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Cardiology > Pathology Of Cardiovascular Tumors > Flashcards

Pathology Of Cardiovascular Tumors Flashcards

1
Q

Vascular tumors characteristics

A

Usually being

1) benign
2) locally aggressive
3) highly malignant

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2
Q

Differences between benign and malignant vascular tumors

A

Benign tumors:

  • composed of vascular blood cells/lymph
  • typical appearances w/monolayer of well organized endothelial cells

Malignant tumors

  • much more cellular
  • proliferative and atypical appearances
  • NO well-organized vessels
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3
Q

Vascular ectasias

A

Ectasia is defined as any local dilation of a structure

can be congenital or acquired and ARE NOT true neoplasms bythemselves

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4
Q

Telangiectasia

A

A form of vascular ectasias that describes permanent dilation of preexisting small vessels that form discrete red lesions

can be congenital or acquired and ARE NOT true neoplasms bythemselves

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5
Q

What is the most common form of vascular ectasias?

A 
Nevus flammeus (birthmark ectasias) 
- light pink/deep purple flat lesion on the head or neck that is composed of dilated vessels

these most commonly regress spontaneously without treatment

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6
Q

Port wine stain

A

A special form of nervus flammeus that is commonly found on the head and neck

  • very large and tend to grow during childhood
  • thicken overtime and DO NOT fade
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7
Q

Sturge-Weber syndrome

A

A specific type of port wine stains that has a distribution pattern along the trigeminal nerve

  • very uncommon and is associated with the following conditions
    1) seizures
    2) mental retardation
    3) radiologic opacities
    4) hemiplegia
    5) venous angiomas in cortical leptomeninges
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8
Q

Spider telangiesctasis

A

Non neoplastic vascular lesions that appear as pulsatile arrays of dilated subcutaneous arteries or arterioles
- look like legs of a spider that blanch w/ pressure applied

Most common sites are face/neck.upper chest
- very associated with hyper estrogen is states (such as pregnancy or patients w/ cirrhosis)

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9
Q

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)

A

Autosomal dominant disorder caused by mutations in genes that encode Components of the TGF-B signaling pathways in endothelial cells
- TGF-B is disrupted

Generates telangiectasias that are distributed over the skin, GI and oral/nasal mucous membranes

These telangiectasis rupture spontaneously causing potential nosebleeds, GI bleeds or hermaturia

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10
Q

Hemangiomas

A

Very common tumors that are composed of blood-filled vessels

comprise 7% of all benign tumors in infancy and childhood

Typically localized lesions that are confined to the head and neck region

Very rare malignant transformation, so usually remain benign

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11
Q

Histologic and clinical variants of hemangiomas

A

Capillary hemangiomas
- most common variant of hemangiomas

Juvenile hemangiomas
- also known as “strawberry hemangiomas”

pyogenic granulomas

cavernous hemangiomas

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12
Q

Capillary hemangiomas

A

Most common type of hemangiomas

Usually occur in the skin, subcutaneous tissues and mucous membranes of the oral cavities/lips
- in rarer instances, can also present in the liver, spleen and kidneys

Composed of thin-walled capillaries w/ scant stroma

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13
Q

Juvenile hemangiomas

A

Also known as “strawberry hemangiomas”
- extremely common in newborns (1:200)

Can be multiple at one time

Grow rapidly for a few months, but often fade by 1-3 years of age
- complete regression is almost always accomplished by 7 years of age

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14
Q

Pyogenic granulomas

A

Capillary hemangiomas that present as rapidly growing red, pedunculated lesions
- most common locations are skin, and oral/lip mucosal layers

Bleed very easily and often ulcerated without treatment

25% will develop after trauma

Requires curettage and cautery to cure

  • curettage = scalpel off
  • cautery = cauterize off
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15
Q

Cavernous hemangiomas

A

Composed of large dilated vascular channels that are more infiltrative and generate in deep structures of the body

These do not spontaneously regress

Appear as a sharply defined unencapsulated mass w/ large blood-filled vascular spaces in histologically views

Overtime, presents w/ intravascular thrombosis and dystrophic calcifications if left untreated
- these are locally destructive but not common

Most commonly, overtime they will be asymptomatic, but can rupture due to trauma and vulnerable to bleeding/ulcerations

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16
Q

What hemangioma is a component of Von Hippel-Lindau disease?

A

Cavernous hemangiomas

Often found in cerebellum, brain stem, retina, pancreas and liver when a part of Von hippel- Lindau disease

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17
Q

Simple lymphangiomas

A

Slightly elevated pedunculated lesions that are small (1-2cm)
- common found in the head/neck/axillary or subcutaneous tissues

Appear composed of networks of endolthium-lined spaces similar vascular ectasias histologically
- ONLY difference in appearance histologically is NO RBCs

18
Q

Cavernous lymphangiomas

Cystic hygromas

A

Found around the neck or axilla of children

Can be large filling the entire axilla or showing gross deformities of the neck

Composed primarily of massively dilated lymphatic spaces lined by endothelial cells and CT stroma containing lymphoid aggregates

Margins are undefined and not capsulated (makes resection difficult)

  • VERY common in tuner syndrome patients*
  • 1 X chromosome in females
19
Q

Glomangiomas (Glomus tumors)

A

Benign and VERY painful tumors arising form SMCs in glomus bodies

  • glomus bodies are found in areas where thermoregulation are important (usually extremities)*
  • very common under fingernails as well and is cured by excision
20
Q

Bacillary angiomatosis

A

Vascular proliferation in immunocompromised patients (especially AIDS patients)
- caused by the bartonella family of bacteria

Produces lesions on the skin,bone,brain or other organs
- skin lesions show red papules/nodules that may or may not be rounded

Two species of bartonella are the most common

  • bartonella henselae: cat scratch fever
  • bartonella Quintana: body lice and trench fever

is defined as Warthin-starry positive when confirmed

21
Q

Pathology behind bacillary angiomatosis

A

The bartonella species drive host tissues to produce hypoxia-inducible factor-1a (HIF-1a)

HIF-1a drives VEGF production and vascular proliferation

These are curable via antibiotic treatments

22
Q

Kaposi sarcomas (KS)

A

Vascular neoplasm that is caused by kaposi sarcoma herpesvirsus (KSHV or HHV-8)

MOST common in AIDS patients

23
Q

4 forms of KS

A

Classical

Endemic African

Transplantation-associated

AIDS-associated

24
Q

Classic kaposi sarcoma

A

A disorder of older men usually of Mediterranean or middle eastern descents
- also includes Eastern European descent

uncommon in US And not associated with HIV or immunosuppressive

Manifests as multiple red-purple skin plaques or nodules usually in the distal lower extremities
- if untreated, increase in size and number and will spread proximally

Usually asymptomatic and remain localized

25
Q

Endemic African KS

A

Occurs in younger (>40yrs) individuals w/out HIV/AIDS

Can be indolent or aggressive and usually manifests in the lymph nodes

More frequent than classic
most common tumor in central Africa

26
Q

Transplantation-associated KS

A

Occurs only in individuals that have received a solid organ transplant w/ T-cell immunosupression

Often involves lymph nodes, mucosa and skin
- very aggressive

Can be treated via removing immunosupression (however this puts the organ at risk)

27
Q

AIDS-associated (epidemic) KS

A
  • MOST common HIV-related malignancy*
  • 1000x higher than normal people (2-3% of all HIV cases)

Involves the lymph nodes and disseminates systemically pretty early

If not treated, further immunosupression the patient and will therefore further increase the risks of dying from opportunistic infections

28
Q

KSHV (HHV-8) information

A

Is a y-herpesvirus similar to Epstein-Barr virus

Is very likely to be transmitted through sexual contact, oral secretions and blood exposures

  • in order to get KS from KSHV, the patient MUST also have immunosuppressive T-cell immunity*
  • can be immunosupression or just by getting older

Causes lyric and latent infections in ECs by doing the following

  • induces VEGF (stimulates endothelial growth)
  • mass produces cytokines from inflammatory cells
  • disrupt normal cellular proliferation controls and prevents apoptosis vis inhibiting p53 (only latent)
  • polyclonal lesions, but overtime become monoclonal (only latent)
29
Q

Classic KS morphology

A

Numerous cutaneous lesions that progress through 3 stages based on longevity

  • patchy
  • plaque
  • nodule

Patches are pink/red/or purple macules and typically stay in the distal lower extremities

Plaques are purple/black macules that become more proximal and raised

Nodular lesions are deep proximal containg proliferating spindle cells w/ interspersed slitlike spaces.
- this stage occurs quicker and more widespread in African and AIDS associated

30
Q

Treatment of the 4 types of KS

A

generally, they are asymptomatic

Classic KS:
- surgical resection w/ excellent prognosis

Transplant-associated KS:
- withdrawal of immunosupression therapy w/ good prognosis

AIDS-associated KS:

  • HIV anti-retro viral therapies in acute
  • radiation therapy and chemo in chronic widespread
  • both produce fair prognosis
31
Q

Hemangioendotheliomas

A

Vascular neoplasms that behave in between benign hemangiomas and malignant angiosarcomas
- arise most commonly in medium-large sized veins

Usually treated via excision

  • however curability is variable, since it has a high chance of reoccurring
  • 15% mortality rate
32
Q

Angiosarcomas

A

Malignant endothelial neoplasms with highly variable tumor characteristics
- can resemble benign tumors or anaplastic lesions and anywhere in between

Most common in older adults and has no gender preference

Can occur anywhere, but most common in skin/soft tissue, breasts and liver
- arise in the setting of lymphedema and usually present several years after lymph node rejections/breast cancer treatments

Very aggressive and metastasize always
- current 5-year survival is 30%

33
Q

Carcinogens associated w/ angiosarcomas

A

Arsenical pesticides and polyvinyl chloride

- most commonly produce angiosarcomas in the liver and take multiple years to develop

34
Q

Angiosarcomas histologically morphology

A

EXTREMELY VARIABLE
- range from atypical endothelial cells alone w/ channels -> undifferentiated spindle cell tumors without any recognizable blood vessels

Has CD31 and vWF markers in the tumors

35
Q

Praimry cardiac tumors

A

Super uncommon and almost all are benign

- the 5 most common (accounting for 80-90%) have no malignancy potential

36
Q

The 5 most common cardiac tumors

A

in order of most common -> least common

1) myxomas
2) fibromas
3) lipomas
4) papillary fibroelastomas
5) rhabdomyomas

37
Q

Myxomas

A

Most common primary tumors of the adult heart

90% of them occurs in the atria
- (80% of these 90% are in the left atrium)

Symptoms:

  • fever
  • malaise
  • obstructive valve symptoms
  • embolisms

Presents these symptoms due to increased IL-6 release.

  • most common way to diagnosis is use of TEE and seeing myxoma*
  • treated by simple resection*
38
Q

Rhabdomyomas

A

most frequent tumors of infant and children hearts

Almost always discovered secondarily to valvular or outflow obstruction

Caused by loss of Tumor Suppressor genes 1 and 2 (TSC-1/2)

  • loss of these genes leads to myocyte overgrowth
  • because of these mutations being the cause, these tumors are often associated or generated via tuberous sclerosis
39
Q

Morphology of rhabdomyomas

A

Gray-white masses that protrude into the ventricular chambers

Histologically shows mixed populations of cells
- most common cells are large, rounded polygonal cells containing numerous vacuoles w/ cytoplasm (these are called “spider cells”)

40
Q

What are the most common origin for heart metastasis

A 
Lung
Breast 
Melanomas 
Leukemias 
Lymphomas
41
Q

Ways that metastasis reach the heart and pericardium

A

Reach the heart and pericardium via 4 ways:

1) retrograde lymphatic extension
- carcinomas

2) hematogenous seeding
- all other non-carcinomas that metastasis to the heart

3) direct continguous extension
- used by lung breast or esophageal cancers

4) venous extension
- used by kidney/liver cancers

All 4 often produce pericardial effusions once manifested in the heart

Cardiology (46 decks)

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