Tumor Flashcards

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

A

10%

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
Q

fibroma

A

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
Q

pheochromocytoma

A

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
Q

cell lineage of pheochromocytoma

A

chromaffin cells.

produce catecholamines (esp NE)
urine catecholamines diagnostic (also CT & MRI)
28
Q

teratomas

A

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
Q

type of cell in sarcomas

A

mesenchymal cells

30
Q

most common types of primary cardiac sarcomas

A

angiosarcoma
rhabdomyosarcoma
fibrosarcoma

31
Q

sarcomas

A

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
Q

sarcoma symptoms

A

nonspecific (fever malaise weight loss)
pericardial effusion
HF

33
Q

prognosis of cardiac sarcomas

A

90% dead by 1 year

with surgery: MR 7%; median survival 2 yrs

34
Q

surgery for sarcoma

A

complete resection; autotransplantation on left side to allow for radical rsection

indicated iin young pts without evidence of distal mets

35
Q

what proportion of patients with carcinoid tumors get malignant carcinoid syndrome?

what proportion of pts with malignant carcinoid syndrome get carcinoid heart disease

A

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
Q

cell lineage of carcinoid tumors

A

argentaffin cells of GI tract (ileum, also pancreas, biliary vessels, ovaries adn testes)

secrete large amt of serotonin

37
Q

malignant carcinoid syndrome: sx

A

flushing, telangiectasias, diarrhea, bronchospasm

hyperdynamic circulation but no HTN

Dx: increased serum serotonin & urine 5-HIAA

38
Q

carcinoid heart disease

A

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
Q

what disease does carcinoid heart resemble

A

fen-phen valve disease

fenfluoromine, phentermine diet drugs

40
Q

surgery of carcinoid heart disease

A

TVR +/- PVR

30 day mortality:
50% if over 60 y.o.
0% if under

operate on young pts with imp HF sx