Pathology Of Cardiovascular Tumors

Question 1

Vascular tumors characteristics

Answer 1

Usually being

1) benign
2) locally aggressive
3) highly malignant

Question 2

Differences between benign and malignant vascular tumors

Answer 2

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

Question 3

Vascular ectasias

Answer 3

Ectasia is defined as any local dilation of a structure

can be congenital or acquired and ARE NOT true neoplasms bythemselves

Question 4

Telangiectasia

Answer 4

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

Question 5

What is the most common form of vascular ectasias?

Answer 5

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

Question 6

Port wine stain

Answer 6

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

Question 7

Sturge-Weber syndrome

Answer 7

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

Question 8

Spider telangiesctasis

Answer 8

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)

Question 9

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)

Answer 9

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

Question 10

Hemangiomas

Answer 10

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

Question 11

Histologic and clinical variants of hemangiomas

Answer 11

Capillary hemangiomas
- most common variant of hemangiomas

Juvenile hemangiomas
- also known as “strawberry hemangiomas”

pyogenic granulomas

cavernous hemangiomas

Question 12

Capillary hemangiomas

Answer 12

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

Question 13

Juvenile hemangiomas

Answer 13

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

Question 14

Pyogenic granulomas

Answer 14

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

Question 15

Cavernous hemangiomas

Answer 15

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

Question 16

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

Answer 16

Cavernous hemangiomas

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

Question 17

Simple lymphangiomas

Answer 17

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

Question 18

Cavernous lymphangiomas

Cystic hygromas

Answer 18

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

Question 19

Glomangiomas (Glomus tumors)

Answer 19

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

Question 20

Bacillary angiomatosis

Answer 20

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

Question 21

Pathology behind bacillary angiomatosis

Answer 21

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

Question 22

Kaposi sarcomas (KS)

Answer 22

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

MOST common in AIDS patients

Question 23

4 forms of KS

Answer 23

Classical

Endemic African

Transplantation-associated

AIDS-associated

Question 24

Classic kaposi sarcoma

Answer 24

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

Question 25

Endemic African KS

Answer 25

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*

Question 26

Transplantation-associated KS

Answer 26

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)

Question 27

AIDS-associated (epidemic) KS

Answer 27

* 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

Question 28

KSHV (HHV-8) information

Answer 28

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)

Question 29

Classic KS morphology

Answer 29

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

Question 30

Treatment of the 4 types of KS

Answer 30

*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

Question 31

Hemangioendotheliomas

Answer 31

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

Question 32

Angiosarcomas

Answer 32

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%

Question 33

Carcinogens associated w/ angiosarcomas

Answer 33

Arsenical pesticides and polyvinyl chloride | - most commonly produce angiosarcomas in the liver and take multiple years to develop

Question 34

Angiosarcomas histologically morphology

Answer 34

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

Question 35

Praimry cardiac tumors

Answer 35

Super uncommon and almost all are benign | - the 5 most common (accounting for 80-90%) have no malignancy potential

Question 36

The 5 most common cardiac tumors

Answer 36

*in order of most common -> least common* 1) myxomas 2) fibromas 3) lipomas 4) papillary fibroelastomas 5) rhabdomyomas

Question 37

Myxomas

Answer 37

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*

Question 38

Rhabdomyomas

Answer 38

*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

Question 39

Morphology of rhabdomyomas

Answer 39

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”)

Question 40

What are the most common origin for heart metastasis

Answer 40

``` Lung Breast Melanomas Leukemias Lymphomas ```

Question 41

Ways that metastasis reach the heart and pericardium

Answer 41

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