Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.

ca) 1

Lecture Hour 16: Vascular Pathology

Lecturer: Dr. Teitlebaum

Buerger Disease
• Buerger disease, a nonatherosclerotic vascular
disease also known as thromboangiitis obliterans
(TAO)
• The first reported case of thromboangiitis
obliterans was described in Germany by von
Winiwarter in an 1879 article titled "A strange form
of endarteritis and endophlebitis with gangrene of
the feet
o Inflammation in the arteries and veins
causing a disturbance of circulation,
leading to gangrenous changes in the
foot

• Clinical Features:
o the absence or minimal presence of
atheromas
o segmental vascular inflammation
o vasoocclusive phenomenon
o involvement of small- and medium-sized
arteries and veins of the upper and lower
extremities
o The typical presentations are rest pain, unremitting ischemic ulcerations, and
gangrene of the digits of hands and feet, and as the disease evolves, the
patients may require several surgical amputations
o Severe pain (claudication) in the affected extremity on excretion

• Epidemiology:
o Young males, usually under 40 old
o The condition is strongly associated with heavy tobacco use, and
progression of the disease is closely linked to continued use
o Common in Israel, India, Japan, and South America

• Pathology:
o Recurrent neutrophilic vasculitis with micro-abscesses
o Segmental thrombosis – vascular insufficiency
• Acute-phase lesions include an occlusive, highly cellular, inflammatory thrombus
with less inflammation in the walls of the blood vessels. Polymorphonuclear
leukocytes, microabscesses and multinucleated giant cells may exist
• Intermediate phase of disease there is progressive organization of the thrombus in
the arteries and veins
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 2

• When only organized thrombus and fibrosis are found in the blood vessels, the
phase is considered to be end-stage

Kawasaki Disease
• Aka Kawasaki syndrome, lymph node syndrome and mucocutaneous lymph
node syndrome
• Kawasaki disease is an acute systemic vasculitis of unknown cause that primarily
affects children under 5 years of age. It was first described in Japan in 1967 by
Dr. Tomisaku Kawasaki and colleagues
• Leading cause of acquired heart disease in children in the developed world,
surpassing acute rheumatic fever in the United States
• Epidemiology:
o infants and young children (age <4)
o Japan, Hawaii, and US mainland
o Mean annual incidence of Kawasaki disease across Canada is about 13
per 100000 children less than 5 years of age

• Clinical manifestations:
o Acute febrile illness
o Conjunctivitis, erythema and erosions of the oral mucosa
o Generalized maculopapular skin rash
o Lymphadenopathy

• Unfortunately, these clinical findings are nonspecific and are commonly found in
many pediatric infectious and immunologic diseases
• Further complicating the diagnosis is the fact that these clinical features may be
absent or may evolve over many days after fever onset
• Distribution of disease:
o large, medium-sized and small arteries
o Coronary artery commonly affected -70% cases

Kawasaki Disease: Strawberry tongue

 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 3

Kawasaki Disease: Conjunctivitis

Kawasaki Disease: Cervical lymphadenopathy

Kawasaki disease: rash

• Pathology:
o Segmental necrotizing vasculitis
o Weakened vascular wall- aneurysm formation
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 4

• Prognosis:
o Self-limited course
o Mortality rate: 1-2% due to rupture of coronary aneurysm or coronary
thrombosis
• Dx: Cardiac duplex sonography
• Tx: Intravenous gamma globulin (IVGG) and acetylsalicylic acid (ASA) - within 10
days of fever onset

Kawasaki Disease: Coronary Aneurysms

• Coronary artery aneurysms occurring in up to 25% of untreated cases.
• Risk factors:
o Male child
o Age of 1 year
o Fever for more than 10 days
• Outcomes:
o myocardial ischemia due to coronary thrombosis
o aneurysm rupture- mortality
o Early (25 yo) onset of CAD in adults
• Most coronary artery aneurysms regress within a year after the acute phase of
Kawasaki disease

Raynaud Disease
• Named after French physician Maurice Raynaud (1834–
1881)
• Primary Raynaud Phenomenon (Raynaud Disease)
o Young women
o Episodic small artery vasospasm in extremities,
nose, or ears
o Results in blanching and cyanosis of fingers or toes
o Precipitated by cold temperatures and emotions
o No underlying disease of pathology

Secondary Raynaud Phenomenon

• Arterial insufficiency secondary to an underlying disease
• 4 potential underlying diseases:
o Scleroderma – CREST
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 5

o SLE
o Buerger Disease
o Atherosclerosis
• Other primary causes:
o Drugs that cause narrowing of arteries, such as amphetamines, certain
types of beta-blockers, some cancer drugs, ergot compounds
o Repeated injury, particularly from vibrations such as those caused by
typing or playing the piano
o Smoking
o Frostbites
o Thoracic outlet syndrome: as a result of compressed subclavian artery

Venous Disease
• Deep vein thrombosis (DVT)
• Clinical features: deep leg veins – 90% iliac, femoral, popliteal veins
o Often asymptomatic: commonly missed diagnosis
o Unilateral leg swelling, warmth, erythema
o Homan sign – increased resistance to passive dorsiflexion of the ankle by
examiner
§ Don’t squeeze calfà this could dislodge the thrombosis and cause
it to move.
• Diagnosis: Doppler “duplex” ultrasound
• Complications: thromboembolism – Pulmonary Embolism!

Varicose veins
• Dilated torturous veins caused by increased intraluminal pressure
• Superficial veins of the lower extremities: lack of structural support of surrounding
superficial tissues and/or venous valvular incompetence
o Female>male, common in pregnancy
o Occurs in 15% of the US population
o Aggravated by prolonged standing or sitting (long flights)
o Edema, thrombosis, stasis dermatitis, ulcerations

Saphenous Veins Varicose

 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 6

Varicose veins: stasis dermatitis

Esophageal Varicesà
This info is in the posted slides, but will not be on the Mod 9 exam, we will go
through this more in the GI unit!!

Hemorrhoids
• Hemorrhoids are not varicosities; they are clusters of vascular tissue (e.g., arterioles,
venules, arteriolar-venular connections), smooth muscle (e.g., Treitz muscle), and
connective tissue lined by the normal epithelium of the anal canal
• Hemorrhoids are present in utero and persist through normal adult life. Evidence
indicates that hemorrhoidal bleeding is arterial and not venous. This evidence is
supported by the bright red color and arterial pH of the blood.
• Hemorrhoids are classified by their anatomic origin within the anal canal and by their
position relative to the dentate line; thus, they are categorized into internal and
external hemorrhoids (see the following image).
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 7

Vessel Development, Growth, and Remodeling

• Vasculogenesis is the de novo formation of blood vessels during
embryogenesis.
• Vascular endothelial growth factor (VEGF) - primary growth factor involved in
this process.
• Angiogenesis (or neovascularization) constitutes the process of new vessel
formation in the mature organism.
• Arteriogenesis refers to the remodeling of existing arteries in response to
chronic changes in pressure or flow, and results from an interplay of endothelial
cell- and smooth muscle cell-derived factors.

Congenital Anomalies of the Vessels

• Developmental or berry aneurysms (BA) occur in cerebral vessels – circle of
Willis, ruptured BA can be causes of fatal intracerebral hemorrhage
• Arteriovenous fistulas - abnormal, typically small, direct connections between
arteries and veins that bypass the intervening capillaries:
o developmental defects or result from rupture of an arterial aneurysm into
an adjacent vein.
o penetrating injuries that pierce arteries and veins
o inflammatory necrosis of adjacent vessels
o intentionally created arteriovenous fistulas are used to provide vascular
access for chronic hemodialysis.
o Clinical significance: similar to berry aneurysms, ruptured arteriovenous
fistulas can be an important cause of intracerebral hemorrhage, large or
extensive arteriovenous fistulas become clinically significant by shunting
blood from the arterial to the venous circulations and forcing the heart to
pump additional volume; high-output cardiac failure can ensue.
• Fibromuscular dysplasia is a focal irregular thickening of the walls of medium
and large muscular arteries, including renal, carotid, splanchnic, and vertebral
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 8

vessels.
o The cause is unknown but is probably developmental; first-degree
relatives of affected individuals have an increased incidence
o Segments of the vessel wall are focally thickened by a combination of
irregular medial and intimal hyperplasia and fibrosis; this results in luminal
stenosis, and in the renal arteries may be a cause of renovascular
hypertension
o Vascular outpouchings (aneurysms) may develop in the vessel
segments with attenuated media and in some cases can rupture
o Fibromuscular dysplasia can manifest at any age, although it is seen
most frequently in young women; there is no association with use of oral
contraceptives or abnormalities of sex hormone expression

Arteriosclerosis
• Aka “hardening” or sclerosis of the arteries
• Three main forms:
o Mönckeberg medial calcification
o Arteriolosclerosis
o Atherosclerosis

Mönckeberg Medial Calcification

• Aka Mönckeberg arteriosclerosis, is a condition that leads to the stiffening of the
elastic layer of the arterial wall, but in contrast to intimal artery calcification, it does
not obstruct the arterial lumen
• Etiology: Unknown
• Pathology: Dystrophic calcification of the media of muscular arteries
• Major lower limb arteries of elderly patients: radial and ulnar arteries affected often
• Also be seen in the head, neck and pelvis, especially the uterine arteries
• Commonly occurs in peripheral arteries of the lower limbs where it is seen as "rail
tracking" on incidental plain radiograph
• Stenosis and atheroma may occur but the lumen usually remains patent, leading to
"pipe-stem" rigidity.
• MMC does not involve primarily the intimal layer of the artery,
• Arterial lumen is kept open (patent) by the rigid media, luminal narrowing is not a
direct consequence
• MCC is a risk factor for cardiovascular disease and peripheral artery obstruction
• MCC is frequently related to glucose intolerance, aging, male gender, autonomic
neuropathy, osteoporosis and, chronic renal failure
• MMC as visualized by plain soft-tissue radiograms is a powerful and easily
recognizable risk marker for markedly increased cardiovascular events unrelated to
conventional risk factors

Mönckeberg Medial Calcification

 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 9

Arteriolosclerosis
• Chronic endothelial cell injury and endothelial dysfunction
• Two-step process:
o Endothelial Injury
o Tissue response of the vascular wall to the injurious agents
o Formation of plaque - decades
• Causes of endothelial injury:
o Hyperlipidemia
o Hypertension
o Toxins in cigarette smoke
o Elevated level of homocysteine
o Hemodynamic forces –turbulent blood flow
• Pathogenesis
o Endothelial cells regulate vasodilation, vasoconstriction, hemostasis,
prevent intravascular coagulation and thrombosis.
o Endothelial dysfunction promotes:
§ thrombosis
§ increased vascular permeability.
§ monocytes and lymphocytes adhesion to the surface and
intraintimal migration
§ high permeability for macromolecules - LDL
Atherogenesis
• Intraintimal macrophages (monocytes) release LDL oxidizing enzymes
• Oxidized LDL is engulfed by macrophages and SMC via scavenger receptors –
foam cells
• SMC: contractile role in the mediaà secretory role in intima – release
extracellular matrix (collagen)
• Plaque development
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 10

Atherosclerosis
• AS begins as an intimal process, medial changes are secondary to the intima.
• Macrophages engulfed LDL undergo apoptosis and release oxidized LDL
causing intracellular and extracellular lipid to accumulate within plaque.
• Elevated levels of high-density lipoproteins (HDL) are protective.
• Men tend to have lower levels of HDL than women.
• Men are more prone to cardiovascular morbidity and mortality than non-
menopausal women.
• HDL substitutes LDL from the wall of the vessel
• Regular exercise demonstrated to increase HDL levels.
• Moderate alcohol consumption also demonstrated to increase HDL levels and
associated with lower cardiovascular and all-cause mortality.
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 11

Schematic of intimal thickening, emphasizing smooth muscle cell migration and

proliferation within the intima, with associated ECM synthesis. Intimal smooth muscle
cells may derive from the underlying media or may be recruited from circulating
precursors; they are shown in a different color from the medial cells to emphasize that
they have a proliferative, synthetic, and noncontractile phenotype distinct from medial
smooth muscle cells.

• Pathology
o Fatty streak: intimal accumulation of foam cells
o Atheroma: intimal accumulation of foam cells and extracellular lipid
o Fibroatheroma: atheroma with development of a fibrous cap
Atherosclerosis: Risk Factors
• Major and modifiable risk factors
o Smoking
§ This is the biggest risk factor (Exam Question)à we can medically
modify other major risk factors, but if the patient does not stop
smoking then everything is null and void
o Diabetes mellitus
o Hypertension
o Hyperlipidemia/obesity
• Metabolic syndrome: a group of metabolic risk factors : insulin-resistance,
hyperlipidemia, hypertension, abdominal obesity
• Minor and non-modifiable risk factors:
o Male gender
o Female gender after menopause (same as male)
o Advanced age
o Family History of CVD
o Sedentary lifestyle
o Stress
• Other risk factors:
o Chlamydophila pneumoniae infection: intimal injury
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 12

o Elevated level of homocysteine, lipoprotein A (LpA) can cause thrombotic

predisposition

Atherosclerosis: Favorite sites

• Elastic and medium-site muscular arteries
• Most severity at ostia (plural from ostium) – turbulent blood flow. Laminar flow
protects vessels from development of atherosclerosis
• Descending order of prominence of vascular involvement:
• Abdominal aorta
• Coronary arteries
• Popliteal Arteries
• Internal carotid arteries
• Circle of Willis
• Interesting fact: patients with severe CAD can have a relatively disease-free
aorta

Complications of atherosclerosis
• Occlusion of vessel
• Disruption of plaque, thrombus formation, vessel wall dissection
• Emboli: plaque breaks free and carried with blood stream: strokes
• aneurysm formation (AAA)
• Peripheral vascular disease: claudication!

Systemic Hypertension

• Elevated BP leading to end-organ damage.

• Sustained DP >90 mm Hg
• Systolic pressure > 140 mm Hg
• Incidence: 25% of population
• African Americans> Caucasians: risk increase with age.
• Etiology:
o Idiopathic or essential primary HTN – 95% cases
o Secondary – 5%
§ Renal: renin-angiotensin-aldosterone system activation
§ Endocrine: Cushing syndrome, Conn syndrome (aldosterone-
secreting tumor), acromegaly, pheochromocytoma,
hyperthyroidism, hypothyroidism
§ Cardiovascular: coarctation of aorta, polyarteritis nodosa, increased
intravascular volume, increased cardiac output
§ Neurologic: increased intracranial pressure, sleep apnea, brain
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 13

tumors
§ Stress-induced: pain, anxiety, hyperglycemia, acute and chronic
stress (high-strung type A personality)
o Gestational HTN: 20 weeks gestation: preeclampsia, eclampsia, HELLP
syndrome

Benign Hypertension
• Slow clinical course
• Aka idiopathic
• 95% cases
• “Despite “benign” all forms of HTN associated with increased morbidity and
mortality
• Mild to moderate (120-160 mm Hg) elevation in BP
• End-organ damage
• No organ is spared
• Microscopically: hyaline arteriosclerosis
• Late manifestations:
o Concentric left ventricular hypertrophy
o Congestive heart failure (CHF)
o Accelerated atherosclerosis
o Myocardial infarction (MI)
o aneurysm formation, rupture and dissection
o Intracerebral hemorrhage (major risk factor)
o Chronic renal failure

Malignant Hypertension
• Malignant hypertension most often occurs in patients with long-standing
uncontrolled hypertension, many of whom have discontinued antihypertensive
therapy. Malignant hypertension is usually associated with a diastolic pressure
above 120 mmHg - 5% of the cases
• Malignant hypertension most often occurs in patients with long-standing
uncontrolled hypertension, many of whom have discontinued antihypertensive
therapy.
• Severe hypertension and ensuing rise in pressure in the arterioles and capillaries
lead to damage to the vascular wall.
• Malignant Hypertension
• Disruption of the vascular endothelium then allows plasma constituents (including
fibrinoid material) to enter the vascular wall, thereby narrowing or obliterating the
vascular lumen – hyperplastic arteriosclerosis or “onion skin”
• Activation of the renin-angiotensin system, may contribute to the development of
fibrinoid necrosis – necrotizing arteriolitis
• Malignant nephrosclerosis is one of many complications of HTN which leads to
acute kidney injury, hematuria, and proteinuria.
• Renal biopsy reveals fibrinoid necrosis in the arterioles and capillaries.
• Fundoscopy: retinal hemorrhages and exudates, papilledema
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 14

• Gross pathology: flea-bitten appearance of petechial hemorrhages on kidneys

• Medical emergency:
o Untreated: most patients die within 2 years from renal failure, intracranial
hemorrhage or CHF

Aneurysms
• Congenital or acquired weakness of the vessel wall media, resulting in localized
dilatation or outpouching.
• Aneurysms are classified into true and false or pseudoaneurysms.
• True aneurysms involve all three layers of the arterial wall (intima, media, and
adventitia)
• False or pseudo-aneurysm is a collection of blood or hematoma which has
leaked out of the artery but is then confined by the surrounding tissue
• Atherosclerotic aneurysms:
o Weakening of media secondary to atheroma formation
o Occur on the abdominal aorta below the renal arteries
o Associated with HTN
o Half of aortic aneurysms > 6 cm in diameter with rupture within 10 years -
living with a ticking bomb inside – sudden rupture
o Symptomatic abdominal aneurysms > 6 cm and cerebral aneurysms that
have not been surgically repaired are an absolute contraindication to
driving any class of motor vehicle
• Syphilitic aneurysms
o Tertiary stage of infection with Treponema pallidum
o Döhle-Heller syndrome: aortitis secondary to syphilis, sometimes
complicated by aortic valve insufficiency, coronary stenosis, and aortic
aneurysm
o Involves ascending aorta
o Pathology: specific inflammation of the adventitia and vasa vasorum –
obliterative endarteritis. Vasa vasorum occlusion due to walls thickening,
ischemia, smooth-muscle atrophy in aortic media, aortic wall weakening
and scar formation
o Predominantly younger males 35-55 years
o Aortic valve ring involvement -dilation: aortic insufficiency
o de Musset's sign (Alfred de Musset) – rhythmic nodding of the head
synchronous with the heartbeat

Aortic Dissecting Aneurysm

• Dissection of the medial layer of the aorta by the blood entered from the lumen
via intimal tear
• Most common catastrophe of the aorta
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 15

• Etiology: degeneration (cystic medial

degeneration) of the tunic media
• Symptoms: severe tearing pain
• May compress renal and coronary aortic
branches
• Predisposing factors: HTN and Marfan
syndrome
• DeBakey classification:
o Type I involves the ascending aorta,
aortic arch, and descending aorta
o Type II is confined to the ascending
aorta
o Type III is confined to the descending
aorta distal to the left subclavian
artery

Berry aneurysm
• Aka intracranial aneurysm, aneurysm
aneurysm of the Circle of Willis, saccular
(pouch-like) aneurysm
• Congenital aneurysm of the circle of Willis
• Highly associated with connective tissue diseases:
o autosomal dominant polycystic kidney disease
o neurofibromatosis type I
o Marfan syndrome
o multiple endocrine neoplasia (MEN) type I
o pseudoxanthoma elasticum
o hereditary hemorrhagic telangiectasia
o Ehlers-Danlos syndrome type II and IV
• Common sites:
o Anterior communicating artery
o Posterior communicating artery
o Middle cerebral artery
o Internal carotid artery
o Tip of basilar artery
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 16

• Acute subarachnoid hemorrhage secondary to

ruptured berry aneurysm: When a saccular
aneurysm ruptures, most of the blood remains in
the subarachnoid space around the major arteries
of the circle of Willis. Blood will impede the flow of
CSF leading to increased intracranial pressure
• Rupture occurs at the tip of aneurysm
• Leakage may precede a rupture - “warning
headache”
• 60-80% patient die immediately after the rupture

Microaneurysms
• Charcot–Bouchard aneurysm – miliary or microaneurysms 300-micron diameter
• Often occurred in HTN and DM
• Location: lenticulostriate vessels of the basal ganglia
• Pathology: hemorrhagic stroke into the putamen, caudate, thalamus, pons, and
cerebellum
• Symptoms: sudden focal paralysis or loss of sensation, vary based on primary
hemorrhage localization

Mycotic Aneurysms
• Septic emboli (mycotic aneurysm) — Septic embolic from the heart can
occlude the vasa vasorum of the vessel or the vessel lumen leading to vascular
wall infection and mycotic aneurysm formation.
• Mycotic aneurysms are often multiple due to their embolic nature, but they can
also be solitary.
• Spontaneous resolution with antibiotic therapy for endocarditis has been reported
• Mycotic aneurysm’s locations:
o Cerebral
o splanchnic
o coronary arteries
o other peripheral vessels typically at arterial bifurcations

Vascular Neoplasms
• Hemangiomas:
o Extremely common benign vascular tumors
o Most common tumors in infants
o Occur on the skin, mucous membranes, or internal organs (liver)
o Major types: capillary and cavernous hemangiomas

Facial hemangiomas
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 17

Port wine stains

• Large, flat vascular ectasias

Hemangioblastoma
• In 1928, Cushing and Bailey introduced the term hemangioblastoma.
• It refers to a benign vascular neoplasm that arises almost exclusively in the
central nervous system.
• According to the World Health Organization classification of tumors of the
nervous system, hemangioblastomas are classified as meningeal tumors of
uncertain origin.
• Associated with von Hippel-Lindau disease.
• Multiple tumors may involve cerebellum, brain stem, spinal cord, and retina.
Hemangioblastoma
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 18

Supratentorial hemangioblastoma
proved by histologic analysis. Carotid
arteriogram demonstrates a vascular,
dense, tumor filled from the anterior
cerebral vessels and not involving the
sagittal sinus.

Glomus tumor (glomangioma)

• Glomus tumors were first described by Hoyer in 1877, while the first complete
clinical description was given by Masson in 1924
• Benign small painful tumor of the glomus body
• Glomus tumors are encapsulated, slowly growing, highly vascular, and locally
invasive tumors
• Histological structure of glomus tumors - dense matrix of connective tissue
among nerve fascicles

Glomus tumor of fingernail

Kaposi Sarcoma
• Low-grade a spindle-cell malignant tumor thought to be derived from lineage of
endothelial cells.
• Variable clinical course ranging from minimal mucocutaneous disease to
extensive organ involvement.
• Associated with Kaposi-sarcoma-associated virus - Human herpesvirus-8 (HHV-
8)
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 19

• Kaposi sarcoma can be primarily categorized into 4 types:

o Epidemic of AIDS-related
o Immunocompromised
o Classic, or sporadic
o Endemic (African)
• Lesions in Kaposi sarcoma may involve the skin, oral mucosa, lymph nodes, and
visceral organs.
• Most patients present with cutaneous disease.
• Visceral disease may occasionally precede cutaneous manifestations.
• Cutaneous lesions in Kaposi sarcoma are characterized as follows:
• Cutaneous lesions may occur at any location but typically are concentrated on
the lower extremities and the head and neck region.
• Lesions may have macular, papular, nodular, or plaquelike appearances
• Nearly all lesions are palpable and nonpruritic
• Lesions may range in size from several millimeters to several centimeters in
diameter
• Lesions may assume a brown, pink, red, or violaceous color and may be difficult
to distinguish in dark-skinned individuals
• Lesions may be discrete or confluent and typically appear in a linear, symmetrical
distribution, following Langer lines
• Mucous membrane involvement is common (palate, gingiva, conjunctiva)
• Gastrointestinal lesions:
o Odynophagia, dysphagia
o Nausea, vomiting, abdominal pain
o Hematemesis, hematochezia, melena
o Bowel obstruction
• Pulmonary lesions:
o Cough
o Dyspnea
o Hemoptysis
o Chest pain
o May be an asymptomatic radiographic
finding
• Epidemic Kaposi Sarcoma
• Gross pathology:
o Multiple red-purple patches, plaques, or
nodules
o May remain confined to the skin or
disseminate

• Classic European form:

o Older men of Eastern European or Mediterranean origin
o Red-purple skin plaques on the lower extremities
• Transplant-associated form:
o Occurs in patients on immunosuppressive therapy for organ transplants.
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 20

o Involves skin and viscera.

o May regress with reduction on immunosuppression.
• African form:
o Occurs in African children and young men.
o Generalized lymphatic spread is common.
• AIDS-associated form:
o Most common in male AIDS patients
o Aggressive form with frequent widespread visceral dissemination
o Common sites: skin, GI tract, lymph nodes, lungs
o Responsive to chemotherapy and interferon-alpha
o Rarely causes death.
Kaposi Sarcoma

Kaposi sarcoma (KS), facial. Confluent,

violaceous nodules involving the chest wall;
extensive facial involvement with accompanied
edema.

Endoscopic view of a Kaposi sarcoma lesion

situated in the gastric antrum.

Angiosarcoma
• Uncommon malignant neoplasms characterized by rapidly proliferating,
extensively infiltrating anaplastic cells derived from blood vessels and lining
irregular blood-filled spaces.
• Wide range of malignant endothelial vascular neoplasms that affect a variety of
sites.
• Angiosarcomas are aggressive and tend to recur locally, spread widely, and have
a high rate of lymph node and systemic metastases.
• The rate of tumor-related death is high.
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 21

Angiosarcoma

This is a classic example of angiosarcoma associated

with chronic lymphedema after lymphadenectomy and
radiotherapy. The patient is a 33-year-old woman who
presented with a recurrent grade 2 angiosarcoma of the
soft tissue after an attempt at wide excision and skin graft.
Two weeks following this procedure, she developed
multiple subcutaneous erythematous nodules involving
the back and overlying the right scapula region, the
supraclavicular fossa, the right breast, and arm. The
window shows a microphotograph of the tissue obtained
from the biopsy (hematoxylin and eosin stain, original
magnification X160). Histologic preparation reveals
neoplastic endothelial cells showing a solid pattern with
occasional mitotic figures and sporadic protruding growth
into the vascular lumens.
 Mod9Week3- PA 3306- Sept. 8- Monica Walsh (mwalsh@cmcc.ca) 22

References

Teitelbaum, A. Vascular Pathology [Lecture notes as PowerPoint]. PA 3306: Clinical

Pathology and Laboratory Testing, Canadian Memorial Chiropractic College. 2023 Sept
6 [cited 2023 Sept 10]. Available from https://kiro.cmcc.ca/

Disclaimer

The material and information within this package is a compendium of information and
course material notes from the DC program. It is to be used by students in the current
academic year of Note Service for only educational purposes and student self-study. It
is not to be reproduced, altered, redistributed in anyway. Materials contained here in are
produced on a cost recovery basis. The CMCC Students’ Council does not profit from
the sale of this material and provides it exclusively as a supplement to the education of
the students of the Canadian Memorial Chiropractic College.