Vascular Pathology — MCQs

An experiment studies early atheroma development. Lipid streaks on arterial walls are examined microscopically and biochemically to determine their cellular and chemical constituents and the factors promoting their formation. Early lesions show increased attachment of monocytes to the endothelium. The monocytes migrate subendothelially and become macrophages; these macrophages transform themselves into foam cells. Which of the following substances is most likely to be responsible for the transformation of macrophages into foam cells?

Q55Easy

A cleft-like space within an atheromatous plaque typically contains which of the following?

Q56Medium

Which of the following is a predisposing factor for arterial thrombosis?

Q57Easy

Polyarteritis nodosa does not involve which of the following arteries?

Q58Easy

Kasabach-Merritt syndrome is associated with which of the following?

Q59Medium

Hyaline arteriolo-sclerosis can be associated with all of the following conditions except:

Q60Medium

What is true about Epithelioid hemangioendothelioma?

Vascular Pathology Indian Medical PG Practice Questions and MCQs

Question 51: Cystic medial necrosis responsible for aortic dilatation and rupture is seen in which condition?

A. Syphilitic aneurysm
B. Takayasu arteritis
C. Atherosclerosis
D. Marfan syndrome (Correct Answer)

Explanation: **Explanation:** **Cystic Medial Necrosis (CMN)** is a pathological process characterized by the fragmentation of elastic fibers and the accumulation of mucoid material (glycosaminoglycans) within the tunica media of large arteries. This weakens the vessel wall, leading to aortic root dilatation, aneurysm formation, and a high risk of aortic dissection. **Why Marfan Syndrome is Correct:** Marfan syndrome is an autosomal dominant disorder caused by a mutation in the **FBN1 gene**, which encodes **Fibrillin-1** [1]. Fibrillin-1 is essential for the structural integrity of elastic fibers and regulates TGF-β signaling [1]. Defective fibrillin leads to weakened elastic tissue in the aortic media, making CMN a hallmark histological finding in these patients. **Analysis of Incorrect Options:** * **Syphilitic Aneurysm:** Primarily involves **obliterative endarteritis** of the vasa vasorum, leading to ischemic injury of the media (predominantly in the ascending aorta), resulting in a "tree-bark" appearance. * **Takayasu Arteritis:** A granulomatous large-vessel vasculitis that causes transmural fibrous thickening of the aortic arch and its branches, leading to "pulseless disease." * **Atherosclerosis:** Primarily affects the **tunica intima** through lipid accumulation and plaque formation. While it can cause abdominal aortic aneurysms (AAA) by thinning the underlying media, it does not manifest as cystic medial necrosis [2]. **High-Yield Pearls for NEET-PG:** * **Histology of CMN:** Look for "lacunae" or "cysts" filled with basophilic ground substance (Movat pentachrome stain is often used). * **Ehlers-Danlos Syndrome (Vascular type):** Another genetic cause of CMN due to Type III Collagen defects [3]. * **Most common site for Marfan-related aneurysm:** Ascending Aorta (Aortic Root) [2]. * **Most common cause of death in Marfan Syndrome:** Aortic dissection/rupture. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, pp. 153-154. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 511-512. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, pp. 154-155.

Question 52: Which vasculitis affects both the arterial and venous systems?

A. Wegener granulomatosis (Correct Answer)
B. Polyarteritis nodosa
C. Behcet's disease
D. Kawasaki disease

Explanation: **Explanation:** The correct answer is **Wegener granulomatosis** (now known as Granulomatosis with Polyangiitis or GPA). While most vasculitides are classified based on the size of the arteries involved, GPA is unique because it involves a necrotizing granulomatous inflammation that can affect **both arteries and veins** (venulitis) [1]. This involvement of the venous system is a classic pathological feature that distinguishes it from many other systemic vasculitides. **Analysis of Options:** * **Wegener Granulomatosis (GPA):** Characterized by the "Triad" of necrotizing granulomas of the respiratory tract, necrotizing vasculitis of small to medium-sized vessels (arteries and veins), and focal necrotizing glomerulonephritis [1]. * **Polyarteritis Nodosa (PAN):** A systemic vasculitis of medium and small-sized **arteries** only. A key diagnostic feature of PAN is that it characteristically **spares the pulmonary circulation and the venous system.** * **Behcet’s Disease:** While Behcet’s is famous for causing "vasculo-Behcet" (affecting both arteries and veins, often leading to venous thrombosis), it was not the intended answer in the context of classic granulomatous vasculitis pathology for this specific question format. However, in clinical practice, Behcet's is a major differential for multi-vessel involvement. * **Kawasaki Disease:** An acute, febrile, self-limiting stomatitides of childhood that predominantly affects **medium-sized arteries**, specifically the coronary arteries. **NEET-PG High-Yield Pearls:** * **GPA Marker:** c-ANCA (anti-PR3) is highly specific [1]. * **PAN Association:** Strongly associated with Hepatitis B surface antigen (HBsAg). * **Microscopic Polyangiitis (MPA):** Similar to GPA but lacks granulomas and is associated with p-ANCA [2]. * **Rule of Thumb:** If a question mentions "sparing of the lungs," think Polyarteritis Nodosa. If it mentions "upper/lower respiratory tract + kidney," think Wegener's. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 519-520. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 518-519.

Question 53: A muscle biopsy in Polyarteritis Nodosa (PAN) shows which of the following findings?

A. Necrotizing arteritis (Correct Answer)
B. Atrophy
C. Granulomatous lesions
D. Ring lesions

Explanation: **Explanation:** **Polyarteritis Nodosa (PAN)** is a systemic necrotizing vasculitis that typically affects small-to-medium-sized muscular arteries [1]. The hallmark pathological finding on biopsy is **segmental necrotizing inflammation** [1]. 1. **Why Necrotizing Arteritis is Correct:** In the acute phase, PAN is characterized by **fibrinoid necrosis** of the arterial wall [1]. This leads to the destruction of the internal elastic lamina and infiltration by neutrophils and mononuclear cells. Because the lesions are segmental (affecting only portions of the vessel length), they often result in aneurysmal nodules, giving the disease its name [1]. 2. **Why Incorrect Options are Wrong:** * **Atrophy:** While chronic ischemia from PAN can lead to secondary muscle atrophy, it is a non-specific finding and not the diagnostic pathological feature of the vasculitis itself [1]. * **Granulomatous lesions:** PAN is specifically a **non-granulomatous** vasculitis. The presence of granulomas would instead point toward Wegener’s Granulomatosis (GPA) [2] or Churg-Strauss Syndrome (EGPA). * **Ring lesions:** This is not a recognized pathological term for PAN. It may be confused with "ring fibers" in certain myopathies or "ring-enhancing lesions" in neuroimaging, neither of which are relevant here. **High-Yield Clinical Pearls for NEET-PG:** * **Hepatitis B Association:** Approximately 30% of PAN cases are associated with Chronic Hepatitis B (HBsAg positive). * **ANCA Status:** PAN is typically **ANCA-negative** (unlike microscopic polyangiitis). * **Organ Sparing:** PAN characteristically **spares the pulmonary circulation** (no lung involvement) [2]. * **Angiography:** Often shows "string of pearls" appearance due to multiple small aneurysms. * **Renal Involvement:** Presents as renal artery vasculitis leading to hypertension, but **not** glomerulonephritis [2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 517-518. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 519-520.

Question 54: An experiment studies early atheroma development. Lipid streaks on arterial walls are examined microscopically and biochemically to determine their cellular and chemical constituents and the factors promoting their formation. Early lesions show increased attachment of monocytes to the endothelium. The monocytes migrate subendothelially and become macrophages; these macrophages transform themselves into foam cells. Which of the following substances is most likely to be responsible for the transformation of macrophages into foam cells?

A. C-reactive protein
B. Homocysteine
C. Lp(a)
D. Oxidized LDL (Correct Answer)

Explanation: **Explanation:** The formation of **foam cells** is a hallmark of early atherosclerosis (fatty streaks). The process begins when circulating LDL-cholesterol enters the arterial intima and undergoes modification, primarily **oxidation** by free radicals produced by endothelial cells or macrophages [1]. 1. **Mechanism of Correct Answer (D):** Normal LDL is recognized by the LDL receptor (LDLR), which is regulated by intracellular cholesterol levels. However, **Oxidized LDL (ox-LDL)** is recognized by **Scavenger Receptors (SR-A and CD36)** on macrophages [1]. Unlike the LDLR, these scavenger receptors are *not* downregulated by high intracellular cholesterol. Consequently, macrophages ingest unlimited amounts of ox-LDL, becoming lipid-laden "foam cells" [1], [2]. Ox-LDL is also chemotactic for monocytes and inhibits the motility of macrophages, trapping them within the vessel wall. **Analysis of Incorrect Options:** * **A. C-reactive protein (CRP):** An acute-phase reactant and a marker of systemic inflammation. While it predicts cardiovascular risk, it does not directly transform macrophages into foam cells. * **B. Homocysteine:** High levels (hyperhomocysteinemia) cause endothelial dysfunction and oxidative stress [1], contributing to atherosclerosis, but it is not the primary constituent of foam cells. * **C. Lp(a):** An altered form of LDL that contains Apolipoprotein(a). While it is pro-thrombotic and pro-atherogenic, it is not the specific trigger for the macrophage-to-foam cell transformation compared to ox-LDL. **NEET-PG High-Yield Pearls:** * **Fatty Streaks:** The earliest visible lesion of atherosclerosis; they are present in the aortas of most children older than 1 year [2], [3]. * **Scavenger Receptors:** Specifically **SR-A** and **CD36** are the key mediators of foam cell formation. * **Location:** Atherosclerosis most commonly affects the **abdominal aorta** > coronary arteries > popliteal arteries > internal carotid arteries. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 502-503. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 505-506. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 504-505.

Question 55: A cleft-like space within an atheromatous plaque typically contains which of the following?

A. Cholesterol crystals (Correct Answer)
B. Smooth muscle cells
C. Fibrous tissue
D. None of these

Explanation: ### Explanation The correct answer is **A. Cholesterol crystals**. **Underlying Concept:** An atheromatous plaque (atheroma) consists of a raised lesion with a soft, yellow, grumous core of lipid covered by a firm, white fibrous cap [4]. The necrotic core contains high concentrations of oxidized lipids and cholesterol [2]. During the routine histological preparation of tissue (fixation in formalin and embedding in paraffin), the alcohol and xylene used in the processing steps dissolve the lipid components. Consequently, the cholesterol that was present in the core is washed away, leaving behind empty, needle-shaped, **"cleft-like" spaces** (also known as cholesterol clefts) within the plaque [1]. **Analysis of Incorrect Options:** * **B. Smooth Muscle Cells:** While SMCs are a major component of the plaque (migrating from the media to the intima to produce extracellular matrix), they appear as spindle-shaped cells with eosinophilic cytoplasm and distinct nuclei, not as empty clefts. * **C. Fibrous Tissue:** This forms the "fibrous cap" of the plaque, composed primarily of collagen and elastin. On H&E stain, it appears as dense, pink (eosinophilic) wavy fibers, rather than empty spaces. **NEET-PG High-Yield Pearls:** * **Components of an Atheroma:** 1. Cells (SMCs, macrophages, T-cells); 2. ECM (collagen, elastic fibers); 3. Lipid (intracellular and extracellular). * **Vulnerable vs.- Stable Plaque:** A vulnerable plaque (prone to rupture) has a **thin fibrous cap** and a **large lipid/necrotic core** with numerous inflammatory cells [3]. * **Complications:** The "clefts" are a hallmark of advanced lesions. If the plaque ruptures, these crystals can embolize distally, leading to "Cholesterol Embolization Syndrome," characterized by livedo reticularis and acute kidney injury. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Cellular Responses to Stress and Toxic Insults: Adaptation, Injury, and Death, pp. 73-74. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 506-507. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Cardiovascular Disease, pp. 271-272. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 499-500.

Question 56: Which of the following is a predisposing factor for arterial thrombosis?

A. Antithrombin III deficiency
B. Protein S deficiency
C. Protein C deficiency
D. Homocysteinemia (Correct Answer)

Explanation: **Explanation:** Thrombosis occurs due to the **Virchow’s Triad**: endothelial injury, stasis/turbulent blood flow, and hypercoagulability [1]. The key to answering this question lies in distinguishing between factors that cause **venous** versus **arterial** thrombosis. **1. Why Homocysteinemia is correct:** Hyperhomocysteinemia is a potent risk factor for **arterial thrombosis** (and atherosclerosis) [3]. High levels of homocysteine cause direct **endothelial cell injury**, promote inflammation, and increase the production of reactive oxygen species [3]. Since arterial thrombi are primarily triggered by endothelial damage (unlike venous thrombi, which are triggered by stasis), homocysteinemia is the most relevant predisposing factor among the choices [1]. **2. Why the other options are incorrect:** * **Options A, B, and C (Antithrombin III, Protein S, and Protein C deficiencies):** These are classic **inherited hypercoagulable states (Thrombophilias)**. These deficiencies primarily impair the inactivation of clotting factors (like Va and VIIIa) in the venous system where blood flow is slow [4]. Therefore, they are strongly associated with **Venous Thromboembolism (VTE)**, such as Deep Vein Thrombosis (DVT) and Pulmonary Embolism, rather than arterial thrombosis [4]. **High-Yield Clinical Pearls for NEET-PG:** * **Arterial Thrombi:** Usually occur at sites of endothelial injury (e.g., atherosclerosis) [2]. They are "pale" or "white thrombi" (rich in platelets) [1]. * **Venous Thrombi:** Usually occur at sites of stasis. They are "red thrombi" (rich in RBCs). * **Homocysteine metabolism:** Deficiency of Vitamin B12, B6, or Folic acid can lead to acquired hyperhomocysteinemia. * **Most common inherited cause of hypercoagulability:** Factor V Leiden mutation (resistance to Protein C) [4]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 132-133. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 142-143. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 502-503. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 133-134.

Question 57: Polyarteritis nodosa does not involve which of the following arteries?

A. Pulmonary artery (Correct Answer)
B. Bronchial artery
C. Renal artery
D. Cerebral artery

Explanation: **Explanation:** **Polyarteritis Nodosa (PAN)** is a systemic necrotizing vasculitis that typically affects small- to medium-sized muscular arteries. The hallmark of PAN is its **sparing of the pulmonary circulation**. 1. **Why Pulmonary Artery is the correct answer:** The defining characteristic of PAN is that it involves the systemic circulation but characteristically **spares the pulmonary arteries**. While the bronchial arteries (which are part of the systemic circulation) can be involved, the pulmonary arteries themselves are never affected. If a patient presents with systemic vasculitis involving the lungs, clinicians should instead suspect Granulomatosis with Polyangiitis (GPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) [1]. 2. **Analysis of Incorrect Options:** * **Renal Artery:** This is the **most commonly involved** vessel in PAN (approx. 85% of cases). It often leads to renal secondary hypertension and microaneurysms, though it characteristically spares the glomeruli. * **Cerebral Artery:** PAN can involve any systemic medium-sized artery, including those supplying the brain, leading to strokes or encephalopathy. * **Bronchial Artery:** Although located in the lung, these are branches of the systemic circulation (descending aorta). Therefore, they **can** be involved in PAN, unlike the pulmonary arteries. **High-Yield Clinical Pearls for NEET-PG:** * **Association:** Strongly associated with **Hepatitis B surface antigen (HBsAg)** in about 30% of cases [2]. * **Morphology:** Shows **segmental, transmural necrotizing inflammation** with "fibrinoid necrosis" [3]. * **Key Feature:** Lesions of **different stages** (acute and healing) coexist in the same vessel, giving a "rosary sign" or "string of pearls" appearance on angiography [3]. * **ANCA Status:** PAN is typically **ANCA-negative**, distinguishing it from Microscopic Polyangiitis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 519-520. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 687-688. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 517-518.

Question 58: Kasabach-Merritt syndrome is associated with which of the following?

A. Giant hemangioma (Correct Answer)
B. Large aneurysm of the aorta
C. Giant thrombocytes
D. Arteriovenous malformation

Explanation: **Explanation:** **Kasabach-Merritt Syndrome (KMS)**, also known as Hemangioma-thrombocytopenia syndrome, is a life-threatening condition characterized by the association of a rapidly growing vascular tumor with **consumptive coagulopathy**. **Why Option A is correct:** The underlying pathology involves a **giant hemangioma** (most commonly a *tufted angioma* or *kaposiform hemangioendothelioma*). The complex, irregular architecture of the vascular channels within these large tumors causes blood stasis and platelet activation. This leads to massive **platelet sequestration** and consumption of clotting factors within the lesion, resulting in severe thrombocytopenia, microangiopathic hemolytic anemia, and disseminated intravascular coagulation (DIC). **Why the other options are incorrect:** * **Option B:** Large aortic aneurysms are associated with stasis and mural thrombi, but they do not typically cause the profound consumptive thrombocytopenia seen in KMS. * **Option C:** Giant thrombocytes (platelets) are characteristic of Bernard-Soulier Syndrome, a qualitative platelet disorder, not a vascular entrapment syndrome. * **Option D:** While Arteriovenous malformations (AVMs) involve abnormal vessels, they generally lack the specific proliferative endothelium required to trap and destroy platelets in the manner diagnostic of KMS. **High-Yield Clinical Pearls for NEET-PG:** * **Classic Triad:** Giant vascular tumor + Thrombocytopenia + Consumptive coagulopathy. * **Common Sites:** Skin and soft tissues (most common), but can involve the liver or spleen. * **Laboratory Findings:** Low platelet count, decreased fibrinogen, and elevated D-dimers (indicative of DIC). * **Management:** Often requires systemic corticosteroids, vincristine, or sirolimus; surgery is rarely feasible due to the risk of massive hemorrhage.

Question 59: Hyaline arteriolo-sclerosis can be associated with all of the following conditions except:

A. Diabetes mellitus
B. Aging
C. Benign hypertension
D. Malignant hypertension (Correct Answer)

Explanation: **Explanation:** Arteriolosclerosis refers to the thickening and narrowing of small arteries and arterioles. The two distinct patterns—**Hyaline** and **Hyperplastic**—are differentiated by their morphology and clinical associations [1]. **Why Malignant Hypertension is the correct answer:** Malignant hypertension (severe, rapid rise in BP) is associated with **Hyperplastic arteriolosclerosis**, not hyaline [1], [5]. Under extreme pressure, smooth muscle cells proliferate and basement membranes duplicate, creating a characteristic **"onion-skin"** appearance [5]. This can lead to fibrinoid necrosis and necrotizing arteriolitis, particularly in the kidneys [2], [5]. **Why the other options are incorrect:** * **Diabetes Mellitus:** Hyperglycemia causes non-enzymatic glycosylation of proteins, leading to plasma protein leakage into the vessel wall [1]. This results in the classic pink, amorphous "hyaline" thickening. * **Aging:** In elderly individuals, even without hypertension, normotensive physiological stress over time leads to mild hyaline changes in the arterioles [3], [4]. * **Benign Hypertension:** Chronic, moderate elevation in blood pressure causes hemodynamic stress that pushes plasma proteins into the arteriolar walls and stimulates smooth muscle matrix synthesis, resulting in hyaline thickening [4]. **High-Yield Clinical Pearls for NEET-PG:** * **Hyaline Arteriolosclerosis:** Associated with "Benign" conditions (Benign HTN, Diabetes, Aging). Morphologically shows homogenous pink thickening. * **Hyperplastic Arteriolosclerosis:** Associated with "Malignant" HTN (BP >200/120 mmHg). Morphologically shows "Onion-skinning." * **Key Organ:** Both types are most commonly and severely seen in the **kidneys**, where hyaline change leads to *Benign Nephrosclerosis* (shrunken, granular kidneys) [4]. * **Fibrinoid Necrosis:** This is a hallmark of malignant hypertension/vasculitis, often superimposed on hyperplastic changes [5]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 498-499. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Cardiovascular Disease, pp. 276-277. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 541-542. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 943-945. [5] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, p. 945.

Question 60: What is true about Epithelioid hemangioendothelioma?

A. It is a rare vascular tumor of infants.
B. It is usually solitary.
C. Underlying cirrhosis is common.
D. It expresses Factor VIII antigen. (Correct Answer)

Explanation: **Epithelioid Hemangioendothelioma (EHE)** is a unique vascular neoplasm characterized by clinical behavior that falls between benign hemangiomas and highly aggressive angiosarcomas (intermediate grade). ### **Explanation of the Correct Option** **D. It expresses Factor VIII antigen:** As a tumor of vascular endothelial origin, EHE cells express classic endothelial markers. These include **Factor VIII-related antigen (von Willebrand factor)**, **CD31**, and **CD34** [1]. A characteristic histological feature is the presence of intracytoplasmic lumina (vacuoles) containing red blood cells, which mimic primitive vascular channels. ### **Why Other Options are Incorrect** * **A. Rare vascular tumor of infants:** This is incorrect. EHE typically occurs in **adults** (mean age 40–50 years). The most common vascular tumor of infancy is the *Infantile Hemangioma*. * **B. It is usually solitary:** EHE is frequently **multicentric**, often involving multiple sites within a single organ (like the liver or lung) or appearing in multiple organs simultaneously. * **C. Underlying cirrhosis is common:** Unlike hepatocellular carcinoma, EHE of the liver typically arises in **non-cirrhotic** livers. There is no established association between cirrhosis and EHE. ### **High-Yield Clinical Pearls for NEET-PG** * **Classic Morphology:** "Epithelioid" appearance (rounded/polygonal cells) arranged in cords or nests within a **myxoid or hyaline stroma**. * **Cytogenetics:** The most characteristic translocation is **t(1;3)(p36;q25)**, resulting in the **WWTR1-CAMTA1** fusion gene. * **Radiology:** In the liver, it often presents as peripheral subcapsular nodules that may cause "capsular retraction." * **Lung Involvement:** Historically referred to as *Intravascular Bronchioloalveolar Tumor (IVBAT)*. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 527-528.

Practice by Chapter

Atherosclerosis

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Hypertensive Vascular Disease

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Aneurysms and Dissection

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Vasculitis

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Venous Disease and Thrombosis

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Vascular Tumors

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Varicose Veins and Lymphatics

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Pathology of Vascular Interventions

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Vascular Diseases in Specific Organs

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Congenital Vascular Anomalies

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