Renal Pathology Practice Questions

Q: Autosomal dominant mutations in which one of the following genes may cause focal segmental glomerulosclerosis associated with abnormal genitalia, Wilms tumor and mental retardation?

WT1. ***WT1*** - Mutations in the **WT1 (Wilms tumor 1) gene** are associated with **Denys-Drash syndrome** and **Frasier syndrome**, both of which feature **focal segmental glomerulosclerosis (FSGS)**, abnormal genitalia, and an increased risk of **Wilms tumor**. - **Denys-Drash syndrome** specifically includes **glomerulopathy**, **pseudohermaphroditism** (abnormal genitalia), and **Wilms tumor**, often with some degree of mental retardation. *INF2* - Mutations in the **INF2 gene** are a common cause of **autosomal dominant FSGS**, often without extra-renal manifestations. - While it causes FSGS, it typically does not present with abnormal genitalia, Wilms tumor, or mental retardation. *LMX1B* - Mutations in the **LMX1B gene** are responsible for **Nail-Patella Syndrome**, which is characterized by abnormalities of the nails, patellae, elbows, and iliac horns. - It can cause FSGS, but it is not associated with abnormal genitalia, Wilms tumor, or mental retardation. *APOL1* - **APOL1 gene variants** (G1 and G2 risk alleles) are strongly associated with a higher risk of developing **FSGS** and other kidney diseases, particularly in individuals of African ancestry. - While it is a significant genetic risk factor for FSGS, APOL1 mutations are not linked to abnormal genitalia, Wilms tumor, or mental retardation.

Q: All the following are features of Polycystic disease of kidneys EXCEPT:

Erythrocytosis. ***Erythrocytosis*** - While other renal conditions like **renal cell carcinoma** can cause erythrocytosis due to increased **erythropoietin** production, it is generally **not a typical feature** of Polycystic Kidney Disease (PKD). - Patients with PKD usually have **normal or even low erythropoietin levels** despite compromised kidney function, and anemia is more common, particularly as **renal failure progresses**. *Renal failure* - **Progressive cyst growth** leads to replacement of normal kidney parenchyma, inevitably culminating in **end-stage renal disease** [1] in the majority of patients. - This is a hallmark feature, often necessitating **dialysis or transplant** later in life for individuals with autosomal dominant polycystic kidney disease (ADPKD) [2]. *Haematuria* - **Gross or microscopic hematuria** is a common symptom in PKD, often resulting from **cyst rupture** [1], bleeding into a cyst, or the passage of a calculus due to urinary stasis. - It can be a presenting symptom and can cause significant pain and anxiety for patients. *Hypertension* - **Hypertension** is an early and frequent complication of PKD, often preceding any significant decline in glomerular filtration rate. - It is primarily caused by activation of the **renin-angiotensin-aldosterone system (RAAS)** [3] due to arterial compression and ischemia from expanding cysts. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 951-955. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 544-545. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 520-521.

Q: A patient presents with a renal mass leading to hematuria and flank pain. The histological image below is shown. What is the likely diagnosis? ![img-31.jpeg](img-31.jpeg)

Clear Cell Carcinoma. ***Clear Cell Carcinoma*** - The image displays cells with **clear cytoplasm** due to the accumulation of lipids and glycogen, a classic histological feature of **Clear Cell Renal Cell Carcinoma** [1]. - These cells often have **small, round nuclei** and are arranged in nests or cords, supported by a rich vascular network, which aligns with the image provided [1]. *Papillary Cell Carcinoma* - This subtype is characterized by cells arranged in **papillary structures** with fibrovascular cores, which is not clearly evident in the image [1]. - The cells typically have **basophilic cytoplasm** and generally do not exhibit the extensive clear cytoplasm seen here. *Chromophobe Renal Cell Carcinoma (RCC)* - Chromophobe RCC is characterized by cells with **pale, flocculent cytoplasm** and prominent cell membranes, often appearing like a "plant cell" pattern [1]. - The nuclei are typically irregular and often contain perinuclear halos, which are not the predominant features in the provided image [1]. *Bellini Duct Carcinoma* - Also known as collecting duct carcinoma, this is a rare and aggressive subtype originating from the medullary collecting ducts. - Histologically, it features a **tubulointerstitial growth pattern**, often with an infiltrative appearance and significant desmoplasia, distinctly different from the clear cell morphology shown. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 958-961.

Q: A patient presents with a renal mass leading to hematuria and flank pain. The histological image below is shown. What is the likely diagnosis?

Clear Cell Carcinoma. ***Clear Cell Carcinoma*** - **Clear cell carcinoma** is the most common type of renal cell carcinoma, classically presenting with a triad of **hematuria**, **flank pain**, and a **palpable mass**. - Histologically, it is characterized by cells with **clear cytoplasm** due to lipid and glycogen accumulation, forming nests or cords, often with a rich **vascular network** [1]. *Chromophobe Renal Cell Carcinoma (RCC)* - This subtype typically presents with **eosinophilic cytoplasm** and prominent cell membranes, often with perinuclear halos, which is distinct from the clear cytoplasm seen in the image [1]. - It generally has a **better prognosis** and differs genetically from clear cell carcinoma [1]. *Bellini Duct Carcinoma* - Also known as **collecting duct carcinoma**, this is a rare and aggressive subtype originating from the medullary collecting ducts. - It usually presents with a **poorly differentiated infiltrative growth pattern** and high-grade nuclear features, which are not typical of the image provided. *Papillary Cell Carcinoma* - Histologically, **papillary renal cell carcinoma** is characterized by cells arranged in a papillary or tubulopapillary architecture with fibrovascular cores [1]. - The cells typically have basophilic cytoplasm and are not noted for the prominent clear cell appearance seen in the image. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 959-961.

Q: A kidney biopsy under electron microscopy shows subepithelial 'spike' formation. Which immunofluorescence pattern would confirm membranous nephropathy?

Granular IgG along GBM. ***Granular IgG along GBM*** - **Membranous nephropathy** is characterized by the immune complexes forming **subepithelial deposits** along the glomerular basement membrane (GBM) [1], [2]. - These deposits appear as **granular IgG** (and often C3) on immunofluorescence, corresponding to the "spike" formation seen on electron microscopy [1]. *Linear IgG along GBM* - This pattern is characteristic of **Goodpasture syndrome** (anti-GBM disease), where antibodies directly bind to the GBM in a uniform, linear fashion [3], [4]. - The electron microscopy findings in Goodpasture syndrome do not show subepithelial "spike" formation but rather a normal or thickened GBM. *Mesangial IgA deposits* - This is the hallmark of **IgA nephropathy** (Berger's disease), where IgA immune complexes accumulate predominantly in the mesangium [2]. - IgA nephropathy would not typically present with subepithelial "spike" formation on electron microscopy. *C3 deposits only* - While C3 can be present in membranous nephropathy, finding "C3 deposits only" without significant IgG or other immunoglobulins suggests conditions like **C3 glomerulopathy** or dense deposit disease. - These conditions have distinct electron microscopy findings and a different pathogenesis compared to membranous nephropathy. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, p. 921. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, p. 911. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, p. 909. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 526-527.

Q: In Goodpasture syndrome, which organ is involved apart from the lung?

Kidney. ***Kidney*** - Goodpasture syndrome is an **autoimmune disease** that primarily targets the a3 chain of **type IV collagen**, which is found in the **basement membranes** of both the glomeruli in the kidneys and the alveoli in the lungs. [1] - This leads to rapidly progressive **glomerulonephritis** and **pulmonary hemorrhage**, making the kidney a key organ involved alongside the lungs. [1] *Heart* - The heart is generally **not directly involved** in Goodpasture syndrome. - Cardiac symptoms are typically **secondary** to severe anemia from pulmonary hemorrhage or fluid overload from kidney failure. *Spleen* - The spleen is **not a target organ** for the autoantibodies in Goodpasture syndrome. - While it plays a role in immune responses, it is not directly damaged by the disease process itself. *Liver* - The liver is **not affected** by the autoantibodies in Goodpasture syndrome. - **Type IV collagen**, the autoantigen, is not a significant component of the liver basement membranes.

Q: Crescent forming glomerulonephritis is:-

RPGN. ***RPGN*** - **Rapidly progressive glomerulonephritis (RPGN)** is a clinical syndrome characterized by a rapid decline in **renal function** over weeks to months, often due to severe glomerular injury. - The hallmark **histological feature** of RPGN is the formation of **crescents** in more than 50% of the glomeruli, which are proliferations of parietal epithelial cells and infiltrating macrophages [1]. *MCN* - **Minimal change nephropathy (MCN)** is characterized by **diffuse effacement of podocyte foot processes** on electron microscopy, with normal findings on light microscopy. - It typically presents as **nephrotic syndrome** and does not involve crescent formation. *MPGN* - **Membranoproliferative glomerulonephritis (MPGN)** involves thickening of the glomerular basement membrane with a "tram-track" appearance and mesangial proliferation [2]. - While MPGN can occasionally have focal crescents in some cases, **crescent formation is not a defining or characteristic feature** of MPGN [2]. - MPGN typically presents with nephritic-nephrotic syndrome. *All of the options* - This option is incorrect because only RPGN is characterized by **crescent formation** as a defining feature. - MCN does not involve crescents, and MPGN does not characteristically present with extensive crescent formation, thus invalidating this choice. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 528-529. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 925-926.

Question 1

Autosomal dominant mutations in which one of the following genes may cause focal segmental glomerulosclerosis associated with abnormal genitalia, Wilms tumor and mental retardation?

Accepted Answer

WT1

Answer

INF2

Answer

LMX1B

Answer

APOL1

Question 2

All the following are features of Polycystic disease of kidneys EXCEPT:

Accepted Answer

Erythrocytosis

Answer

Renal failure

Answer

Haematuria

Answer

Hypertension

Question 3

A patient presents with a renal mass leading to hematuria and flank pain. The histological image below is shown. What is the likely diagnosis?
![img-31.jpeg](img-31.jpeg)

Accepted Answer

Clear Cell Carcinoma

Answer

Papillary Cell Carcinoma

Answer

Chromophobe Renal Cell Carcinoma (RCC)

Answer

Bellini Duct Carcinoma

Question 4

Identify the diagnosis from the given image

Accepted Answer

Malakoplakia

Answer

TB

Answer

BCC

Answer

Drug induced lesion

Question 5

A patient presents with a renal mass leading to hematuria and flank pain. The histological image below is shown. What is the likely diagnosis?

Accepted Answer

Clear Cell Carcinoma

Answer

Chromophobe Renal Cell Carcinoma (RCC)

Answer

Bellini Duct Carcinoma

Answer

Papillary Cell Carcinoma

Question 6

A renal biopsy shows 'bamboo spine' pattern in arterioles. Which immunofluorescence finding would best support thrombotic microangiopathy?

Accepted Answer

Minimal/negative immunofluorescence staining

Answer

Mesangial IgA deposits

Answer

Linear IgG deposits

Answer

Granular C3 deposits

Question 7

A kidney biopsy shows 'tram-track' appearance on silver stain. Which immunofluorescence pattern would best support membranoproliferative glomerulonephritis?

Accepted Answer

Granular C3 and IgG deposits

Answer

Granular C3 deposits

Answer

Mesangial IgA deposits

Answer

Fibrillar deposits

Question 8

A kidney biopsy under electron microscopy shows subepithelial 'spike' formation. Which immunofluorescence pattern would confirm membranous nephropathy?

Accepted Answer

Granular IgG along GBM

Answer

Linear IgG along GBM

Answer

Mesangial IgA deposits

Answer

C3 deposits only

Question 9

In Goodpasture syndrome, which organ is involved apart from the lung?

Accepted Answer

Kidney

Answer

Heart

Answer

Spleen

Answer

Liver

Question 10

Crescent forming glomerulonephritis is:-

Accepted Answer

RPGN

Answer

MCN

Answer

All of the options

Answer

MPGN

Renal Pathology — MCQs

Renal Pathology — MCQs

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