Vascular Disorders and Tumors

Question 1

What is the endothelial cell marker for angiosarcoma?

Answer 1

CD31

Question 2

What are the risk factors for hepatic angiosarcomas?

Answer 2

VAT
* Vinyl chloride
* Arsenic
* Thorotrast

Question 3

What is the cause of Kaposi Sarcoma?

Answer 3

HHV-8 (Herpesvirus-8)

Question 4

What is the most common HIV-related malignancy?

1000x higher increased risk

Answer 4

AIDS-associated (epidemic) KS

Question 5

Endemic African KS is more common in what patient population?

Answer 5

Younger people

Question 6

What type of bacteria is associated with Bacillary angiomatosis?

Answer 6

GNB

Question 7

What specific bacteria is commonly found to cause Bacillary angiomatosis?

Answer 7

Bartonella henselae

Question 8

How do GNB induce bacillary angiomatosis?

Answer 8

• Bacteria cause host to produce HIF-1α
• Drives VEGF production
• Leads to vascular proliferation

Question 9

Cavernous hemangiomas are associated with what other disease?

Answer 9

Hippel-Lindau disease (CNS)

Question 10

What is the difference between a Cherry hemangioma and a Strawberry hemangioma?

Answer 10

Cherry hemangioma
* Adults, benign, do not regress

Strawberry hemangioma
* Newborns, extremely common, grow for months then regress; gone by 1-7 yo

Question 11

Osler-Weber-Rendu Disease is also called?

Answer 11

Hereditary hemorrhagic telangiectasia

Question 12

HHT shows what type of inheritance pattern?

Answer 12

Autosomal dominant

Question 13

What signaling pathway is disrupted to lead to telangiectasias in HHT?

Answer 13

TGF-β signaling

Question 14

A port wine stain is characteristic of which disease?

Answer 14

Sturge-Weber Syndrome

Question 15

Sturge-Weber Syndrome occurs along the distribution of which nerve?

Answer 15

Trigeminal Nerve

Question 16

S/Sx of Sturge-Weber Syndrome.

Answer 16

• Facial port wine nevi (stain) along trigeminal nerve
• Benign vascular tumor of arachnoid and pia mater (leptomeninges)
• Mental retardation
• Seizures
• Glaucoma

Question 17

What does -ectasia mean?

Answer 17

local dilation of a structure

Question 18

Telangiectasia describes what? Is commonly seen in?

Answer 18

• Permanent dilation of small vessels that form discrete red lesions
• Spider telangiectasias are common to the face, neck, or upper chest
• Most associated with hyperestrogenic states (e.g., pregnancy, cirrhosis)

Question 19

What is nevus flammeus?

Answer 19

• Birthmark in a port-wine/flame coloration
• Due to capillary malformations
• Composed of dilated vessels
• May deepen in color and usually will not fade

Question 20

What is the most common form of vascular ectasias?

Answer 20

Nevus flammeus

Question 21

Benign vascular tumors display what type of cell feature?

Answer 21

monolayer of normal-appearing endothelial cells

Question 22

What is the cause of lymphangitis?

Answer 22

Bacterial seeding of lymphatic vessels
* Inflamed lymphatics are red, painful SQ streaks
* Typically associated with tender enlargement of draining lymph nodes (acute lymphadenitis)

Can appear as ascending streaks as they go to center of body

Question 23

What is the difference between DVT and VTE?

Answer 23

DVT
* Formation of blood clots in deep vein, typically in LE

VTE
* Formation of blood clot in vein (e.g., DVT or PE)

Question 24

Why do **DVTs** go unrecognized?

Answer 24

* In 50% of pts, they lead to **pain** and **edema** * However, in 50% they are asymptomatic due to **venous collateral channels**

Question 25

What is a rare **post-partum DVT**?

Answer 25

Pelvic venous plexus DVT

Question 26

**Portal vein thrombosis** can occur do to?

Answer 26

* Peritoneal infections (peritonitis) * Appendicitis * Pelvic abscesses

Question 27

**Trousseau Syndrome** is associated with what type of vascular manifestation?

Answer 27

Thrombophlebitis migrans

Question 28

Thrombophlebitis is usually associated with what **visceral cancers**?

Answer 28

* Pancreatic adenocarcinoma * Gastric adenocarcinoma

Question 29

How do **visceral cancers** (e.g., pancreatic or gastric adenocarcinomas) cause **thrombophlebitis migrans**?

Answer 29

* Tumor releases **mucin** which reacts with **platelets** leading to **microthrombi** * Recurrent & migratory as it disappears and reoccurs elsewhere

Question 30

What does **phlebitis** mean?

Answer 30

Venous inflammation

Question 31

Venous thrombosis is accompanied by what?

Answer 31

Inflammation

Question 32

What are the **3 H's of Virchow's Triad**?

Answer 32

* Hypercoagulability * Venous stasis * Endothelial wall dysfunction/damage (vessel wall injury)

Question 33

What are characteristics of **chronic venous insufficiency**?

Answer 33

* Leg swelling (edema) * Skin color and texture changes * Venous ulcers

Question 34

How does **chronic venous insufficiency** lead to **ulceration**?

Answer 34

* Varicose veins --> Chronic venous congestion & poor vessel drainage * Leakage of Plasma proteins & edema --> **decreased O2 supply** --> **hypoperfusion** and **hypoxia** that leads to **atrophy** and **ulceration** (stasis dermatitis)

Question 35

How does skin dispigmentation occur in **chronic venous insufficiency**?

Answer 35

Extravasated RBCs --> degradation --> release of **hemosiderin** --> brown skin pigmentation: **yellow-brown** or **red-brown**

Question 36

What is the pathogenesis of **varicose veins**?

Answer 36

**Prolonged venous pressure** --> dysfunction of venous **valves** --> varicose veins

Question 37

What is the mechanism behind **varicose veins** seen in **pregnancy**?

Answer 37

Prolonged **elevation** of venous **pressure** caused by **compression** of **inferior vena cava** by the **gravid uterus** during pregnancy

Question 38

How does **cocaine** cause **vasospastic angina**?

Answer 38

* Leads to increased **SANS output** * Increased **HR, BP, contractility** will lead to an **increase** of **O2 demand** * SANS leads to coronary spasm and **vasoconstriction, thrombi formation** leading to a **decrease** of **O2 demand** * Overall, **SANS** stimulation leads to **ischemia, infarction**, and possibly **death** * Cocaine also **decreases Na+ transport** leading to **arrhythmia** (QT & QRS prolongation) and **decreased LV function** * This leads to possible **death**

Question 39

What is **stress cardiomyopathy**? | E.g., Broken heart syndrome

Answer 39

* Stress induced LV ballooning * Stress --> increased catecholamines --> increased HR & contractility --> vasospasm * Becomes "octopus trap"-like (e.g., Japanese Octopus Trap -- Tako-Tsubo)

Question 40

What is **prinzmetal angina**?

Answer 40

Vasospasm of cardiac arterial or arteriolar beds that may lead to MI (if 20-30 mins duration)

Question 41

**Vasospastic angina** is a toxic effect of which drug?

Answer 41

Cocaine

Question 42

What is the basic histopathological difference between primary and secondary **Reynaud's (RP)**?

Answer 42

* **Primary**: normal capillaroscopic pattern * **Secondary**: abnormal capillaroscopic pattern with a **decrease in capillary density **

Question 43

Describe **secondary RP**.

Answer 43

* ≥ 30 yo * secondary to other diseases (e.g., scleroderma, hyperviscocity, PAD, trauma, β-blockers, EPI, smoking) * Nail fold capillary exam is **abnormal** * **digital ulceration, scarring, tissue death (gangrene)** * **asymmetric**

Question 44

Describe **primary RP**.

Answer 44

* < 30 yo * Idiopathic * Nail fold exam is **normal** * Digital arteries are commonly involved * Usually benign (w/o ulceration or necrosis) * Symmetric

Question 45

In **primary RP** what do the **arteries** and **arterioles** respond to?

Answer 45

Clear lines of demarcation to **cold** or **emotional stress**