Vascular Disorders and Tumors Flashcards
What is the endothelial cell marker for angiosarcoma?
CD31
What are the risk factors for hepatic angiosarcomas?
VAT
* Vinyl chloride
* Arsenic
* Thorotrast
What is the cause of Kaposi Sarcoma?
HHV-8 (Herpesvirus-8)
What is the most common HIV-related malignancy?
1000x higher increased risk
AIDS-associated (epidemic) KS
Endemic African KS is more common in what patient population?
Younger people
What type of bacteria is associated with Bacillary angiomatosis?
GNB
What specific bacteria is commonly found to cause Bacillary angiomatosis?
Bartonella henselae
How do GNB induce bacillary angiomatosis?
- Bacteria cause host to produce HIF-1α
- Drives VEGF production
- Leads to vascular proliferation
Cavernous hemangiomas are associated with what other disease?
Hippel-Lindau disease (CNS)
What is the difference between a Cherry hemangioma and a Strawberry hemangioma?
Cherry hemangioma
* Adults, benign, do not regress
Strawberry hemangioma
* Newborns, extremely common, grow for months then regress; gone by 1-7 yo
Osler-Weber-Rendu Disease is also called?
Hereditary hemorrhagic telangiectasia
HHT shows what type of inheritance pattern?
Autosomal dominant
What signaling pathway is disrupted to lead to telangiectasias in HHT?
TGF-β signaling
A port wine stain is characteristic of which disease?
Sturge-Weber Syndrome
Sturge-Weber Syndrome occurs along the distribution of which nerve?
Trigeminal Nerve
S/Sx of Sturge-Weber Syndrome.
- Facial port wine nevi (stain) along trigeminal nerve
- Benign vascular tumor of arachnoid and pia mater (leptomeninges)
- Mental retardation
- Seizures
- Glaucoma
What does -ectasia mean?
local dilation of a structure
Telangiectasia describes what? Is commonly seen in?
- 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)
What is nevus flammeus?
- Birthmark in a port-wine/flame coloration
- Due to capillary malformations
- Composed of dilated vessels
- May deepen in color and usually will not fade
What is the most common form of vascular ectasias?
Nevus flammeus
Benign vascular tumors display what type of cell feature?
monolayer of normal-appearing endothelial cells
What is the cause of lymphangitis?
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
What is the difference between DVT and VTE?
DVT
* Formation of blood clots in deep vein, typically in LE
VTE
* Formation of blood clot in vein (e.g., DVT or PE)
Why do DVTs go unrecognized?
- In 50% of pts, they lead to pain and edema
- However, in 50% they are asymptomatic due to venous collateral channels
What is a rare post-partum DVT?
Pelvic venous plexus DVT
Portal vein thrombosis can occur do to?
- Peritoneal infections (peritonitis)
- Appendicitis
- Pelvic abscesses
Trousseau Syndrome is associated with what type of vascular manifestation?
Thrombophlebitis migrans
Thrombophlebitis is usually associated with what visceral cancers?
- Pancreatic adenocarcinoma
- Gastric adenocarcinoma
How do visceral cancers (e.g., pancreatic or gastric adenocarcinomas) cause thrombophlebitis migrans?
- Tumor releases mucin which reacts with platelets leading to microthrombi
- Recurrent & migratory as it disappears and reoccurs elsewhere
What does phlebitis mean?
Venous inflammation
Venous thrombosis is accompanied by what?
Inflammation
What are the 3 H’s of Virchow’s Triad?
- Hypercoagulability
- Venous stasis
- Endothelial wall dysfunction/damage (vessel wall injury)
What are characteristics of chronic venous insufficiency?
- Leg swelling (edema)
- Skin color and texture changes
- Venous ulcers
How does chronic venous insufficiency lead to ulceration?
- 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)
How does skin dispigmentation occur in chronic venous insufficiency?
Extravasated RBCs –> degradation –> release of hemosiderin –> brown skin pigmentation: yellow-brown or red-brown
What is the pathogenesis of varicose veins?
Prolonged venous pressure –> dysfunction of venous valves –> varicose veins
What is the mechanism behind varicose veins seen in pregnancy?
Prolonged elevation of venous pressure caused by compression of inferior vena cava by the gravid uterus during pregnancy
How does cocaine cause vasospastic angina?
- 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
What is stress cardiomyopathy?
E.g., Broken heart syndrome
- Stress induced LV ballooning
- Stress –> increased catecholamines –> increased HR & contractility –> vasospasm
- Becomes “octopus trap”-like (e.g., Japanese Octopus Trap – Tako-Tsubo)
What is prinzmetal angina?
Vasospasm of cardiac arterial or arteriolar beds that may lead to MI (if 20-30 mins duration)
Vasospastic angina is a toxic effect of which drug?
Cocaine
What is the basic histopathological difference between primary and secondary Reynaud’s (RP)?
- Primary: normal capillaroscopic pattern
- Secondary: abnormal capillaroscopic pattern with a **decrease in capillary density **
Describe secondary RP.
- ≥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
Describe primary RP.
- < 30 yo
- Idiopathic
- Nail fold exam is normal
- Digital arteries are commonly involved
- Usually benign (w/o ulceration or necrosis)
- Symmetric
In primary RP what do the arteries and arterioles respond to?
Clear lines of demarcation to cold or emotional stress
