Renal Pathology Practice Questions

Q: Renal angiomyolipoma is associated with which of the following conditions?

Tuberous sclerosis. **Explanation:** **Renal Angiomyolipoma (AML)** is a benign mesenchymal tumor composed of three distinct elements: thick-walled blood vessels (**Angio**), smooth muscle (**Myo**), and mature adipose tissue (**Lipoma**). 1. **Why Tuberous Sclerosis is correct:** While most AMLs are sporadic, approximately **25–50% of patients with Tuberous Sclerosis Complex (TSC)** develop these tumors [1]. Conversely, when an AML is bilateral or multicentric, it is almost pathognomonic for TSC. The association is linked to mutations in the **TSC1 (hamartin)** or **TSC2 (tuberin)** genes, which lead to overactivation of the mTOR pathway, resulting in abnormal cell growth and hamartoma formation. 2. **Why the other options are incorrect:** * **Neurofibromatosis 1 (NF1):** Characterized by Lisch nodules, café-au-lait spots, and neurofibromas. While NF1 is associated with renal artery stenosis and pheochromocytomas, it is not linked to AML. * **Neurofibromatosis 2 (NF2):** Primarily associated with bilateral acoustic neuromas (schwannomas), meningiomas, and ependymomas. It has no association with renal hamartomas. **High-Yield Clinical Pearls for NEET-PG:** * **Triad of TSC (Vogt’s Triad):** Seizures, mental retardation, and adenoma sebaceum (facial angiofibromas) [1]. * **Imaging:** On CT, the presence of **fat density** within a renal mass is highly suggestive of AML. * **Complication:** Large AMLs (>4 cm) are prone to spontaneous hemorrhage, known as **Wunderlich syndrome**. * **Immunohistochemistry:** AMLs are positive for **HMB-45** (a melanocytic marker), which is a classic "catchy" fact for pathology exams. * **Treatment:** Everolimus (an mTOR inhibitor) is used specifically for TSC-associated AML. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1318-1319.

Question 1

Onion skin lesions, in the muscular layer of arteriole, are seen in which of the following conditions?

Accepted Answer

Malignant nephrosclerosis

Answer

Systemic Lupus Erythematosus (SLE)

Answer

Benign nephrosclerosis

Answer

Rapidly Progressive Glomerulonephritis (RPGN)

Question 2

Hypocomplementemia is seen in which of the following conditions?

Accepted Answer

Lupus nephritis

Answer

Focal glomerulonephritis

Answer

Minimal change disease

Answer

All of the above

Question 3

Renal angiomyolipoma is associated with which of the following conditions?

Accepted Answer

Tuberous sclerosis

Answer

Neurofibromatosis 1

Answer

Neurofibromatosis 2

Answer

None of the above

Question 4

HIV infection is most commonly associated with which type of glomerulonephritis?

Accepted Answer

Collapsing glomerulonephritis

Answer

Membranous glomerulonephritis

Answer

Fibrillary glomerulopathy

Answer

Rapidly progressive glomerulonephritis (RPGN)

Question 5

Nephrocalcinosis is seen in which of the following conditions?

Accepted Answer

Medullary sponge disease

Answer

Acute pyelonephritis

Answer

Acute glomerulonephritis

Answer

Chronic pyelonephritis

Question 6

A patient with clinical features of Glomerulonephritis presents with granular staining on immunofluorescence. All of the following causes are associated with normal C3 levels except?

Accepted Answer

Cryoglobulinemia

Answer

IgA Nephropathy

Answer

Henoch-Schönlein purpura

Answer

Fibrillary GN

Question 7

Characteristic renal biopsy immunofluorescence findings that lead to the diagnosis of Berger's disease are:

Accepted Answer

Predominantly IgA deposition in the mesangium

Answer

Predominantly IgA deposition in the subepithelial region

Answer

Predominantly IgG deposition in the subendothelial region

Answer

Predominantly IgG deposition in the glomerular basement membrane

Question 8

Which gene is commonly involved in mutations leading to renal cell carcinoma?

Accepted Answer

VHL

Answer

TP53

Answer

NF1

Answer

BRCA-1

Question 9

What is the most common predisposing factor for chronic pyelonephritis?

Accepted Answer

Vesicoureteric reflux

Answer

Diabetes mellitus

Answer

Renal stone

Answer

Posterior urethral valve

Question 10

Which of the following statements is FALSE regarding Goodpasture's disease?

Accepted Answer

Linear deposition of IgG along the glomerular basement membrane is characteristic.

Answer

Antibodies are directed against the non-collagenous (NC-1) domain of the alpha-3 chain of type IV collagen.

Answer

Complement levels are typically normal.

Answer

The prognosis is generally poor if untreated.

Renal Pathology — MCQs

Renal Pathology — MCQs

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