Renal Pathology Practice Questions

Q: All are true about infantile polycystic kidney disease except?

Autosomal dominant. **Explanation:** The correct answer is **A (Autosomal dominant)** because Infantile Polycystic Kidney Disease (IPKD) is inherited in an **Autosomal Recessive (AR)** pattern [1]. It is caused by mutations in the **PKHD1 gene** on chromosome 6, which encodes the protein **fibrocystin** [1]. In contrast, the adult form of the disease (ADPKD) is Autosomal Dominant. **Analysis of Options:** * **Option A (Incorrect Statement):** As stated, IPKD is AR, not AD. This makes it the correct "except" choice. * **Option B & D (True Statements):** IPKD is almost invariably associated with liver involvement [1]. This spectrum is known as **Fibrocystic Liver Disease**, characterized by **hepatic cysts** and **periportal fibrosis** (congenital hepatic fibrosis). In older children, this can lead to portal hypertension and splenomegaly. * **Option C (True Statement):** Unlike the adult form, where cysts develop over decades, in the infantile form, **renal cysts are present at birth** [1]. The kidneys are symmetrically enlarged with a "sponge-like" appearance due to cylindrical dilation of the collecting ducts. **NEET-PG High-Yield Pearls:** * **Morphology:** Grossly, the kidneys have a smooth external surface (unlike the bosselated surface in ADPKD) with elongated, fusiform cysts arranged radially. * **Potter Sequence:** Severe cases present in utero with oligohydramnios, leading to pulmonary hypoplasia, flattened facies, and clubfeet. * **Key Association:** The severity of renal disease is often inversely proportional to the severity of hepatic fibrosis. * **Gene:** Remember **PKHD1** for ARPKD and **PKD1/PKD2** for ADPKD [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 952-953.

Q: A specimen shows a nephrectomy. What is your diagnosis?

Renal cell carcinoma. ***Renal cell carcinoma*** - Presents as a **golden-yellow variegated** mass in the **renal cortex** with areas of **hemorrhage and necrosis** on gross examination. - Typically shows **clear cell morphology** with abundant cytoplasm and is the most common primary renal malignancy requiring nephrectomy. *Renal papillary adenoma* - Usually **very small** (typically <5mm) **benign tumors** that rarely require nephrectomy due to their minimal size. - Characterized by **papillary architecture** with **bland cytology** and lack the aggressive features seen in larger masses. *Urothelial cancer of renal pelvis* - Arises from the **renal pelvis** and **collecting system**, not the cortical parenchyma like RCC. - Shows **white-tan** appearance with **papillary or solid growth** pattern extending into the pelviocalyceal system. *Oncocytoma* - Appears as a **well-circumscribed tan-brown mass** with a characteristic **central stellate scar** on cut surface. - **Benign tumor** composed of **oncocytes** with abundant eosinophilic cytoplasm, typically doesn't require nephrectomy unless large.

Question 1

A 49-year-old male who is a long-term smoker presents with frequency and hematuria. Histologic examination of sections taken from an exophytic lesion of the urinary bladder reveals groups of atypical cells with frequent mitoses forming finger-like projections that have thin, fibrovascular cores. These groups of atypical cells do not extend into the lamina propria and muscularis. No glands or keratin production are found. What is the most accurate diagnosis for this bladder tumor?

Accepted Answer

Papillary transitional cell carcinoma (TCC), noninvasive

Answer

Adenocarcinoma, noninvasive

Answer

Inverted papilloma

Answer

Transitional cell carcinoma in situ

Question 2

A 2 kg baby, born at 30 weeks gestation to an 18-year-old primigravida, died after 48 hours. The Apgar scores were 5 and 8 at 1 and 5 minutes, respectively. Autopsy revealed bilateral enlarged kidneys with multiple radially arranged cysts. Which of the following findings is expected to be associated with this condition?

Accepted Answer

Hepatic cyst and fibrosis

Answer

Imperforate anus

Answer

Absence of ureter

Answer

Holoprosencephaly

Question 3

All are true about infantile polycystic kidney disease except?

Accepted Answer

Autosomal dominant

Answer

Hepatic cysts

Answer

Renal cysts are present at birth

Answer

Periporal fibrosis

Question 4

Immune complex-mediated glomerular damage is seen in all of the following except:

Accepted Answer

Focal segmental GN

Answer

Membranoproliferative GN

Answer

Crescentic GN

Answer

RPGN

Question 5

Which subtype of Renal Cell Carcinoma (RCC) has the best prognosis?

Accepted Answer

Chromophobe

Answer

Clear cell

Answer

Papillary

Answer

Collecting duct

Question 6

Casts are produced due to damage to which of the following structures?

Accepted Answer

Distal convoluted tubule (DCT)

Answer

Nephron

Answer

Tubule

Answer

Proximal convoluted tubule (PCT)

Question 7

What is the first manifestation of Alport syndrome?

Accepted Answer

Microscopic hematuria

Answer

Proteinuria

Answer

Oliguria

Answer

Sensorineural deafness

Question 8

Which of the following is NOT associated with Adult Polycystic Kidney Disease?

Accepted Answer

Tricuspid valve prolapse

Answer

Autosomal dominant inheritance

Answer

Mutations involving genes affecting cell-cell-matrix interaction

Answer

Intracranial berry aneurysm may be present

Question 9

A specimen shows a nephrectomy. What is your diagnosis?

Accepted Answer

Renal cell carcinoma

Answer

Renal papillary adenoma

Answer

Urothelial cancer of renal pelvis

Answer

Oncocytoma

Question 10

Which is the most characteristic glomerular nephritis (GN) in HIV patients?

Accepted Answer

Focal Segmental Glomerulosclerosis (FSGS)

Answer

Membranoproliferative Glomerulonephritis (MPGN)

Answer

Minimal Change Disease (MCD)

Answer

Rapidly Progressive Glomerulonephritis (RPGN)

Renal Pathology — MCQs

Renal Pathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?