Tumor Flashcards

(40 cards)

1
Q

primary tumors of the heart:

% benign vs malignant

A

90% benign

10% malignant

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2
Q

list benign primary cardiac tumors in adult (decreasing frequency)

A
  1. myxoma
  2. lipoma
  3. papillary fibroelastoma
  4. rhabdomyoma
  5. fibroma
  6. hemangioma
  7. teratoma
  8. mesothelioma of AV node
  9. granular cell tumor
  10. neurofibroma
  11. lymphangioma
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3
Q

list malignant primary cardiac tumors

A
  1. angiosarcoma
  2. rhabdomyosarcoma
  3. mesothelioma
  4. fibrosarcoma
  5. malignant lymphoma
  6. osteosarcoma
  7. neurogenic sarcoma
  8. malignant teratoma
  9. thymoma
  10. leiomyosarcoma
  11. liposarcoma
  12. synovial sarcoma
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4
Q

how to differentiate myxoma vs thrombi

A

Myxoma is covered by endothelium and have endothelium-lined crevices and clefts

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5
Q

cell lineage of myxomas

A

pluripotent mesenchymal stem cells

Krikler suggests endocardial sensory nerve cells

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6
Q

embolization risk of myxomas

A

LV 64% (usually to brain)
LA 30-45%
RA or RV 10%

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7
Q

constitutional sx of myxomas (esp large LA)

A

fever/wt loss
clubbing of fingers and toes (usually assoc’t w R->L shunt via PFO)
raynaud’s phenomenone
myalgia/arthralgia

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8
Q

blood work findings of myxomas

A
high total globulin level
high ESR/CRP
polycythemia
hemolytic anemia (esp w calcified myxomas)
thrombocytopenia
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9
Q

sporadic (non-familial myxomas)

A
95% of myxomas
middle aged women
94% single
75% in LA
20% aneuploidy
rarely assoc'd w conditions
recurrence in 1-3%
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10
Q

familial myxomas

A
5%
young men
dominant inheritance
33% multiple
62% in LA
100% aneuploidy
recurrence in 30-75%
assoc't conditions
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11
Q

associated conditions with familial myxomas

A
  • 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
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12
Q

predominant sx of LA myxoma

A

episodic dyspnea & hemoptysis associated with syncopy

can rapidly progress to intractable heart faillure

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13
Q

predominant sx of RA myxoma

A

episodic right heart failure (prominant A wave, elevated CVP, hepatomgaly, ascie, peri edema)

absence of orthopnea and PND

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14
Q

once symptomatic death from myxoma occurs within…..years

A

1-2 years

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15
Q

best study for myxomas

A

TEE

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16
Q

which chamber to inject radioopaque dye if cathing for myxoma

A

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

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17
Q

mode of myxoma recurrence

average length of time before recurrence

A
  • tumor seeding at time of removal
  • incomplete removal
  • multicentric origin

30months

18
Q

lipoma

A

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

19
Q

Papillary fibroelastoma % of all cardiac tumors

20
Q

papillary fibroelastoma most likely location

A

AV cusp/MV leaflet

TV, IVS infrequently

aortic wall rarely

21
Q

papillary fibroelastoma problem

A

embolism (either the fronds or thromboembolism)

brain, coronary arteries, lungs, retinal arteries

22
Q

when to resect papillarry fibroelastoma

A

when risk of OR is low, or when embolic event

23
Q

list primary cardiac tumors in children

A
  1. rhabdomyoma
  2. cardiac fibroma
  3. myxoma
24
Q

rhabdomyoma

A

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

25
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
26
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
27
cell lineage of pheochromocytoma
chromaffin cells. ``` produce catecholamines (esp NE) urine catecholamines diagnostic (also CT & MRI) ```
28
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
29
type of cell in sarcomas
mesenchymal cells
30
most common types of primary cardiac sarcomas
angiosarcoma rhabdomyosarcoma fibrosarcoma
31
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
32
sarcoma symptoms
nonspecific (fever malaise weight loss) pericardial effusion HF
33
prognosis of cardiac sarcomas
90% dead by 1 year with surgery: MR 7%; median survival 2 yrs
34
surgery for sarcoma
complete resection; autotransplantation on left side to allow for radical rsection indicated iin young pts without evidence of distal mets
35
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!
36
cell lineage of carcinoid tumors
argentaffin cells of GI tract (ileum, also pancreas, biliary vessels, ovaries adn testes) secrete large amt of serotonin
37
malignant carcinoid syndrome: sx
flushing, telangiectasias, diarrhea, bronchospasm hyperdynamic circulation but no HTN Dx: increased serum serotonin & urine 5-HIAA
38
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
39
what disease does carcinoid heart resemble
fen-phen valve disease | fenfluoromine, phentermine diet drugs
40
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