1

 Vascular disease is responsible for higher

morbidity and mortality

 The most clinically significant lesions involve

arteries

 Two principal mechanisms

Narrowing or completely obstructing the

 1.
lumina

 2.Weakening of the walls, leading to dilation

or rupture

2
 Endothelial cells (ECs) and smooth muscle cells
(SMCs) constitute the bulk of vessel wall
cellularity

 The remainder is extracellular matrix (ECM)

(elastin, collagen, and glycosaminoglycans)

3
 Three concentric layers:-
 Intima - consists of an EC monolayer overlying
a thin ECM sheet
Internal elastic lamina
 Media – composed predominantly of SMCs and
ECM
External elastic lamina in some
arteries
 Adventitia - composed of relatively loose
connective tissue, nerve fibers, and smaller
vessels (vessels of the vessels)

4
 Based on size & structure

1. Large, or elastic arteries

-- elastic fibers alternate in layers with SMCs

2. Medium-sized, or muscular arteries

-- the ratio of wall thickness to lumen diameter actually
increases for these vessels

3. Small arteries (≤2 mm in diameter) and

arterioles (20-100 μm in diameter)
-- lie within interstitial connective tissue of organs
5
 Represent the next level of vascular branching
after arterioles

 Approximately the diameter of a red blood cell

(7-8 μm) and have an endothelial cell lining but
no media

 With thin walls (only one cell thick) and slow

flow, they are ideally suited for the rapid
exchange of diffusible substances between blood
and tissue

6
 Relative to corresponding arteries, veins have
larger diameters, larger lumina, and thinner, less
well-organized walls

 Thus, veins are more prone to dilation,

compression, and easy penetration by tumors
and inflammatory processes
 Contains approximately two-thirds of all
systemic blood

7
 Thin-walled, endothelium-lined channels that
drain excess interstitial tissue fluid

 Flow also contains mononuclear inflammatory

cells and host proteins

 These channels can also disseminate disease by

transporting microbes or tumor cells from
distant sites to lymph nodes and eventually to
the systemic circulation

8
 Three are particularly significant
 Developmental, or berry, aneurysms
 occur in cerebral vessels
 Small, spherical dilatations typically in the circle of Willis
 Fear is rupture
 Arteriovenous fistulas
 Abnormal, typically small, direct connections between
arteries and veins that bypass the intervening capillaries
 High-output cardiac failure can occur if it is large
 Fibromuscular dysplasia
 Focal irregular thickening of the walls of medium and large
muscular arteries
 Cause is unknown but is probably developmental
 Focal wall thickenning by combination of irregular medial
and intimal hyperplasia and fibrosis, causing stenosis

9
 Endothelial Cells

 Smooth Muscle Cells

 Intimal Thickening

10
11
 Means "hardening of the arteries“
 arterial wall thickening and loss of elasticity
 Three patterns
 1. Arteriolosclerosis
 affectssmall arteries and arteriolesb - hyaline and
hyperplastic
 Associated with hypertension and/or diabetes
mellitus
 2. Mönckeberg medial calcific sclerosis
 Characterized by calcific deposits in muscular
arteries, older than age 50
 3. Atherosclerosis
 Greek root words for "gruel" and "hardening"
12
 

Arteriosclerosis

Medial calcific
Atherosclerosis sclerosis Arteriolosclerosis
Mönckeberg
elastic aa +++
muscular aa ++ +

arterioles +

site (layer) intima media "wall"

 arteriolosclerosis

diabetes
Arteriolosclerosis
Medial calcific sclerosis
Moenckeberg
 Characterized by intimal lesions called
Atheromas (also called atheromatous or
atherosclerotic plaques), that protrude into
vascular lumina

 Atheroma consists of a raised lesion with a

soft, yellow, grumous core of lipid (mainly
cholesterol and cholesterol esters) covered
by a firm, white fibrous cap.

17
 Ubiquitous among most developed nations

 The prevalence and severity are related to

several risk factors

 Multiple risk factors have a multiplicative effect

 two risk factors increase the risk fourfold & three risk
factors are present the rate of myocardial infarction
is increased seven times

18
Major Risks Potentially
Controllable
Nonmodifiable -Hyperlipidemia 
- Increasing age -Hypertension
- Male gender -Cigarette
- Family history smoking 
-Diabetes
- Genetic
abnormalities

19
Lesser, Uncertain, or Nonquantitated
Risks
Obsesity
Physical inactivity
Postmenopausal estrogen deficiency
Stress
High carbohydrate intake
Lipoprotein
Hardened (trans)unsaturated fat intake
 Age is a dominant influence

20
 Complications of atherosclerosis are
uncommon in premenopausal women unless
they are otherwise predisposed by diabetes,
hyperlipidemia, or severe hypertension.
Why?

21
 The contemporary view of atherogenesis is
expressed by the response-to-injury hypothesis

 This model views atherosclerosis as a chronic

inflammatory response

 Lesion progression occurs through interactions of

 modified lipoproteins, monocyte-derived
macrophages
 T lymphocytes
 normal cellular constituents of the arterial
wall

22
 Chronic endothelial injury
 Insudation of lipoproteins into the vessel wall
 Modification of lesional lipoproteins by oxidation
 Adhesion and migration of blood monocytes (and
other leukocytes) to the endothelium, intima
 Adhesion of platelets.
 Release of factors from activated platelets,
macrophages, or vascular cells
 Proliferation of SMCs in the intima
 Enhanced accumulation of lipids intra and
extracellularly.
23
 Chronic or repetitive endothelial injury is the
cornerstone of the response-to-injury hypothesis
 Inflammation
 In the presence of high-lipid diets, typical is also an
atheromas ensue important
contributor
 However, early human lesions begin at sites of
morphologically intact endothelium

 The two most important causes of endothelial

dysfunction are hemodynamic disturbances and
hypercholesterolemia
24
 1. Chronic endothelial injury to the normal
vessel
 2. Endothelial dysfunction (increase
permeability, leukocyte adhesion)
 3. macrophage activation with smooth muscle
recruitment
 4. Macrophages & SMC engulf lipid with fatty
streak formation
 5. Smooth muscle proliferation, collagen & other
ECM deposition, extra cellular lipid

25
 Lipids are transported in the bloodstream
bound to specific apoproteins (forming
lipoprotein complexes)

 Common lipoprotein abnormalities include

 (1) increased LDL cholesterol levels,

 (2) decreased HDL cholesterol levels, and
 (3) increased levels of the abnormal Lp(a)

26
 Xanthoma

Hypercholesterolemia
Xanthelasma
 Inflammatory cells and mediators are involved in
the initiation, progression, and the complications
of atherosclerotic lesions

 Dysfunctional arterial ECs express adhesion

molecules that encourage leukocyte adhesion;
vascular cell adhesion molecule 1 (VCAM-1) in
particular binds monocytes and T cells

 They migrate into the intima under the influence

of locally produced chemokines

29
 Monocyte changed to macrophages, engulf
lipoprotien

 Macrophage activation results in cytokine

production (e.g., TNF) that further increases
leukocyte adhesion and chemokine
production, produce reactive oxygen species

30
 Activated T cells in the growing intimal lesions
elaborate inflammatory cytokines, (e.g.,
interferon-γ), which in turn can stimulate
macrophages as well as ECs and SMCs

 As a consequence of the chronic inflammatory

state, activated leukocytes and vascular wall
cells release growth factors that promote SMC
proliferation and ECM synthesis

31
foamy macrophage
 Herpesvirus, cytomegalovirus, and Chlamydia
pneumoniae have all been detected in
atherosclerotic plaque but not in normal
arteries

 Seroepidemiologic studies find increased

antibody titers to C. pneumoniae in patients
with more severe atherosclerosis

33
 Fatty Streaks
 composed of lipid-filled foam cells but are not
significantly raised and thus do not cause any
disturbance in blood flow

 begin as multiple minute yellow, flat spots that

can coalesce into elongated streaks, 1 cm long or
longer

 Fatty streaks can appear in the aortas of infants

34
 Coronary fatty streaks begin to form in
adolescence, at the same anatomic sites that
later tend to develop plaques

• Atherosclerotic Plaque

 The key processes in atherosclerosis are intimal

thickening and lipid accumulation

 grossly appear white to yellow; thrombosis

superimposed over the surface of ulcerated
plaques is red-brown in color
35
 The most extensively involved vessels are the lower
abdominal aorta, the coronary arteries, the popliteal
arteries, the internal carotid arteries, and the vessels of
the circle of Willis

 Atherosclerotic plaques have three principal

components:

(1) cells, including SMCs, macrophages, and T cells

(2) ECM, including collagen, elastic fibers, and
proteoglycans and
(3) intracellular and extracellular lipid

36
fatty streaks
38
39
 Rupture, ulceration, or erosion

 Hemorrhage into a plaque

 Atheroembolism

 Aneurysm formation

 Atheromas often undergo calcification

40
 The impact
of the plaque

narrowing
 100%

45% 55%
 cross section
flow

55%
 cross section
flow

5%

55%
47
48
 Cessation of cigarette smoking
 Control of hypertension
 Weight loss
 Exercise and
 Lowering total and LDL blood cholesterol levels
while increasing HDL (e.g., by diet or through
statins ?)

 Reduction should even begin in childhood

49
 use of aspirin (anti-platelet agent)
 statins and
 beta blockers (to limit cardiac demand)

 Surgical interventions (e.g., coronary artery

bypass surgery, carotid endarterectomy)

 These
can successfully reduce recurrent
myocardial or cerebral events

50
 Atherosclerosis
smoking hypertension
injury  endothelium  dysfunction

hyperlipidemia permeability
LDL enter intima

response  phagocytosis by macs

 oxidation of LDL
plaque
 trapping of foam cells
 release of cytokines
narrowing

ischemia
52
 Systemic and local blood pressure
must be tightly regulated

-- Low pressures result in inadequate organ

perfusion, leading to dysfunction and/or tissue
death
-- high pressures result in blood vessel and end-
organ damage

 Elevated blood pressure is called

hypertension
53
 Sustained diastolic pressure greater than 90 mm
Hg, or a sustained systolic pressure in excess of
140 mm Hg, constitutes hypertension ( ≥140/90
mmHg)

 Systolic blood pressure is similarly important

with diastolic blood pressure in determining
cardiovascular risk

 The prevalence and vulnerability to

complications increase with Age

54
 Involving the interaction of multiple genetic and
environmental factors that influence two
hemodynamic variables:-

 Cardiac output is affected by blood volume, itself

strongly dependent on sodium concentrations

 Peripheral resistance is regulated predominantly at

the level of the arterioles and is influenced by neural and
hormonal inputs

55
 The kidneys (primarily) and adrenals
(secondarily) are central players in blood
pressure regulation

 The kidney influences peripheral resistance and

sodium homeostasis primarily through the renin-
angiotensin system

 Renin catabolizes plasma angiotensinogen to

angiotensin I, which in turn is converted to
angiotensin II by angiotensin-converting enzyme
in the periphery

56
 90-95% of hypertension is idiopathic (essential
hypertension), which is compatible with long life

 Most of the remainder of "benign hypertension" is

secondary to renal disease

 About 5% of hypertensive persons show a rapidly rising

blood pressure that if untreated leads to death within 1 or
2 years termed accelerated or malignant hypertension

 Characterized by severe hypertension (diastolic

pressure over 120mmHg), renal failure, and retinal
hemorrhages and exudates, with or without papilledema

57
Essential Hypertension

58
 Accelerate atherogenesis

 Hypertension-associated degenerative
changes can potentiate both aortic dissection
and cerebrovascular hemorrhage

 Associated with two forms of small blood

vessel disease:-

59
 1. Hyaline Arteriolosclerosis

 Encountered frequently in elderly patients

 More generalized and more severe in pts with

hypertension

 The lesions reflect leakage of plasma

components across vascular endothelium and
excessive ECM production by SMCs

60
 2. Hyperplastic Arteriolosclerosis

 Related to more acute or severe elevations of

blood pressure

 Characteristic of malignant hypertension

(diastolic pressures over 120 mm Hg associated
with acute cerebral and/or renal injury)

 Associated with "onion-skin," concentric,

laminated thickening of the walls of arterioles
with luminal narrowing

61
 Aneurysm is a localized abnormal dilation
of a blood vessel or the heart

 All three layers of the arterial wall or the

attenuated wall of the heart - "true"
aneurysm
Eg. Atherosclerotic, syphilitic, and congenital aneurysms,
and ventricular aneurysms that follow transmural
myocardial infarctions

62
 False aneurysm is a breach in the vascular
wall leading to an extravascular hematoma
that freely communicates with the
intravascular space
Eg. ventricular ruptures after myocardial infarctions that
are contained by a pericardial adhesion, or a leak at the
junction of a vascular graft with a natural artery

63
 Aneurysms are classified by macroscopic
shape and size

Saccular aneurysms

Fusiform aneurysms

64
 Atherosclerosis

 Cystic medial degeneration of the arterial media

 Trauma

 Congenital defects (e.g., berry aneurysms)

 Infections (mycotic aneurysms), or syphilis

 Systemic diseases, such as vasculitis

65
 Atherosclerosis, the most common cause

 The media undergoes degeneration and necrosis,

thus allowing the dilation of the vessel

 Atherosclerotic aneurysms occur most frequently

in the abdominal aorta, but the common iliac
arteries, the arch, and descending parts of the
thoracic aorta can also be involved

66
 In men and rarely develops before age 50

 Hereditary defects in structural components of the aorta

can produce aneurysms

 AAA results from an altered balance of collagen

degradation and synthesis

 AAA is associated with local cytokine environments shifted

toward the production of TH2 cytokines

 TH2 cytokines drive macrophages to produce increased

amounts of elastolytic MMPs.

67
 Usually positioned below the renal arteries and
above the bifurcation of the aorta

 Can be as large as 15 cm in diameter, and as

long as 25 cm
 Frequently contains a bland, laminated, poorly
organized mural thrombus that may fill some or
all of the dilated segment

 AAA is accompanied by smaller aneurysms of the

iliac arteries

68
 Two AAA variants merit special mention

 Inflammatory AAAs - dense periaortic fibrosis

containing abundant lymphoplasmacytic infiltrate
with many macrophages and often giant cells. Their
cause is uncertain

 Mycotic AAAs - atherosclerotic lesions infected by

lodging of circulating microorganisms in the wall,
(primary Salmonella gastroenteritis)

69
 Characteristic of the tertiary stage of syphilis
can involve small vessels in any part of the body

 Involvement of the vasa vasorum of the aorta is

particularly devastating; this results in ischemic
medial injury, leading to aneurysmal dilation of
the aorta and aortic annulus, and eventually
valvular insufficiency

70
 Dissection arises when blood enters the wall of
the artery, as a hematoma dissecting between its
layers

 Occurs principally in two epidemiologic groups:

 (1) men aged 40 to 60 years, with antecedent

hypertension (more than 90%), and

 (2) younger patients with systemic or localized

abnormalities of connective tissue affecting the aorta

71
 Hypertension is the major risk factor for
aortic dissection

 Smaller number of dissections is related to

inherited or acquired connective tissue
disorders

 Recognizable medial damage appears to be

neither a prerequisite for dissection nor a
guarantee that dissection is imminent

72
 The intimal tear marking the point of origin
of the dissection is found in the ascending
aorta, usually within 10 cm of the aortic
valve

 The dissection can extend along the aorta

retrograde toward the heart as well as
distally

 Tears are usually transverse or oblique and 1

to 5 cm in length
73
 “Double-barreled aorta”

 Cystic medial degeneration (CMD) is

characterized by elastic tissue fragmentation
and separation of the elastic and SMC
elements of the media by cystic spaces filled
with the amorphous proteoglycan-rich ECM.

74
 Aortic dissections are generally classified into
two types

 The more common proximal lesions (called type A

dissections), involving either the ascending aorta only
or both the ascending and descending aorta (types I
and II of the DeBakey classification)

 Distallesions not involving the ascending part and

usually beginning distal to the subclavian artery
(called type B dissections or DeBakey type III)

75
76
77
 Inflammation of vessel walls, occurs in
diverse clinical settings

 The two most common pathogenic

mechanisms of vasculitis are

 Immune-mediated inflammation

 Direct
invasion of vascular walls by infectious
pathogens

78
 main immunologic mechanisms that initiate
noninfectious vasculitis are

 (1) immune complex deposition

 (2)
antineutrophil cytoplasmic antibodies
(ANCAs)

 (3) anti-endothelial cell antibodies

79
 Antibody and complement are typically
detected in vasculitic lesions

 Although the nature of the antigens

responsible for such deposition cannot
usually be determined

 DNA-anti-DNA complexes in systemic lupus

erythematosus (SLE)

 Drug hypersensitivity

80
 Circulating antibodies that react with neutrophil
cytoplasmic antigens

 Two types
 Cytoplasmic localization (c-ANCA), wherein the most
common target antigen is proteinase-3 (PR3), a
neutrophil granule constituent
 Perinuclear localization (p-ANCA), wherein most of
the autoantibodies are specific for myeloperoxidase
(MPO)
 e.g., inflammatory bowel disease, primary sclerosing

cholangitis, and rheumatoid arthritis

81
 Antibodies to ECs may predispose to certain
vasculitides

 For example - Kawasaki disease

82
83
 A chronic, typically granulomatous inflammation
of large to small-sized arteries

 Principally affects the arteries in the head-

especially the temporal arteries-but also the
vertebral and ophthalmic arteries, as well as the
aorta (giant-cell aortitis)

 Pathogenesis - a T cell-mediated immune

response to an unknown, possibly vessel wall,
antigen.

84
 Temporal arteritis occurs only rarely in persons
younger than 50 years of age

 Symptoms may be only vague and constitutional-

fever, fatigue, weight loss-or may involve facial
pain or headache

 Ocular symptoms (ophthalmic arteritis)

 Diagnosis depends on biopsy and histologic

confirmation

85
86
 Polyarteritis nodosa (PAN) is a systemic
vasculitis of small or medium-sized muscular
arteries, typically involving renal and visceral
vessels but sparing the pulmonary circulation

Morphology
 Characterized by segmental transmural
necrotizing inflammation of small to medium-
sized arteries

87
 usually involve only part of the vessel
circumference, at branch points

 The inflammatory process weakens the arterial

wall and lead to aneurysms

 During the acute phase, there is transmural

inflammation of the arterial wall with a mixed
infiltrate of neutrophils, eosinophils, and
mononuclear cells, frequently accompanied by
fibrinoid necrosis

88
 Disease primarily of young adults

 Malaise, fever, and weight loss;

hypertension, usually developing rapidly;
abdominal pain and melena (bloody stool)

 Diffuse muscular aches and pains; and

peripheral neuritis, predominantly affecting
motor nerves

89
 necrotizing vasculitis characterized by a
triad of
 Acute necrotizing granulomas of the upper
respiratory tract (ear, nose, sinuses, throat) or
the lower respiratory tract (lung) or both
 Necrotizing or granulomatous vasculitis
affecting small to medium-sized vessels,most
prominent in the lungs and upper airways
 Renal disease in the form of focal necrotizing,
often crescentic, glomerulonephritis

90
 Cell-mediated hypersensitivity response, possibly
to an inhaled infectious or other environmental

 c-ANCAs are present in up to 95% of cases; they

are a useful marker of disease activity, and may
participate in disease pathogenesis

 Following immunosuppressive treatment, a rising

c-ANCA titer suggests a relapse

91
92
 Males are affected more

 Average age of about 40 years

 Classical features include persistent pneumonitis
with bilateral nodular and cavitary infiltrates
(95%), chronic sinusitis (90%), mucosal
ulcerations of the nasopharynx (75%), and
evidence of renal disease (80%)
 Other features include rashes, muscle pains,
articular involvement, mononeuritis or
polyneuritis, and fever

93
 Results from an exaggerated vasoconstriction of digital
arteries and arterioles

 These vascular changes induce paroxysmal pallor or

cyanosis of the digits of the hands or feet; infrequently,
the nose, earlobes, or lips can also be involved

 Prevalence 3% to 5% and a predilection for young women

 Ulceration and ischemic gangrene are rare

94
95
 Varicose Veins
 Abnormally dilated, tortuous veins produced by
prolonged increase in intraluminal pressure and
loss of vessel wall support

 superficial veins of the upper and lower leg are

typically involved

 A familial tendency toward premature

varicosities results from imperfect venous wall
development

96
97
 show wall thinning at the points of maximal
dilation with smooth muscle hypertrophy and
intimal fibrosis ; elastic tissue degeneration
and spotty medial calcifications
(phlebosclerosis) also occur

 Focal intraluminal thrombosis (due to stasis)

and venous valve deformities (rolling and
shortening) are common

98
 venous valves incompetent and leads to
stasis, congestion, edema, pain, and
thrombosis

 secondary ischemic skin changes including

stasis dermatitis and ulcerations

 esophageal varices & forming hemorrhoids

99
 deep leg veins account for more than 90% of
cases

 In patients with cancer, particularly

adenocarcinomas, hypercoagulability occurs as a
paraneoplastic syndrome related to tumor
elaboration of procoagulant factors. In this
setting, venous thromboses classically appear in
one site, disappear, and then reoccur in other
veins, so-called migratory thrombophlebitis
(Trousseau sign)

100
 Thrombi in the legs tend to produce local
manifestations, including distal edema, cyanosis,
superficial vein dilation, heat, tenderness,
redness, swelling, and pain

 Homan sign

101
 SVS is usually caused by neoplasms that
compress or invade the superior vena cava,
resulting marked dilation of the veins of the
head, neck, and arms with cyanosis, respiratory
distress

 IVC obstruction induces marked lower extremity

edema, distention of the superficial collateral
veins of the lower abdomen, and-with renal vein
involvement-massive proteinuria

102
 Primary disorders are extremely uncommon

 secondary processes - inflammation or

malignancies

 Lymphangitis is the acute inflammation

elicited when bacterial infections; the most
common agents are group A β-hemolytic
streptococci

103
 Milky accumulations of lymph in various spaces
are designated

 chylous ascites (abdomen)

 chylothorax, and

 Chylopericardium

 these are caused by rupture of dilated

lymphatics, typically obstructed secondary to an
infiltrating tumor mass

104
105
 Hemangioma

 Characterized by increased numbers of normal or

abnormal vessels filled with blood

 These lesions constitute 7% of all benign tumors

of infancy and childhood

 Many of the capillary lesions eventually regress

spontaneously

 Malignant transformation occurs rarely

106
 Occur in the skin, subcutaneous tissues, and
mucous membranes of the oral cavities and lips,
as well as in the liver, spleen, and kidneys

 The "strawberry type," or juvenile hemangioma

of the skin of newborns is extremely common
and may be multiple

 It grows rapidly in the first few months but then

fades at 1 to 3 years of age and completely
regresses by age 7

108
 Bright red to blue with the surface of the
skin or slightly elevated and have an intact
overlying epithelium

109
 large, dilated vascular channels
 Are less well circumscribed and more frequently
involve deep structures
 No spontaneous tendency to regress
Morphology
 soft, spongy masses 1 to 2 cm in diameter; rare
giant forms

110
 Rapidly growing peduncular red nodule on the
skin, gingival, or oral mucosa

 The proliferating capillaries are often

accompanied by extensive edema and an acute
and chronic inflammatory infiltrate

 Pregnancy tumor (granuloma gravidarum) is a

pyogenic granuloma that occurs in the gingiva of
1% of pregnant women

111
112
 Lymphangiomas are the benign lymphatic
analogue of hemangiomas

 1. Simple (Capillary) Lymphangioma

 2. Cavernous Lymphangioma (Cystic Hygroma)

113
 Benign but often exquisitely painful tumors
arising from modified SMCs of the glomus
body, a specialized arteriovenous structure
involved in thermoregulation

 commonly found in the distal portion of the

digits

115
116
 local dilation of preexisting vessels; they are
not true neoplasms

 Telangiectasia is a term used for a

congenital anomaly or acquired exaggeration
of preformed vessels-usually in the skin or
mucous membranes

117
 “Birthmark” and is the most common form of
ectasia

 Histologically, there is only vascular dilation; most

ultimately regress

 Port wine stain is a special form of nevus flammeus;

these lesions tend to grow with a child, thicken the
skin surface, and demonstrate no tendency to fade

118
 Non-neoplastic vascular lesion grossly
resembles a spider

 Associated with hyperestrogenic states such

as pregnancy or cirrhosis

 Commonly seen on the face, neck, or upper

chest

119
 Autosomal dominant disorder

 Telangiectasias are malformations composed of

dilated capillaries and veins

 Present from birth, they are widely distributed

over the skin and oral mucous membranes, as
well as in the respiratory, GI, and urinary tracts

120
 Opportunistic infection in immunocompromised
persons that manifests as vascular proliferations
involving skin, bone, brain, and other organs

 Liver and spleen called bacillary peliosis

 Two species are implicated:-

 Bartonella henselae (cat-scratch disease)

 B. Quintana (trench fever)

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 Common in patients with acquired
immunodeficiency syndrome (AIDS)

1. Chronic KS (classic or European KS)

 was first described by Kaposi in 1872

 occurs in older men of Eastern European

 Canbe associated with an underlying second

malignancy or altered immunity, it is not
associated with human immunodeficiency virus
(HIV)
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2. Lymphadenopathic KS (African or
endemic KS)
 prevalent among South African, it is also
not associated with HIV

3. Transplant-associated KS

4. AIDS-associated (epidemic) KS

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 Human Herpesvirus 8 [HHV-8] or KS-associated
herpesvirus [KSHV] was identified in a cutaneous
KS lesion in an AIDS patient

Clinical Course

 Classic KS is-at least initially-largely restricted to

the surface of the body

 Disseminated KS

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Characteristic Morphology
 Three stages

1. Patch
2. Plaque
3. Nodule
 Malignant endothelial neoplasms

 Histology varying from highly differentiated

tumors that resemble hemangiomas
(hemangiosarcoma) to anaplastic lesions difficult
to distinguish from carcinomas or melanomas

 Older adults are more commonly affected, with

equal gender predilections; they occur at any
site but most often involve skin, soft tissue,
breast, and liver

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 Cutaneous angiosarcomas can begin as deceptively small,
sharply demarcated, asymptomatic, often multiple red
nodules; most eventually become large, fleshy masses of
red-tan to gray-white tissue

 The margins blend imperceptibly with surrounding

structures. Central areas of necrosis and hemorrhage are
frequent

 Microscopically, all degrees of differentiation can be seen,

from plump, anaplastic but recognizable ECs producing
vascular channels to wildly undifferentiated tumors

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 Hemangiopericytomas are rare tumors derived from
pericytes-myofibroblast-like cells that are normally
arranged around capillaries and venules

 Hemangiopericytomas can occur as slowly enlarging,

painless masses at any anatomic site, but they are most
common on the lower extremities (especially the thigh)
and in the retroperitoneum

 They consist of numerous branching capillary channels and

gaping sinusoidal spaces enclosed within nests of spindle-
shaped to round cells

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