CHAPTER

HEART
9 Cardiovascular System
The human heart is a muscular pump responsible for maintaining the circulation of the blood
and perfusion of different organs of the body. The thickness of the left ventricle is almost three
times that of the right ventricle. The cardiac output is about 5 liters per minute. The heart is
supplied by the coronary arteries:
1. Left coronary artery: It divides into left anterior descending artery (LAD) and the left The volume of the blood pumped

circumflex artery (LCX). The LAD is responsible for supplying the following regions by the heart in a single beat is
of the heart: called as stroke volume (about
– Apex of the heart 70 ml).
–
– Anterior 2/3rd of the interventricular septum
–
– Anterior wall of the left ventricle
–
The LCX mainly supplies the lateral wall of the left ventricle.

2. Right coronary artery: It supplies

– The entire right ventricular free wall Normal ejection fraction is
65%
–
– Posterobasal wall of the left ventricle
–
– Posterior 1/3rd of the interventricular septum.
–
Out of the three layers of the heart (pericardium, myocardium and endocardium), the
least collateral perfusion is present in the endocardium. So in any conditions associated with The most important source of
reduced perfusion (hypotension/shock), there are increased chances of sub endocardial energy for the cardiac myocytes
ischemia. The coronary artery supplying the posterior 1/3rd of the ventricular septum is the fatty acids.
(by giving rise to the posterior descending branch) is called dominant. In majority of the
individuals (~80%), this vessel arises from the RCA, so they have right dominant circulation.
The myocardium or the cardiac muscle is composed of the cardiac myocytes which cannot
undergo division or hyperplasia in response to stress. The only adaptation that can be seen in
a cardiac muscle can be hypertrophy which can be of the following types:
• Concentric hypertrophy/pressure overload hypertrophy: It is due to deposition of the SA node is the “pacemaker” of
sarcomeres (functional intracellular contractile unit of the cardiac muscle) in parallel to the long the heart
axis of the cells. It is associated with hypertension and aortic stenosis. AV node is the “gatekeeper” of
• Volume overload hypertrophy: In this, dilatation with increased ventricular diameter is present. the heart
It is seen with valvular regurgitation (mitral or aortic regurgitation), thyrotoxicosis and severe
anemia.

HEART FAILURE

It is a clinical condition characterized by the inability of the heart to pump blood in

proportion with the requirements of the metabolic tissues of the body or being able to do so at
increased filling pressures. It may be divided into systolic or diastolic failure depending on
whether there is abnormality in the cardiac contractility (systolic failure; as seen in ischemic
heart disease, pressure or volume overload, dilated cardiomyopathy) or in the ventricular
relaxation (diastolic failure; as in constrictive pericarditis, myocardial fibrosis or amyloid
deposition). We can also classify heart failure as:

L v n c / sd d (L F)
eft
e
tri
ular
failure
left
i
e
heart
failure
V
It is most commonly caused by ischemic heart disease, hypertension, aortic or mitral valvular disease
and myocardial disease (non-ischemic). The features include hypertrophy and fibrosis in the Lungs are the most commonly
myocardium associated with secondary involvement of the atria which shows enlargement. affected organ in LVF.
Atrial involvement results in the development of the atrial fibrillation which is responsible
for thrombus formation or embolic stroke. The other organs affected include:

https://kat.cr/user/Blink99/
 Review of Pathology

Lungs
• It is the most commonly affected organ. The increased pressure in the pulmonary vein causes
pulmonary edema (heavy wet lungs). The edema fluid accumulates in the alveolar space.
• There is leakage of hemosiderin and other iron containing particles which are phagocytosed by
macrophages. The iron gets converted to hemosiderin leading to the formation of siderophages
or heart failure cells (hemosiderin containing macrophages).
• The alveolar fluid impairs gaseous exchange giving rise to breathlessness or dyspnea (earliest
Siderophages or heart failure
feature of LVF), orthopnea (dyspnea on lying down) and paroxysmal nocturnal dyspnea (dyspnea
cells are hemosiderin containing
at night).
macrophages.
• There is presence of Kerley B lines on chest X-ray due to transudate in the interlobular septa.
Kidneys
In the early stages, decreased renal perfusion causes activation of the renin-angiotensin-aldosterone
system whereas in the later stages, continued reduced renal perfusion may precipitate prerenal
azotemia.
Brain
It may suffer from hypoxic ischemic encephalopathy.

Left ventricular failure is the Rg sd d

i
ht
i
e
heart
failure
most common cause of the right Left ventricular failure is the most common cause of the right sided heart failure whereas pure right
sided heart failure.
sided failure is seen in chronic severe pulmonary hypertension and is called as cor pulmonale.
Cardiovascular System

Cardiac cirrhosis is character-

ised by nutmeg liver.

The features seen as a result of the inability of the right heart to pump blood in different
organs include:
Liver
There is presence of congestive hepatomegaly. In addition, if associated LVF is present, the centrilobular
necrosis is also seen which is replaced by fibrotic tissue in longstanding cases and is known as cardiac
sclerosis or cardiac cirrhosis.
Spleen
Congestive splenomegaly is seen.
Kidney
These show the congestion resulting in severe azotemia. The congestion is more prominent in RVF
than in LVF.
• Pleural effusion, pericardial effusion and ascites are also seen.

• The hallmark of the right sided heart failure is peripheral edema of the dependent

parts of the body particularly pedal and pretibial edema.
• Anasarca is the generalized massive edema seen in heart failure.

RHEUMATI FE ER A RHEUMATI HEART I EA E (RH )
C
V
ND
C
D
S
S
D
Most commonly affected age Rheumatic fever is an acute immunologically mediated multisystem inflammatory disease that
group in rheumatic fever is 5-15 occurs few weeks after an attack of group A b- hemolytic streptococcal pharyngitis. It is not an
years. infective disease. The most commonly affected age group is children between the ages of
5-15 yearsQ. Only 3%Q of patients with group A streptococcal pharyngitis develop acute
rheumatic fever.

362

https://kat.cr/user/Blink99/
 Cardiovascular System

The disease is a type II hypersensitivity reaction in which antibodies against ‘M’ protein
of some streptococcal strains (1, 3, 5, 6, and 18) cross-react with the glycoprotein antigens in
the heart, joints and other tissues (molecular mimicry).
WHO criteria for diagnosis of RF and RHD [Based on revised (1992) Jones criteria]
1. Major manifestations:
J
Joint involvement (Polyarthritis)
O
N - Nodules (Subcutaneous)
E - Erythema marginatum
S - Sydenham’s chorea
Criteria - Carditis

2. Minor manifestations:
Clinical: Fever polyarthralgia
Migratory polyarthritis is the

Laboratory: Increased ESR or C-RP commonest major manifesta-

ECG: Prolonged PR interval tion of Jones criteria seen clini-
cally.

3. Supporting evidence of a preceding streptococcal infection within last 45 days:
Elevated or rising ASO or other Ab titers

Positive throat culture

Rapid antigen test for group A streptococcus.

Note:
1. Two major or one major and two minor manifestations plus any of the evidence of preceding

group A streptococcal infection is required for diagnosis of primary episode of rheumatic fever.

Cardiovascular System
2. 1992 revised Jones criteria do not include elevated TLC (total leukcocyte count) as a laboratory

minor manifestation [instead, it includes elevated C-reactive protein] and do not include recent
scarlet fever as supporting evidence of recent streptococcal infection.

n so jo c
Salie
t
feature
f
the
ma
r
riteria
Carditis
All the layers of the heart namely pericardium, myocardium and endocardium are involved, so this is
called pancarditis. The pericarditis is associated with fibrinous/serofibrinous exudate and is called
as ‘bread and butter’ pericarditis. Valvular involvement is common in rheumatic heart disease. The
most common valve to be affected is the mitral valve and least commonly affected is pulmonary valve. Pancarditis is a characteristic
In acute rheumatic heart disease, the most common valvular lesion is mitral regurgitation and manifestation of the cardiac
in chronic rheumatic heart disease, it is mitral stenosis. involvement in rheumatic fever.
Migratory polyarthritis
There is involvement of the large joints of the body. It is more commonly seen in the adults as compared
to children. The arthritis involves one joint after the other (migratory) and subsides spontaneously
without any residual deformability in the joints (non-erosive arthritis). Clinically, this is the most
commonly seen manifestation and the joint pain shows dramatic response to salicylates like aspirin.
Subcutaneous nodules
These are painless subcutaneous lesions found on the extensor surface of the elbows, shin and the
occiput.
Erythema marginatum The plump macrophages
There is presence of red macular rash more easily appreciated in fair skinned individuals sparing the called Anitschkow cells are
face and without residual scarring. pathognomonic for rheumatic
fever.
Sydenham’s chorea
It is a late manifestation of the disease characterized by presence of involuntary, purposeless
movements associated with emotional lability of the patient.
Microscopically, the characteristic feature of rheumatic heart disease is Aschoff’s body.
The latter consist of foci of swollen eosinophilic collagen surrounded by T-lymphocytes, few

363

https://kat.cr/user/Blink99/
 Review of Pathology

plasma cells and plump macrophages called Anitschkow cells (pathognomonic for RF).
These distinctive cells have abundant cytoplasm and central round-to-ovoid nuclei in which
the chromatin is disposed in a central, slender, wavy ribbon (hence, they are also called as
“caterpillar cells”).
The myocardium has Aschoff’s bodies in the perivascular location. The involvement
Most commonly affected of the endocardium results in fibrinoid necrosis within the cusps or along the tendinous
valve is mitral valve and the cords which also have small vegetations called verrucae present along the lines of closure. The
least commonly affected is presence of mitral regurgitation also induces irregular thickening in the left atrial wall called
pulmonary valve. as MacCallum plaques.
Chronic RHD is characterized by organization of the acute inflammation and subsequent
fibrosis. The valves show leaflet thickening, commissural fusion and shortening, and
thickening and fusion of the tendinous cords. There is mitral stenosis called as ‘fish-mouth’ or
‘button-hole’ stenosis. Mitral stenosis may also lead to atrial fibrillation and thromboembolic
phenomenon in these patients.

I FE TI E E AR ITI (IE)
N
C
V
NDOC
D
S
It is colonization or invasion of heart valves and mural endocardium by microbiologic
agent leading to formation of bulky, friable vegetations composed of thrombotic debris and
organisms with destruction of underlying cardiac tissues. It can be
• Acute

• Subacute

Acute endocarditis Subacute endocarditis
• Necrotizing, ulcerative, invasive valvular • Insidious infection following a protracted course
infection on a previously normal valve. on a previously damaged valve
Cardiovascular System

• Highly virulent organisms • Low virulence organisms

• Death of the patient within days to weeks • Recover after antibiotic therapy
• MC caused by Staph. aureus • MC caused by a-hemolytic (viridans)
Streptococcus

Important Microbiology Link!

Organism in the infective endocarditis Predisposing condition

Staphylococcus aureus a. Intravenous drug abusers
b. Native cardiac valve
Staphylococcus epididermis Patients having prosthetic or artificial valve
Staphylococcus viridians Previously damaged valve
Staphylococcus mutans Patients who have had recent tooth extraction
Apart from all these enterococci and the HACEK group of bacteria (Hemophillus, Actinobacillus,
Cardiobacterium, Eikenella and Kingella) can cause infective endocarditis.

The aortic valve is the most Mo p o ogy

r
h
l
commonly affected valve in The friable, bulky destructive vegetations containing fibrin, bacteria and inflammatory cells
prosthetic valve endocarditis. are found on the valve cusps. These can also extend on to chordae. The aortic valve and the
mitral valve are most commonly infected whereas the right side of heart is affected in intravenous
drug abusers. When the vegetations erode into myocardium, they can form an abscess called
Ring abscess. The systemic embolisation can result in septic infarcts.

nc s
Blood culture is the
Cli
i
al
feature
investigation of choice in Fever is the most consistent sign of IE. The other features include weight loss, flu-like syndrome,
infective endocarditis. cardiac murmur, systemic emboli, Roth spots (due to retinal emboli), Osler nodes (painful,
subcutaneous nodules on the fingers and toes) and Janeway lesions (red painless lesions on
the palms and soles).
• The disease is diagnosed by Dukes criteria.

364

https://kat.cr/user/Blink99/
 Cardiovascular System

o p c ons
C
m
li
ati
Cardiac complications
• Valvular insufficiency or stenosis
• Myocardial ring abscess
• Suppurative pericarditis
• Valvular dehiscence

Embolic complications
• With left sided lesion – Brain, spleen, kidney
• With right sided lesion – Lung infarct, lung abscess
Renal complications
• Embolic infarct
• Focal (more common) or diffuse glomerulonephritis (less common)

MARA TI E AR ITI / BA TERIAL THR MB TI E AR ITI ( BTE)

N
C
NDOC
D
S
NON
C
O
O
C
NDOC
D
S
N
• It is seen in patients suffering from debilitating diseases like malignancy (carcinoma

pancreas and acute promyelocytic leukemia) and hypercoagulable states (DIC)
• Vegetations on heart valves are sterile and do not contain microorganisms. These

are usually present along the line of closure, single or multiple.

LIBMA - A K E AR ITI ( LE)

N
S
C
S
NDOC
D
S
S
• Seen in patients of SLE

• Vegetations are small or medium sized, sterile, granular and pink on either or both

Cardiovascular System

sides of valve leaflets.
• Mitral and tricuspid valves are involved and show fibrinoid necrosis.

Summary of salient features of vegetations in different endocarditis
Rheumatic Fever Non Bacterial Libman Sack’s Endocarditis Infective
Thrombotic (Marantic Endocarditis
Endocarditis)
• Small, warty • Small, warty • Medium sized (small) • Large
• Firm • Friable • Flat, Verrucous • Bulky
• Friable • Irregular • Irregular
• Along lines of • Along lines of closure • On surface of cusps • Vegetations on
closure • (both surfaces may be in- the valve cusps
volved but the undersurface is • Less often on
more likely affected, less com- mural endocar-
monly mural endocardium is dium
involved
• In pockets of valves
• Sterile (no or- • Sterile • Sterile • Non-sterile
ganism) (bacteria)
• Embolisation is • Embolisation is • Embolisation is uncommon • Embolisation is
uncommon common very common
(max chances)
• In rheumatic • In cancers (like • In SLE • In infective en-
heart disease M3-AML, pancreatic docarditis
cancer), deep
vein thrombosis,
Trosseau syndrome
The most frequent causes of the functional valvular lesions
• Aortic stenosis: Calcification of anatomically normal and congenitally bicuspid

aortic valves
• Aortic regurgitation: Dilation of the ascending aorta due to hypertension and aging

• Mitral stenosis: Rheumatic heart disease

• Mitral regurgitation: Myxomatous degeneration (mitral valve prolapse).
365

https://kat.cr/user/Blink99/
 Review of Pathology

ow synd o

barl
r
me
• It is more common in femalesQ

• In this valvular abnormality, one or both mitral leaflets are “floppy” and prolapse, or

balloon back into the left atrium during systole. This gives rise to the mid systolic click.
• Most of the patients are usually asymptomaticQ

• The condition is discovered only on routine examinationQ by the presence of a

midsystolic click Q as an incidental finding on physical examination.
• In those cases where mitral regurgitation occurs, there is a late systolic or some-

times holosystolic murmur
• Some patients may present with chest pain mimicking angina, dyspnea, and fa-

tigue or, psychiatric manifestations, such as depression, anxiety reactions, and
personality disorders
• Microscopically, The characteristic anatomic change in myxomatous degeneration
is intercordal ballooning (hooding) of complete or portions of the mitral leaflets.
The affected leaflets are often enlarged, redundant, thick, and rubbery. The tendi-
nous cords are elongated, thinned, and occasionally ruptured.
• Annular dilation is a characteristic association(this finding is rare in other causes

of mitral insufficiency)
• Mitral valve prolapse is defined and revealed by echocardiography Q

• The risk of complications like Infective endocarditis, Mitral insufficiency, Stroke or

other systemic infarct and Arrhythmias is higher in men, older patients, and those
with either arrhythmias or some mitral regurgitation, as evidenced by holosystolic
murmurs and left-sided chamber enlargement.

I HEMI HEART I EA E
SC
C
D
S
S
Cardiovascular System

Ischemia of the heart is a result of imbalance between the perfusion and demand of the heart
for oxygenated blood. Atherosclerotic narrowing resulting in coronary arterial obstruction is
the cause of ischemic heart disease in almost 90% of the patients.

udden ardiac eath

S
C
D
Suden cardiac death is most It is defined as the death of an individual within 1 hour of onset of symptoms. It is most
commonly due to ventricular commonly due to ventricular fibrillation.
fibrillation. In case of coronary vessel occlusion leading to ischemia, there is physiological
compensatory vasodilation resulting in augmentation of coronary blood flow. When
atherosclerotic obstruction is 75% or greater, exercise induces symptoms of ischemia and
if stenosis is more than 90%, symptoms occur even at rest. The initiating event in most of
these patients is disruption of partially stenosing plaque with ulceration, rupture of plaque
or hemorrhage into the atheroma.

table Angina
S
In the ECG, subendocardial
Stable angina occurs when the myocardial oxygen demand is more than the supply. It takes
ischemia manifests as ST place when the coronary artery is occluded >75%. Stable angina is characterized by pain on
segment depression. exertion which is relieved on taking rest or taking vasodilators like nitrates. There is neither
any plaque disruption nor any plaque associated thrombus. ECG changes include ST segment
depression and T wave inversion (subendocardial ischemia of the left ventricle).
366

https://kat.cr/user/Blink99/
 Cardiovascular System

rinzmetal or ariant Angina

P
V
It is an episodic angina due to coronary artery spasm resulting in pain at rest. It is characterized
by ST segment elevation on the ECG (due to transmural ischemia).
In the ECG, transmural
Unstable or rescendo Angina ischemia manifests as ST
C
segment elevation.
It is induced by atherosclerotic plaque disruption with superimposed partial thrombosis
or vasospasm or both of them. The pain occurs with increasing frequency and for a longer
duration and is characteristically precipitated by progressively less exertion.

Myocardial Infarction (MI)

Myocardial infarction Concept
The main difference between
angina and the MI is elevation
Subendocardial MI Transmural MI of cardiac enzymes in the
latter which is not seen usually
• Ischemic necrosis limited to 1/3rd of • Ischemic necrosis involves full thickness of ventricular with angina. In one third of
ventricular wall wall the patients, angina may be
• Caused by incomplete coronary • Caused by severe coronary atherosclerosis, with acute associated with elevation of
artery occlusion. plaque rupture and superimoposed occlusive thrombus. cardiac enzymes.

Pathogenesis of MI
Changes in atheromatous plaque (hemorrhage/ulceration/ruputre)
↓
Exposure of underlying collagen and platelet aggregation
↓

Cardiovascular System
Platelets release mediators which cause vasospasm
↓
Activation of extrinsic clotting pathway and increased thrombus formation
↓
Complete occulsion of coronary vessel by thrombus

Myocardial Response

Feature Time
Cessation of aerobic respiration or onset of ATP depletion Seconds
Loss of contractility <2 min
ATP reduced to 50% of normal 10 min
 
ATP reduced to 10% of normal 40 min
 
Irreversible cell injury 20-40 min
Microvascular injury >1 hr

Evolution of Morphological hanges in MI

C
Time Gross Light Microscopy
Reversible injury
0-30 min. None None
Irreversible injury
The neutrophils are present
30 min to 4 hr. None Waviness of fibers at border (earliest change) between 48-72 hours but after
4-12 hr. Occasional dark mottling Beginning of coagulative necrosis, edema and 72 hours, the macrophages
hemorrhage predominate and cause early
phagocytosis.
12-24 hr. Dark mottling Ongoing coagulative necrosis, marginal
contraction band necrosis, beginning of
neutrophilic infiltration

367

https://kat.cr/user/Blink99/
 Review of Pathology

Time Gross Light Microscopy

1-3 days Mottling with yellow tan infarct Coagulation necrosis, interstitial neutrophilic
center. infiltrate
3-7 days Hyperemic borders, central Beginning of disintegration with dying neutrophils,
yellow tan softening early phagocytosis by macrophages
7-10 days Maximum yellow tan and soft Early formation of fibrovascular granulation tissue
depressed red-tan margin at margins
10-14 days Red gray depressed infarct Well established granulation tissue and collagen
borders deposition
2-8 weeks Gray-white scar progressive Collagen deposition, ↓ Cellularity
from border towards infarct core
> 2 months Scarring complete Dense collagenous scar

Note:
• When the infarcts are less than 12 hours old, they can be diagnosed only with the help of the
dye triphenyl tetrazolium chloride (TTC). The dye reacts with the LDH enzyme present only in
the living cardiac fibers so it gives brick-red color to the living tissue whereas the tissue already
undergone infarction shows the unstained pale zone.
• In the case of the infarction being modified by reperfusion, the characteristic, microscopic fea-
ture is the presence of necrosis with contraction band (in irreversible injured myocytes, the
increased calcium ions cause hypercontraction of the sarcomeres).

iagnosis of MI
D
MI should be suspected in any patient developing severe chest pain, rapid weak pulse,
Cardiovascular System

MI is the leading cause of death sweating, dyspnea and edema. Infact, rapid pulse is the first sign and dyspnea is the first
in diabetes mellitus. Diabetics symptom of acute MI. The ECG shows the ST segment elevation in acute MI whereas ‘Q’
have ‘silent MI’. wave indicates old MI.
Laboratory investigations show nonspecific markers like increased ESR, leukcocytosis
and elevated C-reactive protein. The specific markers include:
Enzyme Initiation of rise Peak Return to baseline
CK-MB 2-4 hours 24 hours 48-72 hours
Troponin T and I (TnT, TnI) 2-4 hours 48 hours 7-10 days
AST/SGOT In 12 hours 48 hours 4-5 days

Sequence of elevated enzymes LDH 24 hours 3-6 days 2 weeks

after MI (Time to CALL
emergency) IM RTA T I T AB UT THE AR IA E Z ME
Time to Troponin
PO
N
PO
N
S
O
C
D
C
N
Y
S

C CK-MB
Troponin T and Troponin I

A AST

L LDH1 These are the proteins that mediate calcium mediated contraction of the cardiac and the skeletal

muscles. They are very specific for MI. Troponin I is more important than troponin T (remember, I for
Important). If the patient has another MI (due to reinfarction within 1 week), these enzymes cannot be
used for diagnosis of reinfarction because their levels remain elevated for a long time from the first
attack. In that condition, we prefer an enzyme elevated for a short duration.
Concept Creatine kinase (CK)
Myoglobin: earliest enzyme to
It is an alternative to troponin measurement. It has got 3 isoforms:
increase after MI.
• CK-MM- Present in the skeletal muscle and heart
Troponin: Most sensitive as • CK-MB- Present in the myocardium and a small amount in skeletal muscle
well as specific for MI. • CK-BB –Present in the brain, lung and other tissues.
Elevation of the CK-MB isoforms is seen in MI. Any absence of elevation of CK-MB in the first-two days
CK-MB: enzyme of choice for excludes the diagnosis of MI. This is the enzyme of choice for diagnosing reinfarction.
diagnosing reinfarction.
Myoglobin
It is a small monomer with a rapid rise and fall in serum (has a narrow window). It is the earliest enzyme
to increase after MI.

368

https://kat.cr/user/Blink99/
 Cardiovascular System

LDH
Normally, serum LDH2 is greater than LDH1 but in MI, LDH1 is more than LDH2. This is called “flipping
of LDH ratio”.

o p c ons o MI
C
m
li
ati
f
• Contractile dysfunction resulting in cardiogenic shock.
Dressler syndrome is treated
• Arrhythmia- Ventricular fibrillation is the most common arrhythmia within one hour whereas with the help of NSAIDs with/
supraventricular tachycardia is the most common arrhythmia after one hour of MI. without the use of steroids.
• Cardiac rupture syndrome: Rupture of ventricular free wall is the most common cardiac rupture
syndrome. It results in cardiac tamponade. The anterolateral wall at the midventricular level is the
most common site for postinfarction free wall rupture. It is most frequent 3 to 7 days after MI.
The rupture of ventricular septum leads to formation of left to right shunt. The rupture of papillary
muscles can cause mitral regurgitation.
Concept
• Pericarditis- It is the epicardial manifestation of the underlying myocardial injury and is also
known as Dressler syndrome or post MI syndrome. It is an autoimmune reaction, which takes Rupture of the left ventricle, a
place around 2-3 weeks after a transmural MI. though it has been reported to occur even after 48 complication of acute myocardial
hrs. It is associated with pleural effusion, pleuritic chest pain and pericardial effusion. infarction, usually occurs when
the necrotic area has the least
• Right ventricular infarction.
tensile strength, about 4 to 7
• Ventricular aneurysm: This may contribute to thromboembolism also days after an infarction, when
repair is just beginning.
• Papillary muscle dysfunction: This leads to post infarct mitral regurgitation.

AR IA TUM R
C
D
C
O
S
Cardiovascular System
Myxoma

Myxomas are the most common primary tumor of the heart in adults. Though they may arise
in any cavity of the heart but nearly 90% are located in the atria, with a left-to-right ratio of
approximately 4:1 (atrial myxomas). The major clinical manifestations are due to valvular
“ball-valve” obstruction, embolization, or a syndrome of constitutional symptoms, such as
fever and malaise the latter most commonly due to the effect of interleukin-6.
Approximately 10% of patients with myxoma have a familial cardiac myxoma syndrome
(known as Carney syndrome) characterized by autosomal dominant transmission, multiple The most common cardiac
cardiac and often extracardiac (e.g. skin) myxomas, spotty pigmentation, and endocrine tumor is the secondaries or
overactivity. The familial form is associated with mutation of the gene PRKAR1 on chromosome metastasis.
17 (a tumor suppressor gene).
The tumors are almost always single. The region of the fossa ovalis in the atrial septum
is the favored site of origin. Histologically, myxomas are composed of stellate or globular
myxoma (“lepidic”) cells, endothelial cells, smooth muscle cells, and undifferentiated cells The most common primary
embedded within an abundant acid mucopolysaccharide ground substance and covered on cardiac tumor in the adults is
the surface by endothelium. the myxoma.

Rhabdomyoma

Rhabdomyomas are the most frequent primary benign tumor of the heart in infants and
children. They are actually hamartomas or malformations rather than true neoplasms.
Cardiac rhabdomyoma is associated with tuberous sclerosis due to defect in the TSCI or
TSC2 tumor suppressor gene. The TSC proteins stimulate the cell growth and are involved The most common cardiac
in myocyte overgrowth. tumor in the children is the
Rhabdomyomas are generally small, gray-white myocardial masses protruding into the rhabdomyoma.
ventricular chambers. Histologically they are composed of large, rounded, or polygonal cells
containing numerous glycogen-laden vacuoles separated by strands of cytoplasm running
from the plasma membrane to the more or less centrally located nucleus, the so-called spider
cells.

369

https://kat.cr/user/Blink99/
 Review of Pathology

BL E EL

OOD
V
SS
S
The blood vessels are responsible for the transport of blood in the circulation from the heart
to the various organs and back to the heart.
The histological layers which are seen in a blood vessel (particularly arteries) are:
1. Tunica intima (Innermost layer)

2. Internal elastic lamina

3. Tunica media (Middle layer)

4. External elastic lamina

5. Tunica adventitia (Outermost layer)

The outer half of the tunica media and the whole of tunica adventitia are supplied by vasa
vasorum whereas the other inner layers of the blood vessel get their nourishment by diffusion.
Types of blood vessels in the circulatory system and their important properties
Artery Elastic arteries - Tunica media is rich in elastin fibers e.g. Aorta and its large branches
Muscular arteries - Tunica media is rich in smooth muscle cells e.g. coronary and renal
arteriesQ
Arteriole Principle site of resistance to blood flow, so called Resistance vessels
Capillaries Have maximum cross-sectional surface areaQ
Venules Most important vessel involved in inflammationQ
Vein Maximum blood volume present in the veinsQ
Any injury/denudation of endothelial cells stimulate thrombosis and smooth muscle
cell proliferation. The normal blood vessels particularly the arteries have an elastic recoil
property referred to as the ‘Windkessel effect’. This effect is responsible for maintaining the
blood flow inside the vessels during the diastolic phase of cardiac contraction. ‘Sclerosis”
Cardiovascular System

means loss of elasticity of vessels commonly associated with thickening. It may be of the
following types:
1. Arteriolosclerosis - It affects small arteries and arterioles, it can be of the following

types:
Hyaline arteriolosclerosis
Concept
• Pink, hyaline thickening of arteriolar walls.
Endothelial cells contain
• Seen in elderly, more commonly in benign hypertension, diabetes mellitus (DM) and benign
Weibel Palade Bodies having
von Willebrand factor and are nephrosclerosis.
identified by antibodies to CD31, Hyperplastic arteriolosclerosis
CD34 and vWF.
• ‘Onion skinning’ or concentric thickening of the arteriolar wall seen in malignant hypertension.
• Fibrinoid necrosis/necrotizing arteriolitis (inflammatory cells in vessel wall particularly in kidney)

2. Monckeberg’s medial calcific stenosis

– Seen is muscular arteries of people > 50 years of age.
–
– Associated with dystrophic calcificationQ and is asymptomatic.
–
3. Atherosclerosis

It is characterized by deposition of atheroma/fibrofatty plaque consisting of raised

focal lesion. Plaque is present within the intima, has a core of lipid (cholesterol and
cholesterol esters) and a covering of fibrous cap.
The histopathology shows:
1. Fibrous cap - Consists of smooth muscle cells, macrophages and foam cells.

2. ‘Shoulder’ - Cellular area around cap having macrophages, smooth muscle cells and

Windkessel effect is the elastic T lymphocytes.
recoil property of the blood 3. Necrotic core - Debris of dead cells, foam cells and cholesterol clefts.
vessels.

Risk factors for atherosclerosis (Mnemonic: Atherosclerosis)
Coronary circulation is an A- Age (↑ with age)
example of autoregulatory
circulation. T- Type ‘A’ personality
H- Hyperhomocysteinemia (corrected by adequate folic acid and vitamin B12 in diet)
370 E- Extra lipids (Hyperlipidemia); Extra BP (hypertension); Extra sugar (DM)

https://kat.cr/user/Blink99/
 Cardiovascular System

R- Reduced physical activity Concept

O- Obesity Lipoprotein a, or Lp(a), is an
S- Sex (More common in males, females in pre-menopausal age group are protected) altered form of LDL that contains
the apolipoprotein B-100 portion
C- CMV; Chlamydia infection; Cigarette smoking of LDL linked to apolipoprotein
L- Lipoprotein ‘A’ A. It has structural similarity to
plasminogen. So, it competes
with plasminogen in clots
athogenesis decreasing the latter’s ability to
P
form plasmin and clear clots.
It is best explained by the ‘Response to Injury Hypothesis’Q Increased Lp(a) levels are
associated with a higher risk of
According to this hypothesis, chronic endothelial injury results in increased permeability, coronary and cerebrovascular
leukocyte adhesion and thrombotic potential. This is associated with accumulation of disease, independent of total
lipoproteins (mainly LDL)Q followed by oxidation of lipoproteins in the vessel wall. The cholesterol or LDL levels.
blood monocytes initially adhere to the endothelium followed by their transformation into
macrophages and foam cells inside the intima along with adhesion of platelets. The activated
platelets release factors causing migration of smooth muscle cells from the media to intima
and their proliferation along with release of proteoglycans and collagen. This results in
Foam cells are lipid laden
enhanced accumulation of lipids. In advanced atheroma, the smooth muscle cells may
Smooth muscles cells/Tissue
undergo apoptosis and so, smooth muscle cell paucity may be observed. macrophages or Blood mono-
Foam cells are formed because oxidized LDL is ingested by the scavenger receptors present cytes.
on the macrophages and smooth muscle cells both intracellularly as well as extracellularly.

atural History of Atherosclerosis

N
Cardiovascular System
American Heart Association classification of human atherosclerosis:
Type Gross Microscopy
Clinically silent Leriche syndrome is aortoiliac
Type I Fatty dot (initial lesion) Isolated macrophage; foam cell occlusive disease due to ather-
osclerotic occlusion affecting the
Type II Fatty streak Intracellular lipid accumulation
bifurcation of the abdominal aor-
Type III Intermediate lesion Type II change + small extracellular lipid pool ta as it transitions into the com-
mon iliac arteries. It is character-
Clinically silent or overt ised by: buttock claudication
Type IV Atheroma lesion Type II + core of extracellular lipids plus sexual impotence along
Type V Fibroatheroma Lipid core and fibrotic layer with reduced femoral pulses.

Type VI Complicated lesion Surface defect, Hemorrhage and thrombus

F ys
att
treak
• It is the earliest lesion of atherosclerosis and is composed of lipid filled foam cells. It begins as

yellow flat spots less than 1 mm which gradually progress to atheroma formation.
Fatty streak is the earliest
Significance of involved blood vessels in atherosclerosis lesion of atherosclerosis.
Abdominal Aorta - Most common site of atherosclerotic aneurysm in body
Coronary Arteries - Left Anterior Descending is MC coronary artery involvedQ
Poplitial Artery - MC peripheral vessel showing aneurysm formationQ
Descending Thoracic Aorta Involvement of blood vessels
Internal carotid artery affected in atherosclerosis in
Circle of Willis descending order: ACP of Delhi
Traffic is Cute:
Significance of complications of Atherosclerosis
Abdominal Aorta
• Aneurysm: Due to weakness of the tunica media… Coronary Arteries
• Calcification: Dystrophic calcification is seen Poplitial Artery
• Ulceration: Increases thrombus formation Descending Thoracic Aorta
• Thrombosis: Most feared complication Internal carotid artery
• Embolism: Erosion of athermanous plaque Circle of Willis

371

https://kat.cr/user/Blink99/
 Review of Pathology

A EUR M

N
YS
Complications of Atherosclerosis A localized abnormal dilation of a blood vessel or the wall of the heart is called aneurysm. It
are ACUTE
is of two types:
Aneurysm
Calcification, 1. True aneurysm: Involves intact attenuated arterial wall or thinned ventricular

Ulceration, wall of the heart. The common causes include Atherosclerosis, syphilis and post MI
Thrombosis, ventricular aneurysms.
Embolism, 2. False/Pseudo- aneurysm: It is characterised by a breach in the vascular wall leading

to extravascular hematoma communicating with intravascular space. The two most
common causes of pseudoaneurysm are post MI rupture and leakage at the site of
vascular anastomosis.
Causes of True Aneurysm in Aorta
1. Atherosclerosis *It is the most common cause of true aneurysm in aorta
Atherosclerosis causes *The most commonly affected vessel is the abdominal aorta (below the origin of
abdominal aortic aneurysm. renal artery and above bifurcation into common iliac artery).
2. Syphilis *The thoracic aorta is involved in tertiary stage of syphilis
*Endarteritis of vasa vasorum results in patchy ischemia of tunica media. This is
responsible for the often seen “tree barking” appearance of the thoracic aorta.
*Aortic valve insufficiency can also occur which may result in cardiac hypertrophy.
The increase in the size of heart is called as cor bovinum/cow heart.
3. Other causes Trauma; infection (mycotic aneurysm; mostly due to Salmonella gastroenteritis)
and systemic disease (vasculitis)
Other important points about different causes of aneurysms
• Cystic medial necrosis is characterized by weakness due to media due to degeneration of the
tunica media and it affects the proximal aorta.
Cardiovascular System

• Syphilitic aneurysms affect ascending aorta or aortic arch

• Tuberculous aneurysms affect the thoracic aorta.
• Traumatic aneurysms affect descending thoracic aorta just below the site of insertion of ligamentum
arteriosum.
The inherited causes of aneurysm and their causes are as follows (Robbins 8th/506-7):
• Marfan’s syndrome: defective synthesis of the protein fibrillin

• Ehlers-Danlos syndrome: defect in collagen type III

• Loeys Dietz syndrome: defect in elastin and collagen types I and III due to mutation

in TGF-b receptor.

A RTI I E TI
Hypertension is the commonest
O
C
D
SS
C
ON
cause of ascending aortic It occurs when blood splays apart the laminar planes of the media with the formation of
aneurysm. blood-filled channel within the aortic wall. It is mostly seen in men in the age group of 40-
60 years (with antecedent hypertension) and uncommonly in younger individuals with
connective tissue disease (Marfan’s syndrome). Medial degeneration is a characteristic pre-
existing lesion in most of the patients.
Dissection is classified into two types:
1. Type A - Involves ascending aorta with/without descending aorta. It is more

common and is more dangerous.
2. Type B - Does not involve ascending aorta but lesion begins distal to subclavian artery.

A ULITI
V
SC
S
The inflammation of the vessel wall is called vasculitis. It may be classified on the basis of
pathogenesis or on the basis of size of the involved vessel.

372

https://kat.cr/user/Blink99/
 Cardiovascular System

Classification based on vessel size

Large vessel vasculitis Medium vessel vasculitis Small vessel vasculitis Concept
• Giant cell (temporal) • Classic PAN Antiendothelial antibodies are
arteritis • Kawasaki’s disease found in SLE and Kawasaki’s
• Takayasu arteritis • Buerger’s disease disease.

Cardiovascular System
Immune complex mediated Paucity of Immune complex
• SLE • Wegener’s granulomatosis
• Henoch-schonlein pupura • Microscopic lyangitis
• Cryoglobulin vasculitis • Churg-strauss syndrome
• Goodpature syndrome

Anti-neutrophil cytoplasmic antibodies (ANCAs) are autoantibodies against enzymes

inside the neutrophils c-ANCA is formed against proteinase 3 whereas p-ANCA is formed
against myeloperoxidase. These can be of the following types:

373

https://kat.cr/user/Blink99/
 Review of Pathology

LAR E E EL A ULITI

G
V
SS
V
SC
S
Temporal arteritis is the most
common type of vasculitis in
1. Giant cell (Temporal) arteritis/Cranial arteritis

adults. • It is the most common type of vasculitis in adultsQ (usually >50Q years)
• This vasculitis is characterized by granulomatous arteritis of the aorta and its major
branches particularly the extracranial branches of the carotid artery. Since the superficial
temporal arteryQ is the most commonly involved vessel, the giant cell arteritis is called
as temporal arteritis.
Lastest Information (9th Edn.) • Clinical features include constitutional symptoms like fever, fatigue, weight loss, jaw
• c-ANCA→PR3 ANCA painQ (most specific symptom), facial pain, localized headacheQ (commonest

• p-ANCA→MPO ANCA symptom; most intense along the anatomical course of the superficial temporal artery)

and sudden onset of blindness (due to involvement of ophthalmic artery).
• Biopsy of temporal arteryQ is the investigation of choice.
• Microscopically, there is presence of granulomatous inflammation with multinucleated
Corticosteroids are the drug of giant cells and fragmentation of internal elastic lamina.
choice for treatment of temporal 2. Takayasu arteritis/Aortoarteritis/Aortic Arch syndrome
arteritis.

– It is seen in adult females < 50 years of age.
–
– This condition is characterized by granulomatous vasculitis followed by
–
thickening of the aortic arch and decreased lumen of the vessels arising from the
aortic arch. The pulmonary, renal and coronary arteries may also be involved.
The subclavian artery is most – Clinical features include weak pulses in the upper limbs (so, the disease is
–
commonly involved vessel in also called as pulseless diseaseQ), ocular disturbances, hypertension and
Takayasu arteritis. neurological defects.

ME IUM E EL A ULITI
D
V
SS
V
SC
S
1. Classic Polyarteritis Nodosa (PAN)
Cardiovascular System

• It is a systemic vasculitis of medium sized muscular arteries (no involvement of
arterioles/capillaries/venulesQ).
• The most frequently involved vessels are those of the kidney and other viscera vessels.
The vessels of the pulmonary circulationQ are typically involved.

not
• Characteristic feature of this disease is sharp segmental lesions showing transmural
Glomerulonephritis and vessels inflammation of vessel wall accompanied by fibrinoid necrosis and existence of all
of pulmonary circulation are stages of inflammation in the same vessel.
typically not involved in PAN. • 30% patients have association with Hepatitis B antigenQ in their serum.
• No glomerulonephritisQ is seen.
• It is the commonest cause of mononeuritis multiplexQ.
2. Kawasaki’s disease (Mucocutaneous Lymph Node Syndrome)


– It is the vasculitis affecting children < 5 yearsQ of age. So, it was designated as
–
infantile polyarteritisQ earlier. It is characterized by fever, conjunctivitis and
oral erythema, skin rash often with desquamation, erythema of palms and soles
and cervical lymphadenopathy.
– For the diagnosis of Kawasaki disease, there must be presence of feverQ (most
–
important constitutional symtpom) for greater than 5 days plus any 4 of the
Treatment: High dose intrave- following:
nous g globulin with aspirinQ • C - Conjunctivitis (non-exudativeQ; non purulent conjunctivitis)
is effective in reducing the mor- • R - Rash (polymorphous non-vesicular)
bidity and mortality in Kawasaki’s • E - Edema (or erythema of hands or feet)
disease.
• A - Adenopathy (cervicalQ, often unilateralQ and non suppurativeQ)
• M - Mucosal involvement (erythema or fissures or crusting at times referred as
strawberry tongueQ)
– It is the most important cause of acquired heart disease in children in USA. It
–
may present with myocardial infarctionQ in children. It is having the presence
of anti-endothelial cell antibodiesQ. There is typically intimal proliferation and
mononuclear infiltration of vessel wall. The patients also have elevated platelet
count in this condition.

374

https://kat.cr/user/Blink99/
 Cardiovascular System

MALL E EL A ULITI
S
V
SS
V
SC
S
p-ANCA is present in Micro-
1. Microscopic Polyarteritis/Microscopic Polyangiitis/Leukocytoclastic Vasculitis scopic Polyangiitis but is

not

– Necrotizing vasculitis affecting arterioles/capillaries/venules in which all seen with PAN.
–
lesions are of the same age.
– Granulomatous inflammation is absentQ
–
– Necrotizing glomerulonephritis and capillaritis are common.
–
– Fibrinoid necrosis associated with infiltration of neutrophils which become
Palpable purpura in HSP
–
fragmented (leukocytoclasia). is due to vasculitis and not
2. Henoch-Schönlein purpura (HSP)/Anaphylactoid purpuraQ thrombocytopenia.

– It is the commonest vasculitis in childenQ.
–
– This is a vasculitis with IgAQ deposits affecting small vessels like arterioles,
–
capillaries and venules of the skin, gut and glomeruli and commonly associated
with arthralgia.
– Clinical features include palpable purpura (due to vasculitis and not reduced
–
platelet count)Q, colicky abdominal painQ, arthralgia in multiple joints and
glomerulonephritis.
– It is caused due to immune complex deposition but complement levels are
–
usually normalQ.
3. Hypersensitivity vasculitis/Cutaneous vasculitis

• Defined as inflammation of the blood vessels of the dermis.
• Also called as hypersensitivity vasculitis/cutaneous leukocytoclastic angiitis.
• Microscopic features include presence of vasculitis of small vessels characterized by a
leukocytoclasisQ (refers to the nuclear debris remaining from the neutrophils that have
infiltrated in and around the vessels during the acute stages).

Cardiovascular System
• Hallmark clinical feature is skin involvement typically appearing as palpable
purpuraQ appearing on most commonly lower limbs
• Diagnosis is best made with biopsy showing vasculitis.
• Removal of offending agent (if any) and steroids help most of the patients.
4. Churg-Strauss syndrome (Allergic granulomatosis and angiitis)
Lastest Information (9th Edn.)

– Characteristically have necrotizing vasculitis accompanied by granulomas with
Wegener’s granulomatosis is
–
eosinophilic necrosis. now called as granulomatosis
– p-ANCA present in 50% of patients. with polyangitis
–
– Strong association with allergic rhinitis, bronchial asthma and eosinophilia.
–
– Principal cause of death includes coronary arteritis and myocarditis.
–
5. Wegener’s granulomatosis (Granulomatosis with polyangitis)

Necrotizing vasculitis which is characterized by triad of
1. Acute necrotizing granulomas of either upper (more commonly) or lower respiratory

tract or both.
2. Focal necrotizing or granulomatous vasculitis most commonly affecting lungs and upper

airways.
3. Renal involvement in the form of focal necrotizing, often crescentic glomerulonephritis.
Concept

• Clinical features include fever, weight loss, otitis media, nasal septal perforationQ,
strawberry gumsQ, cough, hemoptyis, palpable purpuraQ, joint pain and ocular features Limited Wegener’s granulo-
(uveitis, conjunctivitis) matosis is characterized by only
• Investigations show serum c-ANCAQ positivity, cavitatory lesionsQ in the chest X ray respiratory tract involvement
and red cell casts (indicative of glomerulonephritis) in the urine. without any renal involvement.

RA AU ’ HE ME
YN
D
S
P
NO
NON
Raynaud’s disease or Primary Raynaud’s phenomenon is seen in young females. It is
characterized by intense vasospasm of small vessels in the digits of hands and feet induced by
cold and emotional stimuli (so, pulses are NOT affected).
• Characteristic sequence of color change is

375

https://kat.cr/user/Blink99/
 Review of Pathology

Systemic sclerosis is the com-

monest cause of secondary
Raynaud’s phenomenon.
• Structural changes are absent except in later course when thickening of intima is seen.

Secondary Raynaud’s phenomenon is associated with conditions like systemic
sclerosisQ (commonest cause), SLE, atherosclerosis and Buerger’s disease. It usually affects
people of age >30 years.

Also Know
• Secondary Raynaud’s phenomenon patients are older in age and have more severe symptoms
and complications
• More commonly have dilatation of the capillary bed at the base of the fingernails.
• Index and middle fingers are more sensitive to attacks. Thumb is least sensitive.

Buerger’s disease (Thromboangiitis obliterans)

Segmental thrombosing vasculitis • It is usually seen among heavy cigarette smokers.
often extends into contiguous • Onset is before age 35
veins and nerves is a charac- • It is associated with hypersensitivity to intradermal injections of tobacco extracts.
teristic features of Buerger’s • Microscopic examination demonstrates segmental thrombosing vasculitis often extends into
disease. contiguous veins and nerves (a feature rarely seen in other types of vasculitis), encasing them
in fibrous tissue.
• Thrombus contains microabscess with granulomatous inflammation.Q
• This patient has distal lower extremity vascular insufficiency which may present as Calf, foot
or hand intermittent claudication, superficial nodular phlebitis and cold sensitivity (Raynaud’s
phenomenon). Severe distal pain even at rest can result and may be due to neural involvement.
Later complications include ulcerations and gangrene of the toes feet or fingers.
Cardiovascular System

• Treatment includes smoking cessation, drugs (peripheral vasodilators) and may even require
surgery.

A ULAR TUM R
V
SC
O
S
Strawberry gums are seen in
Wegener’s granulomatosis.

Benign Tumors
Strawberry tongue is seen in
Kawasaki disease.
Hemangioma
1. Capillary hemangioma

• It is the most common type of vascular tumor which occurs in skin, mucus membrane
Strawberry hemangioma is a and viscera.
type of capillary hemagioma. • “Strawberry” type of capillary hemangioma (also called as juvenile hemangioma) is very
common, growing rapidly in the first few monthsQ and regresses by age 7Q in newborns.
The child is normalQ at birth in almost 90% of cases.
• Histologically, they are lobulated unencapsulated aggregates of closely packed, thin
walled capillaries which are blood filled and lined by a flattened endothelium.
376

https://kat.cr/user/Blink99/
 Cardiovascular System

2. Cavernous hemangioma

– It is less common than capillary hemangioma with same age and anatomic Cavernous lymphangioma occurs
–
distribution. It more frequently involves deep structures as it shows no tendency in Turner’s syndrome at the
to regress. So, it usually requires surgery. neck region.
– Morphologically, Cavernous hemangiomas are made up of large, cavernous
–
vascular spaces in which intravascular thrombosis and dystrophic calcification
is common.
– They may be life-threatening as in von Hippel Lindau disease where they occur
–
in cerebellum, brainstem and the eye.

3.
Pyogenic granuloma

It is a polypoid form of capillary hemangioma seen attached by a stalk to skin or oral

mucosa. It is associated with edema and inflammatory cells.
Granuloma gravidarum is present in the gingiva of pregnant women and it regresses
after delivery.

L M HA I MA
Y
P
NG
O
1. Cavernous lymphangioma (also called as cystic hygroma)
Concept

Capillary lymphangioma is dis-
• This is present in the neck region of the children.
tinguished from the capillary
• Made up of dilated, cystic lymphatic spaces lined by endothelial cells.
channels only by the absence of
• Lesions are non-encapsulated, so, removal is difficult.
blood cells.
2. Capillary Lymphangioma

It is a lesion composed of small lymphatic channels occurring subcutaneously in the

head and neck region and in the axilla.

Cardiovascular System
L MU TUM R ( o ng o )
G
O
S
O
Gl
ma
i
ma
• Benign tumor arising from the smooth muscle cells of the glomus body which is an

arteriovenous anastomosis involved in thermoregulation.
• Most commonly present in the distal portion of the digits (under fingernails).

• Histologically, there is presence of branching vascular channels and stroma

containing nests/aggregates of glomus cells arranged around vessels.

I TERME IATE/B R ERLI E TUM R

N
D
O
D
N
O
S
Kaposi’s arcoma (K )
S
S
It is caused by KS Herpes virus or Human herpes virus 8 (HHV8) and has the following 4
forms:
Type of Kaposi sarcoma Association with HIV Chief affected organs
Classic/Chronic/European KS Absent Skin plaques and nodules
African/Endemic KS Absent No skin lesions; lymphadenopathy
present
Transplant associated/ Absent Lymph nodes, mucosa and visceral
Immunosuppression-associated KS organs
Epidemic/AIDS associated KS PresentQ Lymph nodes and viscera involved
The morphology is characterized by three stages:
1. Patch stage

2. Plaque stage

3. Nodular stage – Often accompanied by involvement of lymph nodes and of viscera

particularly in African and AIDS-associated disease.

377

https://kat.cr/user/Blink99/
 Review of Pathology

MALI A T TUM R

GN
N
O
S
Hepatic angiosarcoma is
associated with carcinogens
including arsenic, thorotrast Angiosarcoma
(a radioactive contrast) and
polyvinyl chloride (PVC; a • Malignant endothelial cell neoplasm most commonly seen in skin, soft tissue, breast and liver.
plastic). • May also arise from dilated lymphatic vessels (lymphangiosarcoma).
• Endothelial cell origin is demonstrated by staining for CD31, CD34 or vWF.

Hemangiopericytoma

•
Tumor derived from pericytes which are the cells present along the capillaries and
venules.
• These tumors most commonly arise from pelvic retroperitoneum or the lower limbs

(particularly thighs).
• Capillaries are arranged in ‘fish-hook pattern’ and silver stain is used for diagnosing

this condition.
Quick review of superior vena cava and inferior vena cava syndromes
Syndromes Associated Cancers Clinical features
Superior Vena • Bronchogenic cancer Q
• Dilation of the veins of the head, neck and
Cava (SVC) • Mediastinal lymphomaQ arms
syndrome • Cyanosis
• Respiratory distress
Inferior Vena • Renal cell carcinomaQ • Lower limb edema
Cava (IVC) • Hepatocellular carcinomaQ • Dilation of the superficial collateral veins of
syndrome the lower abdomen
Cardiovascular System

• Massive proteinuria (if renal vein is involved)

378

https://kat.cr/user/Blink99/