Chapter 11: Veins/Lymph, DVT, Vascular Tumors Flashcards

1
Q

Which 3 types of vessels are most often affected by vasculitis?

A
  • Arterioles
  • Capillaries
  • Venules
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Why is distinguishing between infectious and immune-mediated causes of vasculitis important as far as tx goes?

A
  • Immune-mediated can be treated w/ immunosuppressive therapy
  • This tx would be counter-productive for infectious causes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How can a non-infectious vasculitis be indirectly triggered by an infection?

A

Generation of immune complexes or triggering cross-reactive immune response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Infectious causes of vasculitis do so through ________ invasion

A

Direct

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Which non-infectious vasculitis is the only one to affect the aorta amongst other vessels?

A

Giant Cell (Temporal) Arteritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Which lesions are most common for Drug Hypersensitivity Vasculitis?

A

Skin lesions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Immune complex-associated vasculitis may be seen in which systemic immunologic disorder?

A

SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

A pt >40 yo with history ± polymyalgia rheumatica is most likely to have what type of vasculitis?

A

Giant Cell (Temporal) Arteritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Immune complex vasculitis is due to the production of what?

What deposits in the vascular walls?

A
  • Production of autoantibodies and formation of immune complexes
  • Deposition of antigen-antibody complexes in vascular walls
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Why is it important to consider Drug Hypersensitivity as a cause of vasculitis as far as treatment goes?

A

Stop drug –> resolution of vasculitis!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are very useful diagnostic markers of non-infectious vasculitis and their titers mirror clinical severity?

A

ANCAs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

MPO-ANCA’s are found in what 2 types of non-infectious vasculitis?

A
  • Microscopic polyangiitis
  • Churg-Strauss syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

ANCAs can directly activate which immune cells and cause the release of?

A

Neutrophils, stimulating release of ROS and proteolytic enzymes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Why are ANCA-associated vasculitides often described as “pauci-immune”?

A

ANCA Ab directed against cellular constituents and do NOT form circulating immune complexes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Giant cell arteritis stems from an immune response by which cells and causes inflammation of arteries where?

A
  • T cell mediated (CD4+ > CD8+)
  • Inflammation of arteries in the head, especially temporal a.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Which 2 types of antibodies are seen in 2/3 of pt’s with Giant Cell (temporal) Arteritis?

A

anti-endothelial cell and anti-SM cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What do the classic lesions associated with Giant Cell (Temporal) Arteritis exhibit morphologically?

A
  • Granulomatous inflammation w/ multinucleated giant cells
  • Fragmentation of the elastic lamina and intimal thickening
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are the common signs/sx’s of Giant Cell (Temporal) Arteritis?

A
  • Facial pain or HA, most intense along course of superficial temporal a.; painful to palpation***
  • Diplopia or complete vision loss
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Why is biopsy of at least a 1-cm segment required for diagnosis of Giant Cell (Temporal) Arteritis?

A

As it can be extremely PATCHY and FOCAL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Treatment for Giant Cell (Temporal) Arteritis?

A

Corticosteroidsoranti-TNF therapies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Granulomatous vasculitis of medium and large arteries characterized by ocular disturbances and marked weaking of the pulses and low BP in the UE’s is characteristic of?

A

Takayasu Arteritis (aka pulseless disease)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Takayasu arteritis has similar histological findings as those seen in giant cell arteritis, except involves which vessels?

A

Aortic arch (aortitis) and major branch vessels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Since many features of Takayasu arteritis are shared with Giant Cell arteritis, the distinction between the 2 is primarily made how?

A
  • AGE of the pt
  • Takayasu = younger (<50 yo)
  • Giant cell = older (>50 yo)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Which vessels are the major ones affected in Takayasu Arteritis?

A
  • Aortic arch and major branch vessels
  • Pulmonary a. (1/2 of cases)
  • Coronary and renal arteries
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Major signs/sx's associated with Takayasu Arteritis? Sx's associated w/ involvement of renal, pulmonary, and distal aorta?
- **Reduced BP** and **pulses** in carotids and **UE's** - Ocular disturbances + Neuro deficits - If **distal aorta** involved --\> **claudication of legs** - **Pulmonary a.** involvement --\> **pulmonary HTN** - **Renal a.** involvement --\> **systemic HTN**
26
In 1/3 of pt's with polyarteritis nodosa the vasculitis is attributable to chronic infection by which virus? Immune-complexes composed of what?
**HBsAg** and **anti-HBsAg =** **Chronic HBV**
27
List the 4 vessels most often involved in Polyarteritis Nodosa in descending order of frequency.
- Kidneys - Heart - Liver - GI tract
28
Vessels of which organ are characteristically spared in Polyarteritis Nodosa?
Pulmonary
29
Lesions seen in Polyarteritis Nodosa involve which part of the vessel and have a predileciton for which areas?
Only **part** of the vessel circumference w/ **predilection** for **branch points**
30
Classic Polyarteritis Nodosa is characterized by what morphological pattern of inflammation?
**Segmental TRANMURAL** **necrotizing** inflammation of small- to medium-sized arteries
31
During acute phase of Polyarteritis Nodosa the transmural inflammation of arterial wall shows a mixed inflammatory infiltrate and is often accompanied by what type of necrosis?
Fibrinoid necrosis
32
Polyarteritis Nodosa is typically a disease affecting which age group? Typical course of the disease?
- Primarily **young adults**, but can also affect **geriatric** and **pediatric** pts - Course is **remitting** and **episodic**, with **long** **sx-free intervals**
33
What is a "classic" presentation (signs/sx's) of Polyarteritis Nodosa?
- **Rapidly accelerating HTN** + abdominal pain + bloody stools - Diffuse **myalgias** and **peripheral neuritis**
34
What type of treatment can yield remissions or cures in 90% of pt's with Polyarteritis Nodosa?
Immunosuppression
35
Acute arteritis affecting large, medium, and small-sized vessels, seen in **infants** and **small children** (\<4 yo) is characteristic of what disease?
Kawasaki disease
36
Clinical significance of Kawasaki Disease stems from its predilection for which artery? Leading to what manifestations?
- **Coronary a.** - Aneurysms ---\> thrombosis or rupture --\> **acute MI**
37
Vascular damage in Kawasaki Disease is primarily mediated by which immune cells?
**T cells** and **monocytes/macrophages**
38
What is the morphology of the vasculitis seen in Kawasaki Disease; differs from PAN how?
Dense **transmural inflammatory infiltrate** w/ **_LESS_** prominent **fibrinoid necrosis** than in **PAN**
39
Kawasaki disease typically presents with what signs/sx's? \*First Aid Mnemonic
**\*CRASH** and **burn** **- C**onjunctival injection - **R**ash (polymorphous -\> **desquamating**) - **A**denopathy (cervical LN enlargement = mucocutaneous LN syndrome) - **S**trawberry tongue (erythema and blistering of oral mucosa) - **H**and-foot changes (**edema** and **erythema**) - **Burn** = FEVER
40
If Kawaksaki Disease is recognized early in its course treatment with what will sharply reduce the risk of symptomatic coronary artery disease?
**IV immunoglobulin** and **Aspirin**
41
Microscopic polyangiitis generally affects which type of vessels?
**Capillaries** + **small arterioles** and **venules**
42
How do the age and distribution of the lesions seen in Microscopic Polyangiitis differ from PAN?
Tend to be of the **same age** and are **distributed more widely**
43
Microscopic polyangiitis affects vessels of many organ systems, but which 3 systems are most common?
- Renal (**necrotizing glomerulonphritis**) - Lungs (**pulmonary capillaritis**) - Skin (**palpable purpura**)
44
Hypersensitivity vasculitis or leukocytoclastic vasculitis are another name for what?
Microscopic Polyangiitis
45
Microscopic polyangiitis can be a feature of which 3 immune disorders?
- Henoch-Schonlein purpura - Essential mixed cryoglobulinemia - Vasculitis assoc. w/ CT disorders
46
**Segmental fibrinoid necrosis** of the **media** and **focal transmural necrotizing** lesions w/ **ABSENCE** **of granulomatous inflammation** is characteristic of what type of vasculitis?
Microscopic polyangiitis
47
Which immune cells are seen infiltrating in **microscopic polyangiitis**, many of which are **undergoing apoptosis** and giving rise to the term **leukocytoclastic vasculitis**?
Neutrophils
48
What are the major clinical features (signs/sx's) seen with microscopic polyangiitis?
- Hemoptysis - Hematuria and Proteinuria - Bowel pain or bleeding - Muscle pain or weakness - Palpable cutaneous purpura
49
Churg-Strauss Syndrome is a vasculitis affecting which size vessels? Classically associated with what systemic manifestations?
- **Small**-vessel **necrotizing** vasculitis - **ASTHMA**, **ALLERGIC rhinitis**, lung infiltrates, **peripheral HYPER-eosinophilia**, and **extravascular necrotizing granulomata**
50
Churgg-Strauss syndrome may have inflammation that resembles PAN or microscopic polyangiitis with the addition of what 2 things?
**Eosinophils** and **Granulomas = Granulomatous, necrotizing vasculitis w/ eosinophilia**
51
What is the primary manifestation of Churg-Strauss Syndrome affecting the kidneys?
Focal and segmental glomerulosclerosis
52
What is the primary manifestation of Churg-Strauss Syndrome affecting the myocardium? Clinically significant why?
- **Cardiomyopathy** w/ **eosinophilic infiltrates** - Accounts for **HALF** the **deaths** in this syndrome
53
Cutanous involvement of Churg-Strauss Syndrome presents with what?
Palpable Purpura
54
Behcet Disease is a vasculitis of small-to medium-vessels what classically presents with what triad?
1) Recurrent **oral** **apthous ulcers** 2) **Genital** ulcers 3) **Uvetis**
55
Which HLA haplotype is associated with Behcet Disease?
HLA-B51
56
Vessel inflammation in Behcet Disease is due to what inflammatory cell type? Recruited by which T cells?
- **Neutrophilic** inflammation - Recruited by **TH17 cells**
57
Mortality seen in Behcet Disease is related to what 2 complications?
- Severe **neurologic** involvement - **Rupture** of **vascular aneurysms**
58
Granulomatosis with Polyangitis (Wegener granulomatosis) is a necrotizing vasculitis characterized by a triad of what?
1) **Necrotizing granulomas** of upper or lower respiratory tract, or both 2) **Necrotizing** or **granulomatous vasculitis** most prominenetly in lungs 3) **Focal necrotizing**, often **CRESCENTERIC, glomerulonephritis**
59
Granulomatosis with polyangiitis is a form of hypersensitivity mediated by what?
**T-cell-mediated** hypersensitivity
60
PR3-ANCAs (aka c-ANCA) is associated with what form of non-infectious vasculitis?
**Granulomatosis** w/ **polyangiitis** (Wegener granulomatosis)
61
Which non-infectious vasculitis is characterized by the presence of granulomas and a dramatic response to immunosuppressive therapy?
Granulomatosis w/ polyangiitis (Wegener granulomatosis)
62
Which sex is more often affected by granulomatosis with polyangiitis and at what age?
**M** \> F; average age **40 yo**
63
What are the classic features (signs/sx's) of granulomatosis with polyangiitis?
- Persistent **pneumonitis** w/ **bilateral nodular** and **cavitary infiltrates** - **Chronic sinusitis** - **Nasopharyngeal ulcerations** - Evidence of **renal disease**
64
How serious is granulomatosis with polyangiitis if left untreated?
**Rapidly fatal** w/ **80% mortality** at **one year**
65
What are the morphological characteristics of the granulomas seen in the upper respiratory tract of someone with granulomatosis with polyangiitis?
Granulomas w/ **geographic patterns of CENTRAL necrosis**
66
The necrotizing granulomas seen in granulomatosis with polyangiitis are surrounded by zones of what? Resembles?
- Zones of proliferating fibroblasts assoc. w/ **giant cells** and **leukocytic infiltrate** - Reminiscent of **mycobacterial** or **fungal** infections
67
What may be seen radiographically in the lower respiratory tract of pt w/ granulomatosis with polyangiitis as multiple necrotizing granulomas coalesce?
Radiographically visible **nodules** that can also **cavitate**
68
Which renal lesions may be seen early on in granulomatosis with polyangiitis? Eventually progress to what in late stages?
- Focal and segmental glomerulonephritis - **Crescenteric** glomerulonephritis
69
Thromboangiitis obliterans (Buerger disease) is characterized by what type of vasculitis and especially in which arteries? Occasional secondary extension to affect what?
- **Segmental**, **thrombosing**, acute and chronic vasculitis of small- and medium-vessels - Principally the **radial** and **tibial** arteries w/ occasional 2' extension to **veins** and **nerves** of the extremities
70
Thromboangiitis obliterans (Buerger disease) occurs almost exclusively in which patients and at what age?
**Heavy cigarette smokers** before **age 35**
71
Most patients with Thromboangiitis obliterans (Buerger disease) have hypersensitivity to intradermally injected what?
Tobacco products
72
What are 3 ethnic groups have higher incidences of Thromboangiitis obliterans (Buerger disease)?
- Israeli - Indian subcontinent - Japanese
73
The acute and chronic inflammation seen in Thromboangiitis obliterans (Buerger disease) is accompanied by what other morphological feature?
**Luminal thrombus** w/ small **microabscesses** composed of **neutrophils** surrounded by **granulomatous** inflammation
74
What are the early manifestations of Thromboangiitis obliterans (Buerger disease)?
- Raynaud's - Leg pain **induced by exercise** that is relieved on rest - **Instep foot pain** induced by exercise - Superficial **nodular phlebitis** (venous inflammation)
75
The vascular insufficiency of Thromboangiitis obliterans (Buerger disease) tends to be accompanied by what type of pain/when? Chronic extremity ulcerations eventually progress to?
- **Severe pain** even at rest - due to **neural** involvement - Progress to **frank gangrene** of extremities
76
Which 3 organisms can directly invade vessels and cause infectious vasculitis?
- *Pseudomonas* - *Aspergillus* - *Mucor*
77
Vascular invasion by organisms resulting in infectious vasculitis typically occurs in association with what, but can also less commonly occur via which route?
- Part of **localized tissue infection** - Less commonly via **hematogenous spread**
78
Vascular infections can weaken arterial walls and can result in what clinically significant events?
- **Mycotic aneurysms** or - Induce **thrombosis** and **infarction** (i.e., thrombosis of meningal vessels in meningitis ---\> infarction of underlying brain)
79
How does Primary Raynaud's vs. Secondary differ in their symmetry of involvement of the digits?
- **Primary** has **symmetrical** involvement of the digits - **Secondary** has **asymmetrical** involvement of the digits
80
Secondary Raynaud's may be a component of other arterial diseases such as what?
- SLE - **Scleroderma** - Thromboangiitis obliterans
81
Excessive vasoconstriction of myocardial arteries or arterioles which may cause ischemia or infarct is known as what?
Cardiac Raynaud
82
What are endogenous and exogenous agnts which are vasoactive mediators and can precipitate prolonged myocardial vessel contraction?
- **Endogenous** = epinephrine, pheochromocytoma - **Exogenous** = cocaine or phenylephrine
83
Varicose veins are abnormally dilated, tortous veines produced by prolonged, increased intraluminal pressure and most often affect which veins?
**Superficial** veins of the upper and lower **legs**
84
**Secondary tissue ischemia** resulting from **chronic venous congestion** and poor vessel drainage results in what skin abnormalities?
- **Stasis dermatitis** (aka "brawny induration") - Ulcerations - Poor wound healing and superimposed infections
85
How frequent do emboli arise from superficial LE veins?
Rarely
86
What is the most serious potential complication associated with DVT's and is often the **first** manifestation of thrombophlebitis?
Pulmonary Embolism
87
Patients with what types of cancer and in which locations are at increased risk for thrombophlebitis, why?
- **Adenocarcinoma** of **lung, ovary, pancreas** - **Mucin**-producing **adenocarcinomas** (mucin is thrombogenic) - May occur as **paraneoplastic** syndrome, related to elaboration of procoagulant factors by tumor cells
88
Which factors associated with slow venous return increase the risk for DVT's?
- Prolonged immobilization = **MAJOR** - Pregnancy - CHF - Obesity
89
In the setting of systemic hypercoagulability, including genetic hypercoagulability, and cancers, what is the classical sign of venous thromboses?
- Appear at one site, dissapear, and then occur at another site - **Migratory thrombophlebitis** (Trousseau sign)
90
What are the major causes of Superior Vena Cava Syndrome?
- **Neoplasms** (i.e., bronchogenic carcinoma or mediastinal lymphoma) - Less commonly **aortic aneurysms**
91
Obstruction associated with superior vena cava syndrome produces what characteristic clinical complex?
- **Marked DILATION** of the veins of the: **head, neck,** and **arms w/ CYANOSIS** - If pulmonary vessels compressed --\> **respiratory distress**
92
Which 2 cancers have a tendency to grow within veins and may ultimately lead to inferior vena cava syndrome? May also be caused by thrombosis from where?
- Hepatocellular carcinoma - Renal cell carcinoma - Thrombosis of the hepatic, renal, or LE veins which propogate cephalad
93
What are the signs/sx's associated with inferior vena cava syndrome? With renal involvement?
- Marked LE **edema** + **distention of superficial collateral veins** of **lower abdomen** - Renal involvement ---\> **MASSIVE proteinuria**
94
Which bacteria is most commonly responsible for Lymphangitis?
group A β-hemolytic strep
95
Red, painful subcutaneous streaks w/ painful enlargement of the draining LN's, is characteristic of what?
Lymphangitis
96
Port wine stains a special form of nevus flammeus, found within the distribution of the trigeminal nerve are associated with what congenital disorder?
**Sturge-Weber Syndrome** (encephalotrigeminal angiomatosis)
97
Sturge-Weber syndrome is associated with what clinical findings?
- **Port wine** **nevi** in trigeminal distribution - **Ipsilateral venous angioma** in **cortical leptomeninges** - **Mental retardation** + Seizures - **Hemiplegia** - Skull radio-opacities
98
What are the common sites for hemangiomas?
- **Skin** and **mucous membranes** of **HEAD** and **NECK** - Liver
99
What is the most common type of hemangioma and where are they found?
- **Capillary** hemangioma - Skin, subcutaneous tissues, and mucous membranes of the **mouth, lips, liver, spleen, and kidneys**
100
Which type of hemangioma is very common in newborns and often presents with multiple lesions, growing rapidly for a few months, and then regressing by age 7?
Juvenile Hemangioma ("strawberry type")
101
How do Cavernous Hemangiomas differ in terms of infiltration and regression as compared to Capillary Hemangiomas?
- Are **more infiltrative** and frequently involve **deep structures** - Do **NOT** spontaneously regress - **Locally destructive!**
102
How do Cavernous Hemangiomas appear histologically?
- **Unencapsulated** mass - Large, cavernous blood-filled vascular spaces - Separated by **CT stroma**
103
What is the characteristic histology of Capillary Hemangiomas?
Thin-walled capillaries w/ **scant stroma**
104
Which type of hemangioma is commonly seen in VHL disease and where are the lesions most often seen?
- Cavernous hemangiomas - Cerebellum, brain stem, retina, pancreas, and liver
105
What are 3 sequelae commonly associated with Cavernous Hemangiomas?
- Dystrophic calcifications - Traumatic ulceration - Intravascular thrombosis
106
Which subtype of capillary hemangioma presents as a rapdily growing red pedunculated lesion on the skin, gingival, or oral mucosa which often ulcerate and bleed easily?
Pyogenic granulomas
107
Simple (capillary) lymphangiomas can arise in the head, neck, and axillary regions and appear very similar to capillary hemangiomas, but can be differentiated how?
Absence of RBC's
108
Cavernous lymphangiomas (cystic hygromas) are typically found where and in whom? Common features?
- **Neck** or **axilla** of **children** - Can occasionally be **enormous** (up to 15 cm) and may fill axilla or produce gross deformities of the neck.
109
Cavernous lymphangiomas (cystic hygromas) are a common feature of which congenital disorder?
Turner Syndrome
110
What are the histologic findings associated with cavernous lymphangiomas (cystic hygromas)?
- **Massively** dilated lymphatic spaces lined by **endothelial cells** - Separated by **intervening CT stroma** containing **lymphoid aggregates**
111
What are the characteristics of Glomus Tumor (Glomangioma)? Arise from what cells and are most often found where?
- **Benign** but VERY **painful** tumor - Arising from **modified smooth m. cells** of the **glomus bodies** - Most commonly in **distal portion** of the **digits**, especially **under fingernails**
112
Bacillary Angiomatosis is a vascular proliferation most commonly arising in whom and is due to what organism?
- Immunocompromised hosts (i.e., AIDS) - **Gram negative bacilli** of ***Bartonella*** family
113
The bacillary angiomatosis induced by *B. henselae* infection is caused by induction of which host factors and upregulation of?
**HIF-1** --\> **↑ VEGF**
114
How do the skin lesions in Bacillary Angiomatosis appear both grossly and microscopically?
- **Grossly** = **red nodules** and **papules**, or **rounded SQ masses** - **Histo** = capillary **proliferation** w/ **plump epitheloid** **endothelial cells** exhibiting nuclear atypia and mitoses
115
What stain can help visualize the *Bartonella* bacilli associated with Bacillary Angiomatosis?
Warthin-Starry Silver
116
The infections and lesions associated w/ Bacillary Angiomatosis can be cleared with what med?
**Macolide antibiotics** (i.e., erythromycin)
117
How does the gross morphology of the lesions associated with Bacillary Angiomatosis differ from Kaposi Sarcoma?
- **Bacillary** = raised lesions - **Kaposi** = flat lesions
118
Classic kaposi sarcoma is a disease most often seen in whom and where?
**Older men** of Mediterranean, Middel Eastern, or Eastern European descent
119
Which types of Kaposi Sarcoma are not associated with HIV?
- **Classic KS** - **Endemic African KS** - **Transplant-associated KS**
120
How does **classic** kaposi sarcoma typically manifest? Most common location and spreads how?
- Multiple **red-purple skin plaques** or **nodules** - Usually in **distal LE**; progressively ↑ in size and number and **spread proximally**
121
Endemic African kaposi sarcoma differs from classic KS in that it more frequently involves what?
Lymph nodes
122
How great of risk are transplant patients at for transplant-associated kaposi sarcoma?
100x ↑ risk
123
Which structures are most often involved in Transplant-Associated KS?
**Lymph nodes**, **viscera**, and **mucosa**
124
What is the most common HIV-related malignancy worldwide?
AIDS-associated (epidemic) KS
125
AIDS-associated (epidemic) KS frequently involves what structures and follows what course?
**Lymph nodes** and **disseminates** widely to **viscera** EARLY in its course
126
The **nodular** lesions seen in KS have what histological features and cells?
**Sheets** of **plump**, proliferating **spindle cells**
127
In classic KS, the cutaneous lesions progress through what 3 stages?
1. **Patches** --\> red, purple macules confined to distal LE's 2. **Raised plaques** --\> as lesions spread prox. 3. **Nodular lesions** --\> distinctly neoplastic; sheets of plump proliferating spindle cells in dermis and SQ tissues
128
Which treatment is usually adequate for excellent prognosis in cases of Classic KS?
Surgical resection
129
Which therapy has greatly reduced the frequency of KS in HIV infected patients?
Anti-retroviral therapy
130
**Vascular tumor** occurring around medium and large-sized **veins** with neoplastic cells that are plump and often **cuboidal** is characteristic of?
Epitheliod Hemangioendothelioma
131
Hepatic angiosarcoma is associated with exposures to what?
- Arsenic (i.e., pesticides) - Thorotrast (radioactive constrast agent) - Polyvinyl Chloride (widely used plastic)
132
Angiosarcomas most often occur in which sex?
**Either** = NO gender predilection; typically **older adults**
133
Angiosarcomas may arise from lymph edema, classically where and in what setting? Arise from which vessels?
- **Ipsilateral** UE years after **radical masectomy** (i.e., w/ LN resection) for **breast cancer** - Arising from **lymphatic vessels** (**lymphangiosarcoma**)
134
How invasive and what is the risk of metastasis for angiosarcomas? 5-year survivial?
- **Locally** invasive and can **readily metastasize** - 5-year survival around **30%**
135
What is the gross morphology of cutaneous angiosarcomas?
- Begin as **multiple** small and asymptomatic **red papules** or **nodules** - Eventually become **large, fleshy masses** of **red-tan** to **gray-white** tissue w/ margins blurring into surrounding structures - Central areas of **necrosis** and **hemorrhage** common
136
In angiosarcomas what kind of differentiation can be seen?
- **ALL** degrees of differentiation may be seen: - From **plump**, atypical endothelial cells **forming** vascular channels - **Wildly undifferentiated** tumors w/ **solid spindled** appearance and **no** discernible blood vessels
137
The **endothelial origin** of Angiosarcomas can be demonstrated with staining for what?
- CD31 - von Willebrand factor
138
Angiosarcomas most commonly is found in which 4 locations?
- Skin - Liver - Breast - Soft tissue
139
Small bore artificial grafts generally fail as a result of what 2 complications? If small bore replacement required, best to use which grafted vessels?
- **Thrombosis** or **intimal hyperplasia** at jct. of the graft - Use **saphenous vein** or **left internal mammary arteries** (best option)