D. Post-streptococcal Glomerulonephritis (PSGN):** While PSGN does feature subepithelial deposits, they are specifically described as large, discrete **"subepithelial humps"** [1]. However, MGN is the classic prototype for continuous subepithelial deposition leading to membrane thickening. **High-Yield NEET-PG Pearls:** * **MGN:** Most common cause of Nephrotic syndrome in adults; associated with PLA2R antibodies (primary) and HBV, NSAIDs, or solid tumors (secondary). * **Rule of "Sub-":** * **Subepithelial:** MGN, PSGN (Humps). * **Subendothelial:** MPGN Type I, SLE (Wire loops - Class IV). * **Intramembranous:** MPGN Type II. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 911-927.

Question 1

Sickle cell disease is associated with which type of renal cell carcinoma?

Accepted Answer

Medullary

Answer

Papillary

Answer

Chromophobe

Answer

Colloid

Question 2

Which of the following are renal lesions seen in Multiple Myeloma?

Accepted Answer

All of the above

Answer

Cast nephropathy

Answer

Amyloid deposition

Answer

Plasma cell infiltration

Question 3

What is the most common renal tumour?

Accepted Answer

Renal cell carcinoma

Answer

Wilm's tumour

Answer

Squamous cell carcinoma of the renal pelvis

Answer

Transitional cell carcinoma of the renal pelvis

Question 4

Bilateral renal cell carcinoma is seen in which of the following conditions?

Accepted Answer

Von Hippel-Lindau disease

Answer

Eagle-Barrett syndrome

Answer

Beckwith-Wiedemann syndrome

Answer

Bilateral angiomyolipoma

Question 5

What is the most common infectious agent associated with chronic pyelonephritis?

Accepted Answer

Escherichia coli

Answer

Proteus vulgaris

Answer

Klebsiella pneumoniae

Answer

Staphylococcus aureus

Question 6

Squamous cell carcinoma of the urinary bladder is predisposed to by which of the following?

Accepted Answer

Urolithiasis

Answer

Persistent Urachus

Answer

Schistosomiasis

Answer

Smoking

Question 7

Flea bitten kidney is seen in all of the following conditions except?

Accepted Answer

Diabetes Mellitus

Answer

Malignant hypertension

Answer

Henoch-Schonlein purpura

Answer

Polyarteritis nodosa

Question 8

All of the following are causes of granular contracted kidney except?

Accepted Answer

Diabetes mellitus

Answer

Benign nephrosclerosis

Answer

Chronic pyelonephritis

Answer

Chronic glomerulonephritis

Question 9

All of the following are true about thin basement membrane defect, except:

Accepted Answer

Impaired renal function

Answer

Also known as benign familial hematuria

Answer

Autosomal dominant inheritance

Answer

Persistent or recurrent microscopic hematuria

Question 10

Subepithelial deposits are characteristic findings in which of the following conditions?

Accepted Answer

Membranous glomerulonephritis (MGN)

Answer

Minimal change disease

Answer

Membranoproliferative glomerulonephritis (MPGN)

Answer

Post-streptococcal glomerulonephritis (PSGN)

Renal Pathology — MCQs

Renal Pathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?