Tumor Flashcards
primary tumors of the heart:
% benign vs malignant
90% benign
10% malignant
list benign primary cardiac tumors in adult (decreasing frequency)
- myxoma
- lipoma
- papillary fibroelastoma
- rhabdomyoma
- fibroma
- hemangioma
- teratoma
- mesothelioma of AV node
- granular cell tumor
- neurofibroma
- lymphangioma
list malignant primary cardiac tumors
- angiosarcoma
- rhabdomyosarcoma
- mesothelioma
- fibrosarcoma
- malignant lymphoma
- osteosarcoma
- neurogenic sarcoma
- malignant teratoma
- thymoma
- leiomyosarcoma
- liposarcoma
- synovial sarcoma
how to differentiate myxoma vs thrombi
Myxoma is covered by endothelium and have endothelium-lined crevices and clefts
cell lineage of myxomas
pluripotent mesenchymal stem cells
Krikler suggests endocardial sensory nerve cells
embolization risk of myxomas
LV 64% (usually to brain)
LA 30-45%
RA or RV 10%
constitutional sx of myxomas (esp large LA)
fever/wt loss
clubbing of fingers and toes (usually assoc’t w R->L shunt via PFO)
raynaud’s phenomenone
myalgia/arthralgia
blood work findings of myxomas
high total globulin level high ESR/CRP polycythemia hemolytic anemia (esp w calcified myxomas) thrombocytopenia
sporadic (non-familial myxomas)
95% of myxomas middle aged women 94% single 75% in LA 20% aneuploidy rarely assoc'd w conditions recurrence in 1-3%
familial myxomas
5% young men dominant inheritance 33% multiple 62% in LA 100% aneuploidy recurrence in 30-75% assoc't conditions
associated conditions with familial myxomas
- sertoli cell tumors of testes
- cushing syndrome due to primary adrenocortical nodular dysplasia
- pituitary tumors
- centrofacial and labial lentiginosis
- cutaneous myxomas
- multiple myxoid mammary fibroadenomas
predominant sx of LA myxoma
episodic dyspnea & hemoptysis associated with syncopy
can rapidly progress to intractable heart faillure
predominant sx of RA myxoma
episodic right heart failure (prominant A wave, elevated CVP, hepatomgaly, ascie, peri edema)
absence of orthopnea and PND
once symptomatic death from myxoma occurs within…..years
1-2 years
best study for myxomas
TEE
which chamber to inject radioopaque dye if cathing for myxoma
LA myxoma: inject in PA and wait for dye to get to LA
RA myxoma: inject in cava
*do not cannulate the chamber with the myxoma
mode of myxoma recurrence
average length of time before recurrence
- tumor seeding at time of removal
- incomplete removal
- multicentric origin
30months
lipoma
mature fatty cells; can be encapsulated or diffusely infiltrative
occur anywhere in the heart; atrial septum most common (lipomatous hypertrophy of interatrial septum)
mostly incidental findings
if too large: SCD, VT, ST-T abn, RVOT/LVOT obstruction
–> remove
Papillary fibroelastoma % of all cardiac tumors
10%
papillary fibroelastoma most likely location
AV cusp/MV leaflet
TV, IVS infrequently
aortic wall rarely
papillary fibroelastoma problem
embolism (either the fronds or thromboembolism)
brain, coronary arteries, lungs, retinal arteries
when to resect papillarry fibroelastoma
when risk of OR is low, or when embolic event
list primary cardiac tumors in children
- rhabdomyoma
- cardiac fibroma
- myxoma
rhabdomyoma
most common primary tumor in kids
associated with tuberous sclerosis
benign (spider cell); almost 100% in ventricles (RV=LV); multiples
regresses completely before age 2
resect only if RVOT/LVOT obstruction or ventricular arrythmias
fibroma
benig, spindle cells mixed with collagen adn elastic fibers
w central foci of calcificcation
exclusively in ventricular myocardium; freq in IVS
solitary, circumscribed, firm, grey white
resect if symptomatic (dyspnea, syncope, HF, CP, VT) or very large. consider transplant if unresecctable
pheochromocytoma
very rarely involves cardiac structures (within pericardium, on surface of the heart esp LA, PVs, Aorta and mPA, & coronary arteries)
if diagnosed, resect. surgical mortality 25% due to hemorrage
preop alpha & beta blockade key
cell lineage of pheochromocytoma
chromaffin cells.
produce catecholamines (esp NE) urine catecholamines diagnostic (also CT & MRI)
teratomas
rare
cyctic masses, compresses the heart
80% benign, 20% malignant
diagnoed in fetus and infants; occ in adults
leads to early cardiac death unless resected
type of cell in sarcomas
mesenchymal cells
most common types of primary cardiac sarcomas
angiosarcoma
rhabdomyosarcoma
fibrosarcoma
sarcomas
malignant tumors
metastasize widely
right side: more bulky & infiltrative, causes heart failure, met early
left side & PA: more circumscribed, cause HF early adn met late
sarcoma symptoms
nonspecific (fever malaise weight loss)
pericardial effusion
HF
prognosis of cardiac sarcomas
90% dead by 1 year
with surgery: MR 7%; median survival 2 yrs
surgery for sarcoma
complete resection; autotransplantation on left side to allow for radical rsection
indicated iin young pts without evidence of distal mets
what proportion of patients with carcinoid tumors get malignant carcinoid syndrome?
what proportion of pts with malignant carcinoid syndrome get carcinoid heart disease
10% get MCS
50% of MCS get carcinoid heart
so 5% of pts w carcinoid tumors get carcinoid heart disease!
*nearly all pts with carcinoid heart have hepatic mets!
cell lineage of carcinoid tumors
argentaffin cells of GI tract (ileum, also pancreas, biliary vessels, ovaries adn testes)
secrete large amt of serotonin
malignant carcinoid syndrome: sx
flushing, telangiectasias, diarrhea, bronchospasm
hyperdynamic circulation but no HTN
Dx: increased serum serotonin & urine 5-HIAA
carcinoid heart disease
in 50% of pts w the syndrome; worse outcome
limited to right sided vavles (TR and PS)
glistening white-yellow fibrous deposits on ventricular aspect of TV and downstream of PV
leads to constriction of annulus and adherence of leaflet to RV endocardium
what disease does carcinoid heart resemble
fen-phen valve disease
fenfluoromine, phentermine diet drugs
surgery of carcinoid heart disease
TVR +/- PVR
30 day mortality:
50% if over 60 y.o.
0% if under
operate on young pts with imp HF sx
