Renal Pathology Practice Questions

Q: A 4-year-old child presented with a palpable abdominal mass in the right flank region which was painless and slowly increasing in size along with some episodes of fever and hematuria. On examination, hypertension was noted. CT scan of the abdomen was done. The patient was operated and the mass was resected. The gross specimen and the HPE examination are given below. All of the following genes can be mutated in the above disease except?

RAS. ***RAS*** - **RAS mutations** are classically associated with **neuroblastoma** and other malignancies, not with **Wilms' tumor** (nephroblastoma). - The **Wnt signaling pathway** and **tumor suppressor genes** are the primary molecular mechanisms involved in Wilms' tumor pathogenesis, not RAS oncogenes. *WT1* - **WT1 gene mutations** are found in approximately **10-15%** of Wilms' tumors and are associated with **WAGR syndrome** and **Denys-Drash syndrome**. - Loss of **WT1 tumor suppressor function** leads to abnormal **nephrogenesis** and predisposes to Wilms' tumor development. *CTNNB1* - **CTNNB1 gene** encodes **β-catenin**, a key component of the **Wnt signaling pathway** that is frequently dysregulated in Wilms' tumor. - Mutations in **CTNNB1** lead to **aberrant Wnt pathway activation**, promoting cellular proliferation and tumor formation in nephroblastoma. *AMER1* - **AMER1 (APC membrane recruitment protein 1)** is part of the **Wnt signaling pathway** and acts as a **tumor suppressor**. - **AMER1 mutations** are associated with **Wilms' tumor predisposition** and contribute to **Wnt pathway dysregulation** in nephroblastoma development.

Q: Which of the following statements is true regarding kidney tumors?

Mutated VHL gene is associated with clear cell carcinoma. **Explanation:** **1. Why Option A is Correct:** Clear cell renal cell carcinoma (RCC) is the most common subtype of kidney cancer. The hallmark of this tumor is the loss of sequences on the **short arm of chromosome 3 (3p)**. This region houses the **VHL (Von Hippel-Lindau) gene**. In sporadic cases, both alleles of the VHL gene are inactivated (one via deletion and the other via mutation or hypermethylation). In familial VHL syndrome, a germline mutation is present. The loss of VHL protein leads to the stabilization of **HIF-1\u03b1** (Hypoxia-Inducible Factor), which triggers the overexpression of VEGF and PDGF, driving angiogenesis and tumor growth. **2. Why Other Options are Incorrect:** * **Options B & C:** These are characteristic of **Chromophobe RCC**. Chromophobe RCC is uniquely associated with **extreme hypodiploidy** (loss of multiple chromosomes including 1, 2, 6, 10, 13, 17, and 21), rather than hyperdiploidy [1]. * **Option D:** **Papillary RCC** is associated with trisomies of **chromosomes 7 and 17** and the loss of Y in sporadic cases [1]. The most common genetic defect in hereditary papillary RCC is a mutation in the **MET proto-oncogene** (on chromosome 7), not chromosome 8 [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Clear Cell RCC:** Most common; associated with 3p deletion; "clear" cytoplasm due to glycogen and lipid accumulation [1]. * **Papillary RCC:** Frequently multifocal and bilateral; associated with MET gene [1]. * **Chromophobe RCC:** Best prognosis; features "halos" around nuclei and prominent cell membranes (vegetable cell appearance) [1]. * **Oncocytoma:** Benign tumor; characterized by a central stellate scar and abundant mitochondria. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 958-959.

Q: What is the most common variant of renal cell carcinoma (RCC)?

Clear cell. **Explanation:** Renal Cell Carcinoma (RCC) is the most common primary malignancy of the kidney, arising from the renal tubular epithelium. **1. Why Clear Cell is Correct:** **Clear cell RCC** is the most common histological subtype, accounting for approximately **70-80%** of all renal cancers [1]. It typically arises from the **proximal convoluted tubule**. Microscopically, it is characterized by cells with clear cytoplasm (due to accumulated glycogen and lipids) and a prominent delicate vascular network ("chicken-wire" pattern) [1]. Most sporadic cases are associated with deletions or mutations of the **VHL gene** on chromosome 3p. **2. Why the Other Options are Incorrect:** * **Papillary RCC (10-15%):** The second most common variant [1]. It often presents as multifocal or bilateral tumors and is associated with trisomy 7 and 17 [1]. * **Chromophobe RCC (5%):** Arises from intercalated cells of the collecting duct [1]. It has a better prognosis and is characterized by "perinuclear halos" and a positive Hale’s colloidal iron stain [1]. * **Collecting Duct (Bellini) Carcinoma (<1%):** An extremely rare and highly aggressive variant arising from the medulla. **High-Yield Clinical Pearls for NEET-PG:** * **Classic Triad:** Hematuria (most common), flank pain, and palpable mass (seen in only 10% of cases). * **Paraneoplastic Syndromes:** RCC is the "Great Mimicker," often producing EPO (polycythemia), PTHrP (hypercalcemia), or Renin (hypertension). * **Stauffer Syndrome:** Reversible hepatic dysfunction without liver metastases. * **Spread:** Characteristically shows **hematogenous spread** via the renal vein to the IVC and lungs (cannon-ball metastases) [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 957-961.

Question 1

IgA nephropathy is characterized by which of the following glomerular changes?

Accepted Answer

Mesangioproliferative glomerulonephritis

Answer

Membranoproliferative glomerulonephritis

Answer

Minimal change glomerulonephritis

Answer

Rapidly progressive glomerulonephritis type I

Question 2

Which of the following is rarely associated with renal cell carcinoma?

Accepted Answer

Cushing's syndrome

Answer

Polycythemia

Answer

Amyloidosis

Answer

Hypertension

Question 3

Which condition is characterized by the presence of WBC casts?

Accepted Answer

Pyelonephritis

Answer

Glomerulonephritis

Answer

Chronic renal failure

Answer

Hyaline cast

Question 4

A 4-year-old child presented with a palpable abdominal mass in the right flank region which was painless and slowly increasing in size along with some episodes of fever and hematuria. On examination, hypertension was noted. CT scan of the abdomen was done. The patient was operated and the mass was resected. The gross specimen and the HPE examination are given below. All of the following genes can be mutated in the above disease except?

Accepted Answer

RAS

Answer

WT1

Answer

CTNNB1

Answer

AMER1

Question 5

A middle-aged diabetic female presented with flank pain and fever. On ultrasound, the kidney was irregular and showed a fat-density lesion with calculi. What is the most probable diagnosis?

Accepted Answer

Xanthogranulomatous pyelonephritis

Answer

Tuberculosis of kidney

Answer

Chronic pyelonephritis

Answer

Renal abscess

Question 6

Which of the following statements is true regarding kidney tumors?

Accepted Answer

Mutated VHL gene is associated with clear cell carcinoma

Answer

Extreme hyperdiploidy occurs

Answer

Extreme hypodiploidy occurs

Answer

Renal papillary carcinoma has a defect in chromosome 8

Question 7

A 65-year-old man develops oliguria and peripheral edema over a period of weeks. Urinalysis reveals hematuria and proteinuria; examination of the urinary sediment reveals red cell casts. Radiological and ultrasound studies fail to demonstrate an obstructive lesion. Renal biopsy shows many glomerular crescents. This presentation is most suggestive of which of the following conditions?

Accepted Answer

Anti-glomerular basement membrane disease

Answer

Diabetic nephropathy

Answer

Hypertensive nephropathy

Answer

Lupus nephritis

Question 8

Which of the following conditions is associated with pauci-immune crescentic glomerulonephritis?

Accepted Answer

Microscopic polyangiitis

Answer

Henoch-Schonlein nephritis

Answer

Lupus nephritis

Answer

Alport syndrome

Question 9

What is the most common variant of renal cell carcinoma (RCC)?

Accepted Answer

Clear cell

Answer

Papillary

Answer

Chromophobe

Answer

Collecting duct

Question 10

What is the most common extra-renal manifestation of autosomal dominant polycystic kidney disease?

Accepted Answer

Cysts in the liver

Answer

Diverticulosis of the gastrointestinal tract

Answer

Berry aneurysms

Answer

Pancreatic cysts

Renal Pathology — MCQs

Renal Pathology — MCQs

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