This document discusses different types of vasculitis and cardiac tumors. It defines vasculitis as inflammation of blood vessels of any size, which can be limited or systemic. The causes of vasculitis include immune complexes, ANCAs, infections, and unknown factors. Vasculitis is classified based on the size and location of involved blood vessels, histologic characteristics, and clinical manifestations. Specific types discussed include giant cell arteritis, Wegener's granulomatosis, and microscopic polyangiitis. Cardiac tumors can be primary, such as myxomas, or metastatic from other cancers. Primary cardiac tumors include various sarcomas and rhabdomyomas. The document also covers classifications and features of different vascular tumors

1. Faculty of Medicine
Pathology of
Cardiovascular System
KING
ABDULAZIZ
UNIVERSITYITY
RABIGH BRANCH
VASCULITIS

2. VasculitisVasculitis

3. VasculitisVasculitis
 Inflammation of blood vesselsInflammation of blood vessels of any size, affecting oneof any size, affecting one
or few vessels in a limited area or it could be systemicor few vessels in a limited area or it could be systemic
affecting multiple organ systems.affecting multiple organ systems.

4. VasculitisVasculitis
 Mostly immune reaction related:Mostly immune reaction related:
 Immune complexesImmune complexes..
(SLE, cryoglobulinemic vasc.)(SLE, cryoglobulinemic vasc.)
(hypersensitivity)(hypersensitivity)
(viral infection, hepatitis)(viral infection, hepatitis)
 Antineutrophil cytoplasmic antibodies (ANCAs).Antineutrophil cytoplasmic antibodies (ANCAs).
 p-ANCAs (perinuclearp-ANCAs (perinuclear  myeloperoxidase)myeloperoxidase)
(microscopic polyangiitis, Churg-Strauss syndrome)(microscopic polyangiitis, Churg-Strauss syndrome)
 c-ANCAs (cytoplasmicc-ANCAs (cytoplasmic  proteinase 3)proteinase 3)
(Wegener granulomatosis)(Wegener granulomatosis)

5. VasculitisVasculitis
 MostlyMostly immune reactionimmune reaction related:related:
 Immune complexes.Immune complexes.
 Antineutrophil cytoplasmic antibodies (ANCAs).Antineutrophil cytoplasmic antibodies (ANCAs).
 Antiendothelial Cell Antibodies: induced by defects inAntiendothelial Cell Antibodies: induced by defects in
immune regulation (SLE, Kawasaki)immune regulation (SLE, Kawasaki)
 InfectionInfection

6. Classification ofClassification of
VasculitisVasculitis
Based onBased on
PathogenesisPathogenesis
Direct Infection
Bacterial (e.g., Neisseria)
Rickettsial (e.g., Rocky Mountain spotted fever)
Spirochetal (e.g., syphilis)
Fungal (e.g., aspergillosis, mucormycosis)
Viral (e.g., herpes zoster-varicella)
Immunologic
Immune complex-mediated
Infection-induced (e.g., hepatitis B and C virus)
Henoch-Schönlein purpura
Systemic lupus erythematosus and rheumatoid arthritis
Drug-induced
Cryoglobulinemia
Serum sickness
Antineutrophil cytoplasmic autoantibody-mediated
Wegener granulomatosis
Microscopic polyangiitis (microscopic polyarteritis)
Churg-Strauss syndrome
Direct antibody attack-mediated
Goodpasture syndrome (anti-glomerular basement membrane antibodies)
Kawasaki disease (antiendothelial antibodies)
Cell-mediated
Allograft organ rejection
Inflammatory bowel disease
Paraneoplastic vasculitis
Unknown
Giant cell (temporal) arteritis
Takayasu arteritis
Polyarteritis nodosa (classic polyarteritis nodosa)

7. Classification of vasculitisClassification of vasculitis
 The systemic vasculitides are classified on the basis ofThe systemic vasculitides are classified on the basis of
thethe
 Size andSize and
 Anatomic site of the involved blood vessels,Anatomic site of the involved blood vessels,
 Histologic characteristics of the lesion, andHistologic characteristics of the lesion, and
 Clinical manifestations.Clinical manifestations.
 There is considerable clinical and pathologic overlapThere is considerable clinical and pathologic overlap
among these disorders,among these disorders,

8. Classification of vasculitisClassification of vasculitis
 Polyarteritis nodosaPolyarteritis nodosa::
 Medium - sized & small arteries.Medium - sized & small arteries.
 Wegener’s granulomatosisWegener’s granulomatosis::
 Arterioles,venules,capillaries and small blood vesseles.Arterioles,venules,capillaries and small blood vesseles.
 Microscopic polyarteritisMicroscopic polyarteritis (hypersensitivity vasculitis):(hypersensitivity vasculitis):
 Venules, capillaries & arterioles.Venules, capillaries & arterioles.
 Temporal (giant cell,cranial) arteritisTemporal (giant cell,cranial) arteritis::
 Mainly affects large blood vesseles.Mainly affects large blood vesseles.

10. Giant Cell (Temporal) ArteritisGiant Cell (Temporal) Arteritis
 TheThe most commonmost common of the vasculitis, is an acute andof the vasculitis, is an acute and
chronic, often granulomatous inflammation of arteries ofchronic, often granulomatous inflammation of arteries of
large to small size (mainly in the head-especially thelarge to small size (mainly in the head-especially the
temporal arteries but also the vertebral and ophthalmictemporal arteries but also the vertebral and ophthalmic
arteries (Blindness).arteries (Blindness).
 Lesions have also been found in other arteries throughoutLesions have also been found in other arteries throughout
the body, including the aorta (giant cell aortitis).the body, including the aorta (giant cell aortitis).

11. Giant Cell (Temporal) Arteritis:Giant Cell (Temporal) Arteritis:
MorphologyMorphology
 Characteristically, segments of affected arteries developCharacteristically, segments of affected arteries develop
nodular thickenings with reduction of the lumen and maynodular thickenings with reduction of the lumen and may
become thrombosed.become thrombosed.
 Common variant:Common variant:
 granulomatousgranulomatous inflammation of the inner half of the mediainflammation of the inner half of the media
centered on thecentered on the internal elastic membraneinternal elastic membrane marked bymarked by
 a lymphocytic infiltrate,a lymphocytic infiltrate,
 multinucleate giant cells,multinucleate giant cells,
 fragmentation of the internal elastic laminafragmentation of the internal elastic lamina,,
 macrophages are seen close to the damaged elasticmacrophages are seen close to the damaged elastic
lamina.lamina.

12. Giant Cell (Temporal) ArteritisGiant Cell (Temporal) Arteritis
(Morphology Cont..)(Morphology Cont..)
 Less common pattern, a nonspecific panarteritis with aLess common pattern, a nonspecific panarteritis with a
mixed inflammatory infiltrate (lymphocytes, macrophages,mixed inflammatory infiltrate (lymphocytes, macrophages,
neutrophils and eosinophils).neutrophils and eosinophils).
 Healed stage of both of these patterns reveals collagenousHealed stage of both of these patterns reveals collagenous
thickening of the vessel wall; organization of the luminalthickening of the vessel wall; organization of the luminal
thrombus sometimes transforms the artery into athrombus sometimes transforms the artery into a fibrousfibrous
cordcord..

13. Giant Cell (Temporal) Arteritis:Giant Cell (Temporal) Arteritis:
PathogenesisPathogenesis
 Evidence points to aEvidence points to a T-cell-mediated immuneT-cell-mediated immune response toresponse to
an unknown, possiblyan unknown, possibly vessel wall, antigenvessel wall, antigen..
 Supporting this hypothesis are a granulomatousSupporting this hypothesis are a granulomatous
inflammatory response with the presence ofinflammatory response with the presence of CD4+ T cellsCD4+ T cells..

14. Giant Cell (Temporal) Arteritis:Giant Cell (Temporal) Arteritis:
Clinical FeaturesClinical Features
 Rare before the age of 50 (F:M = 2:1) .Rare before the age of 50 (F:M = 2:1) .
 Symptoms are constitutional fever, fatigue, weight loss-Symptoms are constitutional fever, fatigue, weight loss-
without localizing signs or symptomswithout localizing signs or symptoms
 The diagnosis depends on biopsy and histologicThe diagnosis depends on biopsy and histologic
confirmation.confirmation.
 Treatment with anti-inflammatory agents is remarkablyTreatment with anti-inflammatory agents is remarkably
effective.effective.

15. Temporal (giant cell) arteritis.Temporal (giant cell) arteritis.
Giant cells at the degenerated internalGiant cells at the degenerated internal
elastic membrane in active arteritis andelastic membrane in active arteritis and
intimal thickening.intimal thickening.

16. Temporal (giant cell) arteritis.Temporal (giant cell) arteritis.
Elastic tissue stain demonstrating focalElastic tissue stain demonstrating focal
destruction of internal elastic membrane (arrow)destruction of internal elastic membrane (arrow)
and intimal thickening (IT) characteristic of long-and intimal thickening (IT) characteristic of long-
standing or healed arteritis.standing or healed arteritis.

17. Cardiac TumorsCardiac Tumors

18. Cardiac TumorsCardiac Tumors
 Heart tumor are rareHeart tumor are rare
 Metastatic NeoplasmsMetastatic Neoplasms: metastases may reach the heart via: metastases may reach the heart via
lymphatic, venous, or arterial channels.lymphatic, venous, or arterial channels.
 seen in up to 10% of patients dying of disseminatedseen in up to 10% of patients dying of disseminated
cancer, mostly involving pericardium.cancer, mostly involving pericardium.
 The most common primary neoplasms that metastasizeThe most common primary neoplasms that metastasize
to the heart are:to the heart are:
 carcinomas of thecarcinomas of the lung and breastlung and breast,,
 malignant melanomas,malignant melanomas,
 lymphomas & leukemiaslymphomas & leukemias..

19. Cardiac tumorsCardiac tumors
Primary tumors include:Primary tumors include:
 MyxomaMyxoma: is commonest heart tumor in adults, benign,: is commonest heart tumor in adults, benign, 90%90%
in Lt atrium.in Lt atrium.
 They appear as sessile or pedunculated gelatinous massThey appear as sessile or pedunculated gelatinous mass
covered by endotheliumcovered by endothelium
 Microscopically: multinucleated stellate (Microscopically: multinucleated stellate (Star-shapedStar-shaped))
cells, edema and mucoid stroma.cells, edema and mucoid stroma.

20. Cardiac tumorsCardiac tumors
 RhabdomyomaRhabdomyoma
 Common (Common (infancy and childreninfancy and children))
 Associated with tuberous sclerosisAssociated with tuberous sclerosis
 Grossly: myocardial masses project into the ventricularGrossly: myocardial masses project into the ventricular
lumen, solitary or multifocal.lumen, solitary or multifocal.
 Microscopically: eosinophilic, polygonal cells (containMicroscopically: eosinophilic, polygonal cells (contain
large, glycogen-rich cytoplasmic granules).large, glycogen-rich cytoplasmic granules).
 Lipoma, and Papillary Elastofibromas,Lipoma, and Papillary Elastofibromas,
 Sarcomas: Angiosarcomas, and Rhabdomyosarcomas.Sarcomas: Angiosarcomas, and Rhabdomyosarcomas.

21. Vascular TumorsVascular Tumors

22. Benign Neoplasms, Developmental and Acquired Conditions
Hemangioma
Capillary hemangioma
Cavernous hemangioma
Pyogenic granuloma (lobular capillary hemangioma)
Lymphangioma
Simple (capillary) lymphangioma
Cavernous lymphangioma (cystic hygroma)
Glomus tumor
Intermediate-Grade Neoplasms
Kaposi sarcoma
Hemangioendothelioma
Malignant Neoplasms
Angiosarcoma
Hemangiopericytoma
Classification of Vascular Tumors

23. Benign tumors: HemangiomasBenign tumors: Hemangiomas
 Characterized by increased numbers of normal orCharacterized by increased numbers of normal or
abnormal vessels filled with blood.abnormal vessels filled with blood.
 Mostly localized but may involve large segments of theMostly localized but may involve large segments of the
body (entire extremity) and called angiomatosis.body (entire extremity) and called angiomatosis.
 The majority are superficial lesions often of theThe majority are superficial lesions often of the head andhead and
neck, possible in liver.neck, possible in liver.
 Common in childhood and constitutes 7% of all benignCommon in childhood and constitutes 7% of all benign
tumors. May present at birth.tumors. May present at birth.
 The strawberry type of the skin of the newborn is commonThe strawberry type of the skin of the newborn is common
(juvenile hemangioma).(juvenile hemangioma).

24. Capillary HemangiomasCapillary Hemangiomas
 Capillary Hemangiomas are theCapillary Hemangiomas are the most common typemost common type. Mostly. Mostly
in the skin, subcutaneous tissues, and mucous membranesin the skin, subcutaneous tissues, and mucous membranes
of the oral cavity and lips. Many regress spontaneouslyof the oral cavity and lips. Many regress spontaneously
 Color (bright red to blue), size varies (mm to centimeters),Color (bright red to blue), size varies (mm to centimeters),
flat or slightly elevatedflat or slightly elevated
 Lobulated but unencapsulated aggregates of closelyLobulated but unencapsulated aggregates of closely
packed thin walled capillaries which are filled with bloodpacked thin walled capillaries which are filled with blood
and lined by flat benign endotheliumand lined by flat benign endothelium
 The Lumina may contain thrombiThe Lumina may contain thrombi

25. Hemangioma of the tongueHemangioma of the tongue

26. Cavernous HemangiomasCavernous Hemangiomas
 Less common, and characterized by large vascular spaces.Less common, and characterized by large vascular spaces.
 Cavernous Hemangiomas are less circumscribed andCavernous Hemangiomas are less circumscribed and
more frequentlymore frequently involve deep structuresinvolve deep structures..
 Rarely giant forms occur, that affects large subcutaneousRarely giant forms occur, that affects large subcutaneous
areas of the face or extremities.areas of the face or extremities.
 Are soft, red-blue measuring 1-2 cm.Are soft, red-blue measuring 1-2 cm.
 Histologically, sharply defined but not encapsulated.Histologically, sharply defined but not encapsulated.
Composed of large cavernous vascular spaces filled withComposed of large cavernous vascular spaces filled with
blood.blood.
 Are mostly of little clinical significance.Are mostly of little clinical significance.

27. Cavernous hemangioma

28. Pyogenic GranulomaPyogenic Granuloma
(Lobular capillary hemangioma)(Lobular capillary hemangioma)
 Polypoid form of capillary hemangiomas.Polypoid form of capillary hemangiomas.
 Occurs as rapidly growing red nodule attached byOccurs as rapidly growing red nodule attached by a stalka stalk toto
the skin and oral mucosa , which bleeds easily and isthe skin and oral mucosa , which bleeds easily and is
ulcerated.ulcerated.
 One third of the lesions develop after trauma.One third of the lesions develop after trauma.
 The proliferating capillaries are accompanied by edema andThe proliferating capillaries are accompanied by edema and
inflammatory cellsinflammatory cells
 The appearance resembles granulation tissue.The appearance resembles granulation tissue.
 Pregnancy tumor ( granuloma gravidarum) is a pyogenicPregnancy tumor ( granuloma gravidarum) is a pyogenic
granuloma that occurs in the gingival of pregnant ladies andgranuloma that occurs in the gingival of pregnant ladies and
regresses after deliveryregresses after delivery

29. Pyogenic granuloma of the lipPyogenic granuloma of the lip

30. Pyogenic granulomaPyogenic granuloma
Lobular capillary hemangiomaLobular capillary hemangioma

31. Glomus Tumor (Glomangioma)Glomus Tumor (Glomangioma)
 Benign but often painful tumorsBenign but often painful tumors arising from modifiedarising from modified
SMCs of theSMCs of the glomus bodyglomus body,, a specializeda specialized arteriovenousarteriovenous
structure involved in thermoregulation.structure involved in thermoregulation.
 They are most commonly found in the distal portion of theThey are most commonly found in the distal portion of the
digits, especially under the fingernails. Excision is curative.digits, especially under the fingernails. Excision is curative.
 Morphology: Glomus tumors are round, slightly elevated,Morphology: Glomus tumors are round, slightly elevated,
red-blue, firm nodules (generally much less than 1 cm inred-blue, firm nodules (generally much less than 1 cm in
diameter) that can initially resemble a minute focus ofdiameter) that can initially resemble a minute focus of
hemorrhage under the nail.hemorrhage under the nail.
 Histologically, these are aggregates, nests, and masses ofHistologically, these are aggregates, nests, and masses of
specialized glomus cells, all within a connective tissuespecialized glomus cells, all within a connective tissue
stroma.stroma.
 Individual tumor cells are small, uniform, and round or cuboidal,Individual tumor cells are small, uniform, and round or cuboidal,
with scant cytoplasm and ultrastructural features similar to SMCswith scant cytoplasm and ultrastructural features similar to SMCs

32. Borderline Malignancies:Borderline Malignancies:
HemangioendotheliomasHemangioendotheliomas
 A wide spectrum of vascular neoplasms showing histologicA wide spectrum of vascular neoplasms showing histologic
features and clinical behavior intermediate between benignfeatures and clinical behavior intermediate between benign
hemangiomas and angiosarcomas.hemangiomas and angiosarcomas.
 The most common isThe most common is epithelioid hemangio-endotheliomasepithelioid hemangio-endotheliomas
which occurs around medium sized and large veins in thewhich occurs around medium sized and large veins in the
soft tissues of adults.soft tissues of adults.
 Most are cured by excision but up to 40% recur and 30%Most are cured by excision but up to 40% recur and 30%
metastasize.metastasize.

33. EpithelioidEpithelioid
hemangioendothelioma.hemangioendothelioma.

34. Epithelioid hemangioendothelioma.Epithelioid hemangioendothelioma.
Prominent intracytoplasmic lumenProminent intracytoplasmic lumen
formationformation

35. Kaposi SarcomaKaposi Sarcoma
 A.A. Chronic typeChronic type::
 Called classic or European mainly occurs in elderlyCalled classic or European mainly occurs in elderly
 Red to purple nodules in the distal lower extremities,Red to purple nodules in the distal lower extremities,
increasing in size slowly and locally persistent.increasing in size slowly and locally persistent.
 B.B. LymphadenopathicLymphadenopathic::
 Called African or endemic mainly among children ofCalled African or endemic mainly among children of
south Africasouth Africa
 Localized or generalized lymphadenopathy. It is anLocalized or generalized lymphadenopathy. It is an
aggressive tumoraggressive tumor

36. Kaposi SarcomaKaposi Sarcoma
 C-C- Transplant AssociatedTransplant Associated::
 Occurs several months to a few years postoperatively inOccurs several months to a few years postoperatively in
solid organ transplant in recipient who receive high dosessolid organ transplant in recipient who receive high doses
of immunosuppressive therapy.of immunosuppressive therapy.
 Lesions are localized or generalizedLesions are localized or generalized
 Skin lesions may regress.Skin lesions may regress.
 D.D. AIDS associatedAIDS associated::
 In one fourth of AIDS patients especially homosexualsIn one fourth of AIDS patients especially homosexuals
 Common to involve lymph nodes and the gut.Common to involve lymph nodes and the gut.

37. Kaposi sarcomaKaposi sarcoma
A. Gross photograph illustrating coalescent red-purple maculesA. Gross photograph illustrating coalescent red-purple macules
and plaques of the skin.and plaques of the skin.
B. Histologic view of the nodular form demonstrating sheets ofB. Histologic view of the nodular form demonstrating sheets of
plump, proliferating spindle cells and vascular spaces.plump, proliferating spindle cells and vascular spaces.

38. Malignant tumors:Malignant tumors:
AngiosarcomasAngiosarcomas
 Occur in both sexes ant tend to affect adultsOccur in both sexes ant tend to affect adults
 Mostly affectsMostly affects skin, soft tissues, breast and liverskin, soft tissues, breast and liver..
 Hepatic angiosarcomas are associated with carcinogensHepatic angiosarcomas are associated with carcinogens
like arsenic.like arsenic.
 Shows local invasion and metastatic spread.Shows local invasion and metastatic spread.
 Has poor outcome.Has poor outcome.

39. AngiosarcomaAngiosarcoma
A. Gross photograph of angiosarcoma of the heart (right ventricle).A. Gross photograph of angiosarcoma of the heart (right ventricle).
B. Moderately well differentiated angiosarcoma with dense clumps ofB. Moderately well differentiated angiosarcoma with dense clumps of
irregular, moderate anaplastic cells and distinct vascular lumens.irregular, moderate anaplastic cells and distinct vascular lumens.
C. Immunohistochemical staining of angiosarcoma for the endothelial cellC. Immunohistochemical staining of angiosarcoma for the endothelial cell
markermarker CD31CD31, proving the endothelial nature of the tumor cells., proving the endothelial nature of the tumor cells.