Pathology Of Cardiovascular Tumors Flashcards
Vascular tumors characteristics
Usually being
1) benign
2) locally aggressive
3) highly malignant
Differences between benign and malignant vascular tumors
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
Vascular ectasias
Ectasia is defined as any local dilation of a structure
can be congenital or acquired and ARE NOT true neoplasms bythemselves
Telangiectasia
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
What is the most common form of vascular ectasias?
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
Port wine stain
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
Sturge-Weber syndrome
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
Spider telangiesctasis
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)
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)
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
Hemangiomas
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
Histologic and clinical variants of hemangiomas
Capillary hemangiomas
- most common variant of hemangiomas
Juvenile hemangiomas
- also known as “strawberry hemangiomas”
pyogenic granulomas
cavernous hemangiomas
Capillary hemangiomas
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
Juvenile hemangiomas
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
Pyogenic granulomas
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
Cavernous hemangiomas
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
What hemangioma is a component of Von Hippel-Lindau disease?
Cavernous hemangiomas
Often found in cerebellum, brain stem, retina, pancreas and liver when a part of Von hippel- Lindau disease
Simple lymphangiomas
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
Cavernous lymphangiomas
Cystic hygromas
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
Glomangiomas (Glomus tumors)
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
Bacillary angiomatosis
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
Pathology behind bacillary angiomatosis
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
Kaposi sarcomas (KS)
Vascular neoplasm that is caused by kaposi sarcoma herpesvirsus (KSHV or HHV-8)
MOST common in AIDS patients
4 forms of KS
Classical
Endemic African
Transplantation-associated
AIDS-associated
Classic kaposi sarcoma
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
Endemic African KS
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
Transplantation-associated KS
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)
AIDS-associated (epidemic) KS
- 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
KSHV (HHV-8) information
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)
Classic KS morphology
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
Treatment of the 4 types of KS
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
Hemangioendotheliomas
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
Angiosarcomas
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%
Carcinogens associated w/ angiosarcomas
Arsenical pesticides and polyvinyl chloride
- most commonly produce angiosarcomas in the liver and take multiple years to develop
Angiosarcomas histologically morphology
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
Praimry cardiac tumors
Super uncommon and almost all are benign
- the 5 most common (accounting for 80-90%) have no malignancy potential
The 5 most common cardiac tumors
in order of most common -> least common
1) myxomas
2) fibromas
3) lipomas
4) papillary fibroelastomas
5) rhabdomyomas
Myxomas
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*
Rhabdomyomas
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
Morphology of rhabdomyomas
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”)
What are the most common origin for heart metastasis
Lung Breast Melanomas Leukemias Lymphomas
Ways that metastasis reach the heart and pericardium
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
