Takayasu Arteritis

1. Medium & Large arteries 2. Weak or Pulse less upper extremities 3. Fibrous thickening of aorta
(aortic arch) 4. Asian young (15-30, <40) females 5. Autoimmune etiology 6. HLA haplotype A24-B52-DR2
in Japanese 7. Intimal wrinkling 8.coronary & renal artery involvement 9. Granulomatous inflammation
10. Patchy medial necrosis 11. Steroids 12.collagenous fibrosis 13.MI from narrowing of coronary Ostia
14. Lower BP 15. Coldness/numbness of finger 16. Visual defect 17.retinal hemorrhages 18. Blindness
19. Hypertension 20.neurologic defects 21. Leg claudication if distal aorta 22.raynaud phenomenon
23.malaise 24.night sweats 25.arthralgia 26. Fever 27.weght loss 28. Fatigue 29. Anemia 30. Increased
ESR 31. Arterial stenosis 32. Thrombosis 33. Aneurysm

Temporal/Giant Cell Arteritis

1. Commonest systemic vasculitis 2. Granulomatous large to small arteries 3. Temporal, vertebral,

ophthalmic arteries & aorta 4. Thoracic aortic aneurysm 5. Blindness 6. Nodular thickening 7. Reduction
of lumen 8. Thrombosed artery 9. Granulomatous inflammation 10. Multinucleate giant cell of foreign &
langhans type 11.mononuclear infiltrate 12. Fragmentation of internal elastic lamina 13. Immune
reaction against arterial wall component 14. HLA-DR antigen 15.steroids 16. Age>50 17. Predilection for
superficial temporal artery 18. Extraordinary trophism 19. Fever 20. Fatigue 21. Weight loss 22. Facial
pain 23. Headache 24.painful palpated artery 25. Diplopia 26. polymyalgia rheumatica 27. increased ESR

Polyarteritis Nodosa

1. medium to small arteries 2. No arterioles, capillaries or venules 3. renal & visceral vessels 4. no
pulmonary vessel 5.ischemia & infarction 6. kidney heart liver GIT 7. segmental erosion 8. arterial wall
weakening 9. perfusion impaired 10. ulceration, infarcts, hemorrhages, ischemic atrophy 11. transmural
arterial wall inflammation 12. fibrinoid necrosis 13. thrombosed lumen 14. fibrous vessel wall thickening
15. young adult 16. classic PAN child & old 17. malaise, fever, weight loss, hypertension, abdominal pain,
malena, muscular pain, peripheral neuritis (motor) 18. no glomerulonephritis 19. HbAg antigen 30% 20.
no ANCA
21. corticosteroid & cyclophosphamide

Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)

1. Coronary arteries 2. Infants & children 80% 3. acute but self limiting 4. fever 5. hand & feet edema 6.
oral, conjunctival, palm & sole erythema 7. desquamated skin rash 8.cervical lymph node enlargement 8.
cardivascular sequalae 20% 9. aspirin & gamma globulin in lowering aneurysm & death 10. PAN like 11.
less fibrinoid necrosis but pronounced inflammation 12. complicated by aneurysm, thrombosis or MI 13.
obstructive intimal thickening by healed lesion
Microscopic Polyangiitis ( Microscopic Polyarteritis/Hypersensitivity/Leucocytoclastic vasculitis)

1. arterioles, capillaries, venules 2. all lesion of same age in contrast to PAN 3. palpable purpura involving
skin, mucous membrane, lungs, brain, heart, GIT, kidney, muscle 4. skin biopsy dignostic 5. necrotizing
glomerulonephritis & pulmonary capilaritis in contrast to PAN 6. hemoptysis, arthralgia, abdominal pain,
hematuria, proteinuria, hemorrhage, muscle pain, weakness 7. immunologic reaction to antigen
(penicillin, streptococci, heterogenous protein, tumor antigen) precipitating cause 8. P-ANCA 70% 9. no
mascular/large arteries or macroscopic infarcts like PAN 10. granulomatous inflammation absent 11.
segmental fibrinoid necrosis 12. postcapillary venules in LCA 13. Ig & complement components 14.
Henoch-Schonlein purpura 15. essential mixed cryoglobulinemia

Churgh-Strauss Syndrome (Allergic granulomatosis & angiitis)

1. necrotizing vasculitis 2. eosinophillic necrosis 3. granuloma 4. P-ANCA 50% 5. allergic rhinitis, bronchial
asthma, eosinophillia 6. coronary arteritis & myocarditis for morbidity & mortality 5. cysteinyl
leukotriene receptor antagonist type 1 triggers in asthma

Wegener Granulomatosis

1. Acute necrotizing granuloma of upper resp. tract (ear, nose, sinus, throat), lower resp. tract (lung) 2.
small or medium vessels ( capillaries, venules, arterioles, artieries) 3. focal necrotizing renal disease 4.
PAN+respiratory involvement 5. inflammatory sinusitis 6. mucosal granuloma 7. ulcerative lesion of nose,
palate, pharynx 8. fibroblastic proliferation of giant cell & leucocytic infiltrate in lung 9. cavitating
nodules 10. mycobacterial or fungal infection 11. alveolar hemorrhage 12. crescentric
glomerulonephritis 13. hematuria & proteinuria 14. resembles PAN & serum sickness, immunologic 15.
males>females, 40-50 16. persistent pneumonitis w/ bilateral nodular & cavitary infiltrate, chronic
sinusitis, mucosal ulceration of nasopharynx, renal disease 17. skin rash, muscle pain, articular
involvement, mono/polyneuritis, fever 18. C-ANCA 95% 19. rising titer of C-ANCA is relapse

Lymphomatoid Granulomatosis

1. Pulmonary nodule of lymphoid & plasmacytoid cell 2. celluar atypia 3. lymphoproliferative disorder, T
cell non Hodgkin lymphoma

Thromboangiitis Obliterans (Buerger's disease)

1. Segmental thrombosis (lumen) 2. medium & small arteries (tibial & radial) 3. veins & nerves of
extremities 4. age<35, jewish chain smokers, men 5. HLA-A9 & HLA-B5 6. small microabscess 7. central
focus of neutrophil & granulomatous inflammation 8. chronic ulceration of toes, feet, fingers & frank
gangrene 8. smaller arteries wit severe pain in contrast to atherosclerosis, even at rest

Vasculitis e.g. hypersensitivity angiitis/classic PAN relates to : RA, SLE, malignancy, mixed
cryoglobulinema, Henoch-Schonlein Purpura

Lupus vasculitis : aggressive anti inflammatory therapy Antiphospholipid antibody syndrome : aggressive
anti thrombotic/anticoagulant therapy

Rheumatoid Vasculitis : from RA, small & medium arteries, visceral infarction & aortitis

Infectious Arteritis : bacteria & fungi ( aspergillus & mucomycosis), bacterial pneumonia or casseous
tuberculous abcess, meningitis of superficial cerebral vessels, mycotic aneurysm, infarction, aneurysm,
thrombosis

Raynaud Disease

1. Paroxysmal Pallor or cyanosis of digits of hand & feet, tips of nose or ears (acral parts) 2. Cold induced
vasoconstriction of digital arteries, precapillary arterioles, cutaneous arteriovenous shunt 3. white blue
red finger colour change 4. intimal thickening at late stage only 5. exaggeration of local & central
vasomotor response to cold or emotion 6. age ~ 14 7. atrophy of skin, muscles, subcutaneous tissue 8.
ulceration/ischemic gangrene rare 9. Primary

Raynaud Phenomenon

1. arterial insufficiency of extremities by SLE, scleroderma, atherosclerosis, buerger's disease 2. age>30

3.more severe episodes 4.associated skin lesions 5. connective tissue disease 6.secondary in nature

Varicose Veins

1.abnormally dilated & tortuous elongated & scarred vein caused by prolonged intraluminal pressure &
loss of vessel wall support 2. superficial veins of upper & lower legs 3. long standing occupation & long
automobile/airplane rides cause venous stasis & pedal edema 4. age>50 & obese & pregnant woman 5.
thinning at maximum dilation 6. intraluminal thrombosis & vulvar deformities ( thickening, rolling,
shortening of cusps) 6. elastic tissue generation 7. spotty calcification within media (phlebosclerosis) 8.
venous stasis, congestion, edema, pain & thrombosis 9. stasis dermatitis, ulceration, injury vulnerablity,
poorly healing wound & infection causing varicose ulcer 10. esophageal varices in liver cirrhosis , massive
GI hemorrhage if ruptured 11. Hemorrhoids at anorectal junctions may thrombose & painful ulceration
& distended

Thrombophlebitis & Phlebothrombosis

1. Deep leg veins 2. cardiac failure, neoplasia, pregnancy, obesity, post-operative stage, prolonged bed
rest, immobilization 3. genetic hypercoaguability syndrome 4. adenocarcinoma of pancreas, colon &
lung, hypercoaguability occurs as paraneoplastic syndrome 5. trousseau sign in migratory
thrombophlebitis 6. periprsotatic venous plexus in male & female pelvic vein additional site 7.
peritonitis, pelvic abscess, acute appendicitis & salpingitis leads to portal vein thrombosis &
inflammation 8. calf muscle squeezing or foot dorsiflexion are Homan's sign 8. Plegmasia alba dolens
( painful white leg) is iliofemoral venous thrombosis in pregnant woman also called milk leg 10.
pulmonary emobolism common & serious sequel

Superior Vena Caval Syndrome

1. Neoplasm compressing or invading Sup. Vena cava 2. primary bronchogenic carcinoma or mediastinal
lymphoma 3. dusky cyanosis 4. dilation of head neck arm veins 5. compressed pulmonary vessels causing
respiratory distress

Inferior Vena Caval Syndrome

1. Neoplasm compressing or penetrating wall of IVC or a thrombus from femoral or iliac vein propagating
upwards 2. Hepatocellular & Renal Carcinoma 3. Massive proteinuria 4. Leg Edema 5. distention of
superficial collateral veins of lower abdomen 6. right atrium involvement

Lymphangitis & Lymphedema

1. Secondary related with inflammation or cancer 2. Group A beta hemolytic streptococci 3. Dilated &
exudated lymphatics by neutrophil & histiocytes 4. Cellulitis & focal abcess in serious case 5. painful
subcutaneous red streak 6. bacteremia & septicemia 7. Obstructive lymphedema 8. Lymphatic blockage
in malignant tumor spread, surgical process, post irradiation fibrosis, filariasis, post inflammatory
thrombosis & scarring 9. Chylous ascites, chylothorax, chylopericardium 10. primary lymphedema as
isolated congenital defect or as familial Milroy disease 11. lymphedema praecox in female 10-25 years in
feet, swollen super imposed infection & ulceration 12. increased interstitial fluid subcutaneous
interstitial fibrous tissue increase 13. brawny induration 14. skin ulcer & peau d orange skin
Hypertension

Essential Hypertension (Idiopathic/Benign)

1. Combined effect of mutation or polymorphism at several gene loci 2. Environmental (stress, salt
intake) 3. Mendelian form rare 4. Sustained HTN requires kidney 5. established HTN has increased blood
vol & peripheral resitance both 6. Salt sensitive HTN as Liddle Syndrome causes mutation in Enac protein

Cause :
Genetic factor : Family History, African Lineage
Environmental factor: dietary Na intake, Stress (Type A personality), Obesity. Smoking, Physical Inactivity

Secondary Hypertension (Accelerated/Malignant)

1. 200/120 2. Renal failure, rentinal hemorrhage & exudate, with/or papilledema

Cause :
Renal : acute GMN, chornic renal disease, polycystic disease, renal artery stenosis , renal artery
fibromuscular dysplasia , renal vasculitis, renin producing tumor
Endocrine : Adrenocortical Hyperfunction ( Cushing Syndrome, primary Aldosteronism, congenital
adrenal hyperplasia, licorice ingestio), Exogenous hormones ( glucocorticoid, estrogen, OCP),
sympathomimetics & tyramine rich food, MAO inhibitors, Pheochromocytoma, Acromegaly, Myxedema,
Thyrotoxicosis
Cardiovascular : Coarcation & rigidity of aorta, PAN, increased intrvascular volume & cardiac output
Neurologic : Psychogenic, Sleep Apnea, Acute stress, Surgery, Increased intracranial pressure

BP = Cardiac Output X Peripheral resistance

Humoral Factors >>> Peripheral resistance

Vasoconstrictor : angiotensin II, catecholamines, endothelin, thromboxane, leukotriene
Vasodilator : Kinin, Prostaglandin, NO, Natriuretic peptide (endogenous inhibitor of renin angitensin
system)

Local Factor: Autoregulation, pH, Hypoxia >>>>Peripheral Resistance

Neural Factors >>>> Peripheral resitance

Constrictor : alpha adrenergic Dilator: beta adrenergic

Cardiac Factors : Heart rate, contractility >>>>>Cardiac Output

Blood Volume : Na, Mineralocorticoid, atriopeptin

Blood Pressure Regulation :

Angiotensinogen (OCP) via RENIN to Angiotensin I via ACE to Angiontensin II > Aldosterone secretion >
Mineralocorticoids (GRA aldosterone syntase, 11beta & 17alpha hydroxylase deficiency,
aldosteronoma)> mineralocorticoid receptor activity (Licorice ingestion, Cushing syndrome)> Na
reabsorption > NaCl Co transporter (Gitelman syndrome) or Epithelial Na channel (Liddle syndrome
beta/gamma subunit of Enac or Pseudohypoaldosteronism 1 alpha/beta subunit of Enac)

Fibromuscular dysplasia, renal artery stenosis, renin secreting tumor > renin

HTN associated with Arteriosclerosis

Monckeberg Medial Calcific Stenosis : Palpable Calcific deposit in muscular artery in >50, dont encroach
on vessel lumen, radial & ulnar artery involved, arterial flow unobstructed due to non involvement of
intima, pipestem artery, unrelated to atherosclresosis but coexistence possible

Hyaline Arteriosclerosis

1. homogenous pink hyaline thickening 2. loss of underlying structure 3. narrowing of lumen 4. Old
norm,otensive hypertensive diabetics 5.characterisitic of benign nephrosclerosis causing renal blood
supply impairment, nephron loss, symmetric contraction of kidney

Hyperplastic Arteriosclerosis

1. severe BP laignant HTN 2. onion skin concentric laminated thickening of arteriolar wall w/ narrowing
of lumen consisting SMC 3. deposit of fibrinoid & acute necrosis of vessel wall i.e. necrotizing arteriolitis,
kidney specially

Aneurysm

1. Localized abnormal dilation of blood vessel or heart wall

True Aneurysm : Bounded by arterial wall component or attenuated heart wall e.g. Atherosclerotic,
Syphilitic, Congential vascular, left ventricular
False Aneurysm : Breach in the vascular wall leading to extravascular hematoma freely communicating
with intravascular space (pulsating hematoma) e.g. post MI rupture

Aortic Aneurysm : atherosclerosis & cystic medial degenration of arterial media cause it, decreased level
of tissue inhibitor of metalo proteinases TIMP
inhibitors of MMP, MMP gene disruption, overexpression of TIMP-1 blocks aneurysm development

Berry Aneurysm : small saccular (spherical) lesion in smaller arteries of brain, in circle of Wilis, unrelated
to birth & atherosclerosis but develop at sites of congential weakness at bifurcation of cerebral arteries,
most frequent cause of subarachnoid hemorrhage, adult polycystic kidney disease associated
Mycotic (infectious) Aneurysm : from embolization & arrest of septic embolus , as extension of adjacent
suppuration, by circulating organisms

Atherosclerotic Aneurysm : In abdominal aorta, descending thoracic aorta, common iliac artery, iliac
arch, saccular or fusiform (long segment), atheromatous ulcer covered by granular mural thrombi, prime
site for atheroemboli, affects renal/superior/inferior mesenteric artery origin, men >50,

a) Inflammatory Abdominal Aortic Aneurysm : dense periaortic fibrosis, abundant inflammation rich in
lymphocytes, plasma cell, macrophages, giant cell.
b) Mycotic Abdominal Aortic Aneurysm : microorganism, bacteremia by primary salmonella
gastroenteritis, suppuration destroys media

Clinical Course : Rupture into peritoneal cavity/retroperitoneal tissue w/ massive fatal hemorrhage,
obstruction of iliac, renal, mesenteric, vertebral vessel leading to ischemic unjury, embolism from
atheroma/mural thrombi, impingement on adjacent structure, pulpably pulsating abdominal mass,
stimulating a tumor, stent graft rather than surgery

Syphilitic Aneurysm ( Leutic) : Predilection for small vessel, complication in aorta & NS, inflammation
starts from aortic adventitia, involves vasa vasorum, induce obliterative endarteritis rimmed by
lymphocyte & plasma cell infiltrate, lumina narrowing causes ischemic injury to aortic media, patchy loss
of medial elastic fiber & muscle cell , inflammation & scarring, media destruction, loss of eleastic recoil
of aorta, 'tree barking' wrinkling of aortic intima, aortic valve ring dilation, widened commissure b/w
cusps, turbulence induced thickening & rolling of free margins, left ventricular wall undergoes massive
vol overload hypertrophy known as Cor Bovinum

Thoracic Aortic Aneurysm : encroachment of mediastinal structure, resp difficulty due to encroachment
on lung & airways, swallow difficulty due to esophagus compression, persistent cough due to irritation or
pressure on recurrent laryngeal nerves, pain from bone erosion, cardiac disease & rupture
Patient die of Heart Failure.

Aortic Dissection : Dissection of blood b/w & along laminar plane of media with formation of blood filled
channel w/in aortic wall, massive hemorrhage by outward rupture, aortic dissection may or may not be
present with marked dilation of aorta unlike atherosclerotic or leutic aneursym, men 40-60 years with
antecedent HTN, younger patient if connective tissue disease e.g. Marfan Syndrome, traverse/oblique
intimal tear with sharp but jazzed edges from origin but not through media of ascending aorta,
dissecting hemotoma spreads along laminar plane of aorta, massive hemorrhage by rupture & re-
rupture to produce secondary intimal tear thus produces double barreled aorta with false channel which
becomes endothelialized ( chronic dissection), cystic medial degenration characterized by elastic tissue
fragmentation & separation of elastic & fibromuscular elements of tunica media by small cleftlike spaces
filled with amorphous ECM of connective tissue but not cyst truly , ultimately causes loss of elastic
lamina, no necrosis or cyst or inflammation (e.g. Marfan Syndrome), Aggressive anti HTN therapy

Clinical Course : more common proximal lesion involving only ascending or ascending-descending aorta
both (type A), less common distal lesion beginning distal to subclavian artery ( type B) , sudden
excurciating pain from anterior chest, radiating to back * moving downward (confused with MI), death
from outward rupture into pericardial/pleural/peritoneal cavity, retrograde dissection causes cardiac
tamponade, aortic insufficiency, MI, extension of dissection into great arteries of neck,
coronary/renal/mesenteric/iliac arteries, transverse myelitis from spinal artery compression.

Hemangioma : localized, entire extremity (angiomatosis), superficial lesion of head & neck & liver,
infants/childhood, benign, spontaneous regress before puberty

Capillary Hemangioma : largest single type of vascular tumor, skin, subcutaneous tissue, mucous
membrane of oral cavity & lips, also in liver spleen & kidney, juvenile hemangioma is strawberry typeof
skin in newborn, bright red to blue, intact covering epithelium, ocassionally pedunculate, lobulated,
uncapsulated aggregates of closely packed thin walled capillaries, blood filled & lined by endothelium
separated by scant connective tissue stroma, lumina partially or completely thrombosed, scarring by
vessel rupture accounting for ocassional hemosiderin pigment.

Cavernous Hemangioma : less common but larger than capillary variety, less well circumscribed, involves
deeper structures than capillary, locally destructive, no regress thus surgery, red-blue soft spongy
uncapsulated sharply defined mass, giant forms in subcutaneous areas of face/extremities, made up of
large cavernous vascular spaces,
blood filled & lined by endothelium separated by scant connective tissue stroma, dystrophic calcification,
occurs w/in cerebellum, brain stem, eye, similar angiomatous lesion, cystic neoplasm of pacreas & liver

Pyogenic Granuloma (Lobular Capillary Hemangioma) : Polypoid form of capillary hemangioma, rapid
growing exophytic red nodules attached by a stalk to skin/gingival or oral mucosa, lesion easily bleeds &
ulcerates, extensive edema * chornic inflammatory infiltrate, exuberant granulation tissue like,
recurrence as solitary/satelite nodule, granuloma gravidarum in gingiva of pregnant woman regresses
after delivery

Lymphangioma Circumscriptum ( Capillary Lymphangioma ) : small lymphatic channel subcutaneously in

head-neck-axilla, slight elevated & pedunculated lesion, blister like blebs filled w/ exudates, absence of
blood cell distinguishes from capillary,

Cavernous Lymphangioma ( Cystic Hygroma ) : analogous to cavernous hemangioma, children's neck &
axilla. retroperitoneally rarely. non discreet & uncapsulated tumour difficult to remove, in Turner's
syndrome (neck)

Glomangioma ( Glomus Tumor) : small rounded red-blue slightly elevated firm nodule, benign but
painful from modified SMC of glomus body involved in thermoregulation, distal portion of digits, under
fingernails minute foci of fresh hemorrhage, excision is curative, resemble cavernous hemangioma,
round small regular round/cuboidal cell resemble SMC

Nevus Flammeus : ordinary birthmark, most common ectasia, forms on head-neck light pink to deep
purple & flat, port wine stain in child thickens the skin surface & no fade

Sturge Weber syndrome : port wine stain, trigeminal nerve involved ( Encephalo trigeminal
angiomatosis), faulty mesodermal/ectodermal element development, venous angiomatous masses in
leptomeninges over cortex & ipsilateral port wine nevi of face,mental retardation, seizure, hemiplegia,
skull radiopacities thus a large vascular malformation in face may indicate presence of more extensive
vascular malformation in child who exhbits mental deficiency

Spider Telangiectasia ( Arterial Spider ) : non neoplastic vascular lesion, more/less radial & pulsatile array
of subcutaneous arteries/arterioles about a central core which blanches upon pressure applied to
centre. face-neck-upper chest & pregnant women & liver cirrhosis patient, hyperestrinism found

Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Disease) : autosomal dominant, genetic

malformation of dilated capillaries & veins, from birth to skin & mucous membrane of oral cavity, lips,
resp, urinary & GI tract, rupture, epistaxis,hematuria, gut bleeding.

Bacillary Angiomatosis : Opportunistic infection in immunocompromised, gram -ve a)Bartonella

Henselae which causes cat scratch disease in immunocompetents & b) B. Quintana causing Trench Fever,
red papules or nodules, rounded subcutaneous masses, protuberant epithelloid endothelial cell w/
nuclear atypia & mitoses, stromal neutrophil abundance, nuclear dust, purplish granular material are
distinguishers from KS, pyogenic Granuloma, angiosarcoma
Cat flee vector for B. Henselae & body louse for B. Quintana, Macrolides treatment.

Bacillary Peliosis: vascular lesion of liver-spleen

Kaposi Sarcoma :

a) Chronic /Classical/European : Older Ashkenazi Jews/Mediteranean descent, unrelated to AIDS,

homosexual at risk, multiple red to purple skin plaques/nodules in arms or legs, slowly growing in size &
number, spreads proximally & confluent, localized & locally persistent w/ lapse or remission

b) Lymphadenopathic/African/Endemic : south african bantu children, local/generalized

lymphadenopathy, sparse skin lesion, most frequent in Central africa extremely aggressive

c) Transplant/Immunosuppresion associated : Due to immunosuppresive therapy, aggressive, lymph

node-mucosa-visceral organ involved with 50% skin lesion absent, fatal if internal organ involved, needs
reduction of immunosuppresive therapy
d) AIDS associated : male homosexual AIDS patient, no site of predilection but lymph node & gut early,
wide dissemination

Description : 3 stages : patch-plaque-nodule, pink-red-purple solitary patches/multiple macules, distal

lower extremities/feet, dilated irregular angulated vessels, interspersed infiltrate of lymphocytes, plasma
cells, macrophages, lesion difficult to distinguish from granulation tissue, large violaceous raised plaques,
dermal-dilated-jagged vascular channels lined by plump spindle cells, red cell-hemosiderinladen-
macrophages-lymphocytes-plasma cell inside, pink hyalin globules, occasional mitotic figure, nodular
neoplastic at later stage, plump sheets & proliferating spindle cell, marked hemorrhage, hemosiderin
pigment & lymphosite admixed w/ cellular background, round pink cytoplasmic globules & mitotic figure
common, human herpes virus 8 HHV8/KSHV (member of gamma herpes virus causing
lymphoma/lymphoproliferative disorder, sexual transmission, latent infection, reactive polyclonal lesion
at early stage, produces p53 inhibitor & viral homologue of cyclin d67, primary infection asymptomatic,
radiation for multiple lesion in restricted area, chemotherapy for extensive/disseminated disease,
withdrawal of immuno suppresive agent,AIDS medication, angigenisis inhibitors, Interferon alpha.

Hemangioendothelioma : Intermediate b/w benign hemangioma & malignant angiosarcoma, medium to

large veins in adult soft tissue, inconspicuous well defined vascular channels, cuboidal plump tumor cells
resembles epithelial cell, d/d metastatic carcinoma-melanoma-sarcoma looking epithelioid
appearance,excision curative,recurrence 30%

Angiosarcoma : malignant endothelial, resemble hemangioma, anaplasia makes the d/d difficult from
carcinoma-melanoma, both sex older adult in skin, soft tissue, breast, liver, after 10 years of radical
mastectomy, arises from dilated lymphatic vessel, induced by radiation, foreign material
iotragenically/accidentlly, deceptively small sharply demarcatedasymptomatic often multiple red nodule
& becomes large-fleshy masses of pale gray white soft tissue, central softening, necrosis & hemorrhage,
all degrees of differentiation, solid spindle cell appearance, EC derivation demostrated by staining for
CD31, CD34, vWF, local invasion & distal metastatic spread

Hepatic Angiosarcoma : caused by arsenic, thorotrast, PVC, rare

Hemangiopericytoma : Heterogenous group neoplasm with gross fleshy/spongy consistency, thin walled
STAGHORN vascular branching, arising from pericyte in pelvic retroperitoneum, lower extremities, thigh
of middle aged woman, 1/3 malignant, necrosis & high mitotic rate, nuclear pleomorphism & aggressive

Atherosclerosis : intimal lesion called atheroma/atheromatous/fibrofatty/lipid/fibrolipid plaques

protruding into & obstruct vascular lumen & weaken underlying media, beginning w/ isolated foam
cell/fatty dot through stage of fatty streaks-atheroma-fibroatheromas to complicated lesion, Fatty streak
earliest composed of lipid filled foam cell, insignificantly raised causing no flow disturbance, yellow flat
spots coalesce into elongated streak, T lymphocyte & extra cellular lipid, in aorta of children 1-10 years,
coronary streak in adolescents, risk factor in adults, not all fatty streak destined to be plaques,
Atherosclerotic plaques develop in elastic arteries (aorta-carotid-iliac artery) large & medium sized
muscular arteries (coronary-popliteal)
Symptomatic Atherosclerotic Disease involves artery supplying heart-brain-kidney & lower extremity, MI-
cerebral infarction-aortic aneurysm-peripheral vascular disease (leg gangrene) major consequences of
atherosclerosis, mesenteric occlusion-sudden cardiac death-chronic ischemic heart disease-ischemic
encephalopathy secondarily, lumen occlusion in small artery, comprise bllod flow to distal organ thus
ischemic injury, plaques undergoes disruption & precipitate thrombi, encroachment on subjacent media
& weakening of vessel wall causing aneurysm in large artery,, extensive atherosclerosis can be friable &
shed emboli into distal circulation

Description : Intimal Thickening , lipid acculumation, soft yellow grumous core of lipid ( cholesterol & its
ester) covered by firm white fibrous cap, appear white to whitish yellow & impinge on artery lumen,
involve only partial circumference (eccentric lesions, patchy & variable), abdominal aorta>>>thoracic
aorta>coronary>popliteal>internal carotid>vessel of circle of Wilis , much prominent in ostia (origin) of
major branches, upper extremity vessels-renal-mesenteric arteries spared except at ostia, 3
components : a) SMC-macrophage-leucocytes b) ECM (collagen, elastic fibers, proteoglycans) c)
intra/extra cellular lipid.
superficial fibrous cap by SMC-ECM, necrotic core deep into it contains disorganized lipid, cholesterol
clefts, dead cell debris, foam cells, fibrin, variable thrombus, plasma proteins, Foam Cells are large lipid
laden cellsderive from blood monocyte (tissue macrophages), SMC can also imbibe to become foam cell,
around periphery is neovascularization, plaques, atheroma undergoes calcification risky for coronary
events, focal rupture, ulceration, erosion of luminal surface of plaques results in thrombus formation or
debris discharge into bloodstream producing microemboli, hemorrhage from fibrous cap/capillaries
rupture induced by contained hematoma, superimposed thrombosis on disrupted lesion partially/fully
occlude lumen & most risky, healing of thrombi incorporated & enlarge intimal plaque
aneurysmal dilation from ATH induced atrophy of media w/ loss of elastic tissue causing weakness &
rupture.

Risk factors: Scandinevian Country, Middle Age 40-60, Male>Female, post-menopausal female due to
estorgen decrease, polygenic IHD, derangement in lipoprotein metabolism, familial
hypercholesterolemia, diet, life style, personal habit,
HYPERLIPIDEMIA/HTN/SMOKING/DIABETES most risky

Hyperlipidemia : hypercholesterolemia, LDL:HDL=4:1, LDL is vehicle of delivery of chlesterol to peripheral

tissue where HDL mobilizes chlesterol from developing & existing atheroma & transport it to liver for
excretion in bile, exercise & moderate ethanol raises HDL, obesity & smoking lowers HDL, egg yolk,
animal fat, butter rich in saturated fat. omega 3 fatty acid in fish oil beneficial where hardened trans
unsaturated fat from polyunsaturated vegetable fat/baked good/margarine are injurious, STATIN lowers
cholesterol by blocking HMG CoA reductase.

HTN: both systolic & diastolic pressure are important but systolic more
Other factors are homocystinuria & urinary homocysteine in premature vascular disease,
Hyperhomocysteinemia due to low folate & vit-B6, markers of hemosttic & thombotic function e.g.
plasminogen activator inhibitor-1 & CRP, Lipoprotein Lp(a); lack of exercise, type A personality
competitive stressful life, weight gain, hypertriglyceridemia

Pathogenesis :
a) Chronic subtle endothelial injury>endothelial dysfunction>increased permeability>leucocyte adhesion
& thrombotic potential
b) lipoprotein LDL accumulation in vessel wall>modification by oxidation
c) monocyte & other leucocyte adhesion to endothelium>migration into intima>thransformed into
macrophage & foam cell
d) platelet adhesion>factor realease from activated platelet & macrophage causing migration of SMC
from media to intima
e) SMC proliferation into intima>ECM eloboration leading to collagen & proteoglycan
accumulation>enhanced lipid accumulation

Hyperlipidemia to Atherogenesis : chronic hypercholesterolemia impairs EC function by production of

oxygen free radical which deactivates NO, major endothelial relaxing factor>lipoprotein accumulation
w/in intima at site of increased endothelial permeability>oxydized lipid ingestion by macrophages by
scavenger receptor ( not LDL receptor)>foam cell formation>increased monocyte accumulation>GF &
cytokine stimulation>cytotoxicity of EC & SMC.

two organisms in atherogenesis : chlamydia pneumoniae & cytomegalovirus

Arteriovenous fistula : Rare small abnormal communication b/w arteries & veins, developmental
(congenital) defect, from rupture of arterial aneurysm & penetrating injuries, from inflammatory vessel
necrosis, , shortcircuit blood from artery to vein, cause heart to pump additional volume, high output
cardiac failure, brain hemorrhage 7 rupture, , intential fistula for providing vascular access for chronic
hemodialysis