Vascular Pathology Quiz
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Which condition is characterized by segmental, thrombosing inflammation of medium- and small-sized arteries, especially in smokers?

  • Infectious Vasculitis
  • Thromboangiitis Obliterans (correct)
  • Microscopic Polyangiitis
  • Granulomatosis with Polyangiitis

    • Microscopic polyangiitis lesions can vary significantly in age within the same patient.

    False

    What is the primary immune response mechanism involved in microscopic polyangiitis?

    Recruitment and activation of neutrophils

    Infectious vasculitis is commonly caused by _____ or fungi, particularly Aspergillus and Mucor species.

    <p>bacteria</p> Signup and view all the answers

    Match the following conditions with their key characteristics:

    <p>Microscopic Polyangiitis = Necrotizing glomerulonephritis and pulmonary capillaritis Thromboangiitis Obliterans = Vascular occlusion in heavy smokers Infectious Vasculitis = Caused by bacteria leading to mycotic aneurysms Granulomatosis with Polyangiitis = Associated with nasal and lung granulomatous inflammation</p> Signup and view all the answers

    What is the 5-year survival rate for patients with angiosarcomas?

    <p>30%</p> Signup and view all the answers

    Angiosarcomas typically begin as large, painful tumors.

    <p>False</p> Signup and view all the answers

    What is the primary immunohistochemical marker used to demonstrate the endothelial origin of angiosarcoma tumors?

    <p>CD31</p> Signup and view all the answers

    Giant Cell Arteritis primarily affects __________ arteries.

    <p>large- to small-sized</p> Signup and view all the answers

    Match the following mechanisms of vasculitis with their descriptions:

    <p>Immune-mediated inflammation = Involves immune complex deposition and antibody response Direct invasion = Involves infectious pathogens invading vascular walls</p> Signup and view all the answers

    What is a common complication of ophthalmic artery involvement in Giant Cell Arteritis?

    <p>Permanent blindness</p> Signup and view all the answers

    Angiosarcomas are unable to metastasize.

    <p>False</p> Signup and view all the answers

    What cytokine is particularly involved in the pathogenesis of Giant Cell Arteritis?

    <p>TNF</p> Signup and view all the answers

    Which type of hemangioma is most commonly found in newborns?

    <p>Juvenile hemangioma</p> Signup and view all the answers

    Cavernous hemangiomas are composed of large, dilated vascular channels that do not spontaneously regress.

    <p>True</p> Signup and view all the answers

    What is the typical regression age for juvenile hemangiomas?

    <p>by age 7</p> Signup and view all the answers

    Pyogenic granulomas often develop after __________.

    <p>trauma</p> Signup and view all the answers

    What type of lymphangioma is commonly found in Turner syndrome?

    <p>Cavernous lymphangiomas</p> Signup and view all the answers

    Match the type of hemangioma with its description:

    <p>Capillary hemangiomas = Thin-walled capillaries with scant stroma Juvenile hemangiomas = Common in 1 in 200 newborns Cavernous hemangiomas = Large, dilated vascular channels Pyogenic granulomas = Rapidly growing red lesions on skin</p> Signup and view all the answers

    Glomus tumors arise from endothelial cells.

    <p>False</p> Signup and view all the answers

    Which of the following statements about pyogenic granulomas is true?

    <p>They commonly occur in the oral mucosa and are often ulcerated.</p> Signup and view all the answers

    What virus is most commonly associated with intermediate-grade tumors in AIDS patients?

    <p>Human herpesvirus 8 (HHV8)</p> Signup and view all the answers

    Cavernous hemangiomas can be identified as benign lesions on imaging studies.

    <p>False</p> Signup and view all the answers

    The cutaneous lesions in Kaposi's sarcoma (KS) first appear as red-purple ________.

    <p>macules</p> Signup and view all the answers

    What is the histological characteristic of capillary hemangiomas?

    <p>thin-walled capillaries with scant stroma</p> Signup and view all the answers

    Match the types of Kaposi's sarcoma with their descriptions:

    <p>Classic KS = Multiple red-purple skin plaques in lower extremities Endemic African KS = Commonly seen in immunocompromised patients Transplant-associated KS = Occurs after organ transplantation AIDS-associated KS = Epidemic form of KS seen in AIDS patients</p> Signup and view all the answers

    Which marker is NOT associated with distinguishing simple lymphangiomas?

    <p>Presence of erythrocytes</p> Signup and view all the answers

    Excision is curative for glomus tumors.

    <p>True</p> Signup and view all the answers

    What do glomus bodies primarily participate in?

    <p>Thermoregulation</p> Signup and view all the answers

    Which of the following symptoms is most commonly associated with temporal arteritis?

    <p>Facial pain along the superficial temporal artery</p> Signup and view all the answers

    Ocular symptoms in temporal arteritis can include complete vision loss.

    <p>True</p> Signup and view all the answers

    What is required to confirm a diagnosis of temporal arteritis?

    <p>Biopsy and histologic confirmation</p> Signup and view all the answers

    Takayasu arteritis is characterized by ______ disturbances and marked weakening of the pulses in the upper extremities.

    <p>ocular</p> Signup and view all the answers

    Match the following characteristics to their respective type of arteritis:

    <p>Temporal arteritis = Rare before age 50 Takayasu arteritis = Usually affects those under 50 Giant cell aortitis = Typically seen in those over 50 Both = Can involve ocular symptoms</p> Signup and view all the answers

    What treatment is typically effective for giant cell arteritis?

    <p>Corticosteroids or anti-TNF therapies</p> Signup and view all the answers

    Takayasu arteritis can lead to severe luminal narrowing of major branch vessels.

    <p>True</p> Signup and view all the answers

    What common attribute do giant cell arteritis and Takayasu arteritis share?

    <p>Clinical features and histology</p> Signup and view all the answers

    What is the major cause of morbidity and death in Churg-Strauss syndrome?

    <p>Cardiomyopathy due to eosinophilic infiltrates</p> Signup and view all the answers

    Polyarteritis Nodosa (PAN) typically affects the pulmonary circulation.

    <p>False</p> Signup and view all the answers

    What type of inflammation is associated with the morphology of Polyarteritis Nodosa?

    <p>Segmental transmural necrotizing inflammation</p> Signup and view all the answers

    A typical presentation of Polyarteritis Nodosa includes rapidly accelerating hypertension due to _______ artery involvement.

    <p>renal</p> Signup and view all the answers

    What percentage of patients typically shows cardiac involvement in Churg-Strauss syndrome?

    <p>60%</p> Signup and view all the answers

    The majority of Polyarteritis Nodosa cases have a known cause.

    <p>False</p> Signup and view all the answers

    What type of cells are predominantly involved in the inflammatory infiltrate in the morphology of PAN?

    <p>Neutrophils, eosinophils, and mononuclear cells</p> Signup and view all the answers

    Study Notes

    Pathology of Veins and Lymphatics, Vascular Tumors, & Vasculitis

    • The presentation covers the pathology of veins and lymphatics, vascular tumors, and vasculitis.
    • The presenter is Dr. Aileen Azari-Yam M.D., Ph.D.

    Objective

    • Varicose veins
    • Phlebothrombosis/Thrombophlebitis

    Varicose Veins

    • Varicose veins are dilated, tortuous veins caused by prolonged increased intraluminal pressure leading to vessel dilation and venous valve incompetence.
    • Superficial veins of the upper and lower legs are commonly affected due to elevated venous pressure, potentially up to ten times normal, from prolonged standing.
    • Up to 20% of men and a third of women develop lower extremity varicose veins.
    • Obesity is a risk factor for varicose veins.

    Varicose Veins, Clinical Features

    • Incompetent venous valves cause stasis, congestion, edema, pain, and thrombosis.
    • Chronic venous congestion creates tissue ischemia leading to stasis dermatitis (brawny induration).
    • Hemolysis of red blood cells creates brawny coloration and ulcerations.
    • Poor wound healing and infections are common complications.
    • Thromboembolism from deep veins is more common than embolism from superficial veins.

    Varicosities in Two Other Sites: Esophageal Varices

    • Liver cirrhosis leads to portal hypertension.
    • Portal hypertension leads to the opening of portosystemic shunts, increasing blood flow to veins at the gastroesophageal junction, rectum, and periumbilical veins (caput medusa).
    • Esophageal varices may rupture and cause massive (potentially fatal) upper gastrointestinal (GI) hemorrhage.

    Varicosities in Two Other Sites: Hemorrhoids

    • Hemorrhoids result from primary dilation of the venous plexus at the anorectal junction, often due to prolonged pelvic vascular congestion, like pregnancy or straining during defecation.
    • Hemorrhoids are painful, uncomfortable swellings prone to bleeding and thrombosis.
    • Hemorrhoids are prone to ulceration.

    Thrombophlebitis = Phlebothrombosis

    • Venous thrombosis and inflammation.
    • Deep leg veins account for over 90% of cases.
    • Prolonged inactivity reduces blood flow leading to lower extremity deep venous thrombosis (DVT).
    • Risk factors for DVT include congestive heart failure, pregnancy, oral contraceptive use, malignancy, obesity, and systemic hypercoagulability.
    • Cancer, especially adenocarcinomas, can cause paraneoplastic syndromes and hypercoagulability.
    • Migratory thrombophlebitis (Trousseau syndrome) involves migrating venous thromboses.

    Clinical Aspects

    • Thrombi in the legs often have few visible signs or symptoms, especially in bedridden individuals.
    • Physical pain may be elicited by pressure over affected veins, squeezing calf muscles, or forced dorsiflexion of the foot.
    • Absence of symptoms does not exclude DVT.

    Pulmonary Embolism: A Serious Clinical Complication of DVT

    • DVT fragments and detaches, causing a pulmonary embolism.
    • Pulmonary embolism is a common manifestation of thrombophlebitis.
    • The outcome of pulmonary embolism varies from asymptomatic to fatal, depending on the size and number of emboli.

    Vascular Tumors

    • Vascular neoplasms originate from endothelial cells or from cells supporting blood vessels (e.g., glomus tumor).
    • Primary tumors of large vessels (aorta, pulmonary artery, vena cava) are frequently sarcomas.

    Benign Tumors (Hemangiomas)

    • Hemangiomas are very common tumors made of blood-filled vessels.
    • 7% of benign tumors in infants and children are hemangiomas.
    • Hemangiomas commonly regress, but frequently start at birth and increase in size.
    • They often present in the head, neck, and thoracic skin.
    • Types include capillary hemangiomas, juvenile hemangiomas, and pyogenic granulomas.

    Capillary Hemangiomas

    • The most frequent hemangioma type affecting the skin, subcutaneous tissues, oral mucosa and cavities, lips, liver, spleen, and kidneys.
    • Composed of thin-walled capillaries with little supporting stroma.

    Juvenile Hemangiomas (Strawberry-Type)

    • Extremely common in newborns (1 in 200).
    • Can be multiple hemangiomas.
    • Grow rapidly, then fade, and typically disappear by age 7 for most patients.

    Cavernous Hemangiomas

    • Composed of large dilated vascular channels.
    • More invasive, deep structures are frequently affected, and they don't typically disappear.
    • Imaging studies may not differentiate them from malignancy.
    • Composed of large cavernous blood-filled spaces separated by connective tissue stroma.
    • A component of von Hippel-Lindau disease where lesions commonly affect the cerebellum, brain stem, retina, pancreas, and liver.

    Pyogenic Granulomas

    • Rapidly growing red, pedunculated lesions on the skin, gingival tissues, and oral mucosa.
    • They frequently bleed easily and are often ulcerated.
    • A quarter of the lesions emerge after trauma and reach 1-2cm in size within weeks.
    • Curettage and cautery are typically curative.
    • Pregnancy tumors (granuloma gravidarum) are a subtype occurring infrequently (1%) in pregnant women's gingival tissues.
    • May regress spontaneously after pregnancy or undergo fibrosis, but may require surgical excision.

    Other Benign Lesions (Lymphangiomas)

    • Simple or capillary lymphangiomas are characterized by lymphatic endothelial markers (e.g., VEGFR-3, LYVE-1) and typically show absence of erythrocytes.
    • Cavernous lymphangiomas (cystic hygromas) occur in the neck and axilla of children and rarely affect the retroperitoneum.
    • Neck lesions are commonly linked to Turner syndrome.

    Glomus Tumors (Glomangiomas)

    • Benign, exquisitely painful tumors emerging from modified smooth muscle cells (SMCs) in glomus bodies.
    • Glomus bodies are arteriovenous structures involved in thermoregulation.
    • While superficially similar to hemangiomas, glomangiomas arise from SMCs rather than endothelial cells (ECs).
    • Commonly found in the distal digits, especially on fingertips.
    • Excision is a curative treatment.

    Intermediate-Grade (Borderline) Tumors (Kaposi Sarcoma)

    • Caused by human herpesvirus 8 (HHV8).
    • Most common in individuals with AIDS.
    • Classification includes classic KS, endemic African KS, transplant-associated KS, and AIDS-associated (epidemic) KS.
    • Classic KS involves multiple red-purple plaques or nodules in the lower extremities, spreading proximally.

    KS Pathogenesis

    • HHV8 causes lytic and latent infections in endothelial cells (ECs).
    • A virally encoded protein induces vascular endothelial growth factor (VEGF) production which stimulates endothelial growth.
    • Cytokines produced by inflammatory cells trigger local proliferation.
    • HHV8 encoded proteins disrupt normal cellular controls, like cycle controls (e.g., cyclin D homologues), and inhibit apoptosis by inhibiting p53.

    KS Morphology

    • Patches are red-purple macules with areas of dilated irregular EC-lined vascular spaces.
    • Interspersed lymphocytes, plasma cells, and macrophages (sometimes with hemosiderin) are common.
    • Lesions may be challenging to differentiate from granulation tissue.
    • Lesions can become larger, violaceous, raised plaques with dilated vascular channels.
    • Spindle cells surround the vascular channels; erythrocytes, hemosiderin-laden macrophages, and other mononuclear inflammatory cells are frequently found between vascular channels.
    • Nodular stage shows sheets of plump proliferating spindle cells embedded in dermal and subcutaenous tissues.
    • Small vessels and slit-like spaces with red blood cells, marked hemorrhage, hemosiderin pigments, and mononuclear inflammatory cells are prominent.
    • Mittic figures are common, with cytoplasmic globules representing degenerating erythrocytes.
    • The nodular stage typically precedes lymph node and visceral involvement.

    KS Clinical Features

    • Most primary HHV8 infections are asymptomatic.
    • Classic KS starts as superficial skin lesions.
    • Surgical resection is a frequent treatment option for multiple skin lesions.
    • Radiation therapy may be used for multiple lesions.
    • Chemotherapy may be used for disseminated diseases.

    Hemangioendothelioma

    • A spectrum of borderline vascular neoplasms that affects adults and involves blood vessels (veins).
    • Epithelioid hemangioendothelioma in is a subtype that commonly involves medium and large sized veins.
    • Well-defined vascular channels are often subtle and the neoplastic cells are frequently plump and often cuboidal resembling epithelial cells.
    • Clinical behaviours vary; however, most are typically cured by surgical excision.

    Angiosarcoma

    • Malignant endothelial neoplasms whose histology ranges from highly differentiated tumors that mimic hemangiomas to profoundly anaplastic lesions.
    • Commonly affects older adults (both men and women).
    • Commonly affects skin, soft tissue, breasts, and liver.
    • It can emerge in lymphedematous regions, especially after radical mastectomy and/or radiation therapy for breast cancer.

    Hepatic Angiosarcomas

    • Associated with many carcinogenic exposures, including arsenic (e.g., in pesticides), Thorotrast (a historic radioactive contrast agent), and polyvinyl chloride.
    • Long latency exists between exposure and tumor development.
    • Angiosarcoma is a locally invasive tumor type that readily metastasizes.
    • 5-year survival rates for hepatic angiosarcoma approximate 30%.

    Angiosarcoma, Morphology

    • Cutaneous angiosarcomas may begin as multiple, deceivingly small, asymptomatic papules or nodules that appear as red.
    • More advanced lesions manifest as large, fleshy masses ranging from red-tan to gray-white in colour with blurry margins into surrounding tissues.
    • Necrosis and hemorrhage are common.
    • Microscopically, the lesions display varied differentiation degrees from plump atypiacal endothelial cells (ECs) forming vascular channels, to tumors with no clear vascular patterns.
    • Endothelial nature is detectable by IHC staining for CD31 and von Willebrand factor.

    Vasculitis

    • The presentation covers mechanisms and examples of diseases categorized together as vasculitis.

    Pathogenic Mechanisms of Vasculitis

    • Immune-mediated inflammation, resulting from immune complex deposition, antineutrophil cytoplasmic antibodies (ANCAs), anti-EC antibodies, and autoreactive T cells.
    • Direct invasion of vascular walls by infectious pathogens.

    Giant Cell (Temporal) Arteritis

    • Chronic granulomatous inflammation of large-to-small arteries, often targeting those in the head.
    • Commonly affects elderly adults in the US and Europe.
    • Temporal arteries are frequently affected, along with vertebral and ophthalmic arteries, and the aorta (giant cell aortitis).
    • Prompt diagnosis and treatment are crucial because ophthalmic artery involvement can lead to permanent blindness.

    Giant Cell Arteritis, Pathogenesis

    • Result of a T-cell-mediated immune response to an unknown vascular wall antigen.
    • Proinflammatory cytokines (e.g., TNF) and anti-endothelial cell (EC) antibodies contribute.
    • The precise reason for the predilection for vessels in the head remains unknown.

    Temporal Arteritis, Clinical Features

    • Rare before age 50.
    • Symptoms may include non-specific symptoms such as fatigue, weight loss, fever, vague complaints of facial pain, and headaches, mostly intense along the superficial temporal artery.
      • Painful to palpation of the artery.
    • Ocular symptoms (associated with ophthalmic artery involvement) are a concern, ranging from diplopia to complete vision loss.
    • Diagnosis depends on biopsy.
    • A 1cm segment for adequate biopsy is crucial despite being sometimes spotty and focalised within an artery.
    • Negative biopsy results don't rule out the diagnosis fully.
    • Corticosteroids or anti-TNF therapies are generally effective.

    Takayasu Arteritis

    • A granulomatous vasculitis of medium and large arteries.
    • Characterized by ocular disturbances and weakened pulses in the upper extremities (also called pulseless disease).
    • Transmural fibrous thickening is common, especially in significant vessels like the aorta and its branches.
    • Features share similarities with giant cell aortitis; however, the distinction is frequently based on age. Patients over 50 are classified as having giant cell aortitis, while those under 50 are classified as Takayasu arteritis.
    • Global distribution of the disease.
    • The etiology is likely autoimmune.

    Takayasu Arteritis, Morphology

    • Typically affects the aortic arch, but involvement extends to other parts of the aorta and branches, frequently including the pulmonary artery, coronary, and renal arteries.
    • Thickening of the vessel walls and intimal hyperplasia is characteristic.
    • Involvement of the aortic arch often leads to narrowed or even obliterated great vessel lumens. This reduces peripheral pulse strength.

    Takayasu Arteritis, Morphology (Microscopic)

    • Microscopic changes vary from adventitial mononuclear cell infiltrates with perivascular cuffing of vasa vasorum
    • to intense mononuclear inflammatory infiltrates
    • including granulomas and giant cells throughout the arterial media.
    • Patchy medial necrosis can also occur.
    • Microscopic features tend to be similar to giant cell (temporal) arteritis.

    Takayasu Arteritis, Clinical Features

    • Initial symptoms are usually nonspecific such as weight loss, fever, and fatigue.
    • Vascular symptoms like weaker pulses in upper extremities, visual disturbances, retinal hemorrhages, total blindness, and neurological deficits prevail later during the disease.

    Clinical Features, Takayasu Arteritis (Continued)

    • Involvement of the more distal aorta commonly leads to lower limb claudication.
    • Pulmonary artery involvement may result in pulmonary hypertension.
    • Narrowing of coronary ostia can result in myocardial infarction.
    • Renal artery involvement frequently causes systemic hypertension.
    • The disease course varies both in terms of rapid progression or quiescent stage that last 1-2 years, leading to long-term survival with neurological or visual deficits.

    Churg-Strauss Syndrome

    • Allergic granulomatosis and angiitis (or a small-vessel necrotizing vasculitis).
    • Often associated with asthma, allergic rhinitis, lung infiltrates, peripheral eosinophilia, extravascular necrotizing granulomas, and eosinophilic infiltration of vessels and tissues.

    Churg-Strauss Syndrome (Clinical Features)

    • Cutaneous involvement (palpable purpura), gastrointestinal bleeding and renal disease (primarily focal and segmental glomerulosclerosis) are common.
    • Myocardial eosinophilic infiltrates often cause cardiomyopathy, which is a significant cause of morbidity and mortality.
    • The disease is thought to be triggered by hyperresponsiveness to a previously innocuous allergic stimulus.
    • Many cases associate with myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCAs).
    • Microscopic examination typically shows both granulomas and eosinophils.

    Polyarteritis Nodosa (PAN)

    • Systemic vasculitis affecting small and medium sized muscular arteries.
    • Typically involves renal and visceral vessels.
    • The pulmonary circulation remains unaffected.
    • A third of affected patients show chronic hepatitis B with the presence of HBsAg-HbsAb complexes in affected vessels.
    • The etiology is not consistently known in a vast majority of cases.

    PAN, Morphology

    • Segmental transmural necrotizing inflammation of small and medium sized arteries.
    • Often features superimposed aneurysms and/or thrombosis.
    • The kidney, heart, liver, and gastrointestinal tract vessels tend to be affected in descending order.
    • Lesions frequently involve only a variable portion of the vessel circumference with a predilection for branch points.
    • Impaired perfusion of affected areas leads to ulcerations, infarcts, ischemic atrophy, or hemorrhage.

    PAN, Morphology (Microscopic)

    • Transmural inflammation shows a mixed infiltrate of neutrophils, eosinophils, and mononuclear cells.
    • Fibrinoid necrosis and luminal thrombosis are frequent accompanying features.

    Microscopic Polyangiitis

    • Hypersensitivity vasculitis or leukocytoclastic vasculitis.
    • Necrotizing vasculitis that frequently affects capillaries as well as small arterioles and venules.
    • Lesions at various stages of development are equally prevalent for microscopic polyangiitis in a single patient.
    • Typically associated with a single episode of antibody or immune complex deposition.

    Microscopic Polyangiitis (Clinical Features)

    • The skin, mucous membranes, lungs, brain, heart, gastrointestinal tract, kidneys, and muscles can be affected.
    • Necrotizing glomerulonephritis and pulmonary capillaritis are common.
    • Most cases show an association with myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCAs). The neutrophils' recruitment and activation are responsible for the manifestations.

    Thromboangiitis Obliterans (Buerger Disease)

    • Characterized by segmental, thrombosing, acute and chronic inflammation of medium and small sized arteries.
    • Mostly affects the tibial and radial arteries in the extremities.
    • Exclusively seen in heavy cigarette smokers, typically before age 35, possibly due to idiosyncratic endothelial cell (EC) toxicity.

    Thromboangiitis Obliterans (Buerger Disease), Morphology

    • Affected vessels show acute and chronic inflammation with luminal thrombosis.
    • Thrombi may feature microabscesses of neutrophils.
    • Inflammatory process extends to nearby veins and nerves.
    • In time, inflammation will encapsulate all three structures in a fibrous tissue.

    Infectious Vasculitis

    • Infectious agents like bacteria or fungi (e.g., Aspergillus and Mucor) may cause this condition.
    • Vascular infections can result in the weakening of arterial walls leading to mycotic aneurysms.
    • Thrombosis and downstream infarction can also occur following a vascular infection.

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