Cardiac Tumors (M/A)

Cardiac Tumors (MA76)

-most are benign (>90%)

PRIMARY BENIGN TUMORS

Epi:

-0.02% incidence

-Types- rhabdomyomas, fibromas, myxomas, teratomas, hemangiomas

Dx:

-CXR abNl in 80% pts- CM, abNl silhouette, Ca'ion

-ECG abNl in 47%- ST abnly, strain, ventric hypertrophy' +/-dysrhythmias

-Echo- great for dx

-MRI- also good- location, number, size of tumors; see mediastinum etc

-Angio- indirect, nonSp...

Rhabdomyomas:

-half or more tumors are rhabdo

-may have prenatal Dx, sudden death or stillbirth

-multiple, well circumscribed, noncapsulated, white/gray intramural/intracavitary nodules anywhere in heart (usually ventricles).

-considered hamartomas- cells unable to undergo mitosis ???

-rarely, can have a diffuse form where much of myocardium/conduction system has rhabdo histologic changes

-see recurrent atrial tachy and sudden death fr VT

-Sx/Si d/o location, may --> HF or conduction xx/dysrhythmias...

-on echo they are multiple, well circ intramural/intracavitary nodules anywhere, or as a single pedunculated intracav/mural mass; homogeneous echo-bright, finely speckled pattern

-DDx thrombi, myxomas, & hemangiomas are circumscirbed echolucent areas (bc of hemorrhages)

-Assoc w Tuberous Sclerosis- auto dom transmission, but most are sporadic mutations

-52-79% of fetuses w cardiac tumors have Tuberous Sclerosis

-60-80% pts w +rhabdo cv tumor have Tub Sclerosis

-->shagreen patches, adenoma sebaceum, sz, MR, brain tubors...

-Surgery can be very succesful, w/o myocardial/valve xx if intracavitary

-Even large tumors might reguress w/o Tx

-So no surgery unless life threatening HD xx or arrhythmias

Fibromas:

-#2 CV tumor, ?#3

-mainly in 1mo to 1yr, rare in older pts

-assoc w Gorlin syndrome- multiple nevoid basal cell carcinomas, jaw cysts, diffuse skeletal abNlies

-single, white, firm, nonencapsulated, intramural tumors, at LV free wall/IVS, often at apex

-might be multiple or invade RV free wall, atria

-if extensive it could encroach into/obliterate ventric cavity

-might entangle MV or TV--> regurg

-made of fibroblasts, collagen fibers

-Sx- d/o size/location...; can cause Ao/pulm obst, CHF etc,

-ECG might show BBB or delayed progression of the terminal R wave, or QRS deviation, big atria, or ventric hypertrophy...; ST changes of strain or ischemia

-Might present w bad arrhythmias- av block, VT, rarely SVT

-echo- single, bright, intramural, echogenic mass at IVS or LV wall usually +/-Ca'ion,

-Unlike rhabdo, they don't get smaller

-Surgery if signif HD xx, OHT if extensive...

Myxomas:

-often Dx in 20-50s

-in kids they look like CHD, in older kids can --> severe M&M

-Pt often has only vague constitutional Sx (fever, malaise, wt loss, arthralgias, myalgias; anemia/thrombocytopena, high ESR, high gamma globulins)

-Most in LA alone, or RA alone less often

-friable, pedunculated, gelatinous, yellow-brown/red lobular tumors +/-Ca'ion

-Pedicle usually attached to FO; can protrude fr LA into RA

-histo- pale paucicellular myxoid background, w small bld vssls, lymhos

-rarely are they malignant (more pleomorphic)

-Sx- cardiac obstruction, emboli, and systemic illness

-80% w valve obst Sx --> MS/TS by to and fro motion through the valves asthe pedunculate tumor goes thru the AV vlv during diastole, then back to atrium during systole

-may obst pulm vn inflow

-can--> RH or LH failure...

-might prolapse into ventricle then obstruct Pulm vlv/Ao vlv

-hear valve regurg on exam

-if obstruct AV vlv, might --> dyspnea, dizzy, syncope w sit/standing, improved w laying down

->70% have peripheral emboli fr fragmentation of the tumor or fr thrombi on it's surface

-Echo-

-see pedunculated tumor in atria, check if cross AV vlv...

-Tx

-must remove surgically at Dx

-good surgical results w resolved sx postop

-must remove it all or it may return.... (4-7% recurrence rate)

-+FHx in 7% cases

-Assoc dz- multiple lentigines syndromes....

Intrapericardial Teratomas:

-rare

-seen in fetus/newborn

-high mortality, improving w earlier dx/tx

-single, encapsulated, gray-tan, bosselated tumors, attached to base of the heart

-w multiple cysts in a yellow mucoid stroma

-broad based stalk or narrow pedicle attaches it to root of the Ao or PA

-gets Q fr Ao vasa vasorum fr a single bld vssls near cor art origin

-might be 3-4x size of the baby's heart!, and in older kids might be relatively small

--> obstruct/compress heart bc of mass effect of the solid tumor w restrictive fibrous pericardium

-usually R sided, attaching to Asc Ao, wedged bn Ao and SVC

-rarely could be intracardiac

-1/2 Dx by <1mo, 2/3 by 1yo

-Sx- distant/muffled heart sounds, absent precordial impulse

-HSM/diminished pulses

-may p/w cyanosis fro compression of pulm parench or fr a R to L atrial shunt

-ECG- low voltage QRS; NO arrhythmias, ST changes, block

-Pericardiocentesis- only Dx'ic if +bloody effusion which is always assoc w malignant cardiac or pericardial tumor

-Echo- single, nonhomogeneous, lobular, intrapericardial mass

-cystic formations are echo lucent

-Ca'ions are echhogenic foci

-check attachmt to Ao/PA and for GA/chamber compression

-MRI helps ddx intrapericardial teratoma and bronchogenic cysts

-High survival rates in critically ill infants if early dx, surgical removal, pericardial effusion decrompression

-Surgery for older ASx pts bc they can cause sudden death

Other Primary Benign Myocardial Tumors:

-hemangiomas- single, at epicardial/intramural/intracavityar space

-polypoid or sessile, w central necrosis/Ca'ion

-made of large bld vssls and small vasc channels within myocardium

-Echo- vasc channels are large echolucent areas

-may regress w IFN or steroids

-surgery has been successful, c/s OHT if severe invasion

-Papillary tumors, accessory endocardial cushion tumors, cardiac lipomas, fibroelastomas

PRIMARY MALIGNANT MYOCARDIAL TUMORS

-<10% of all cv tumors

-fibrosarcoma, angiosarcoma, lymphosarcoma, giant cell sarcoma, fibromyxosarcoma, leiomyosarcoma, neurogenic sarcooma, rhabdomyosarcom

-angiosarcomas #1- often in RA and pericardium

--> tamponade, RH fail, SVC obst; hemorrhageic pericardial effusion

-mets to liver, lungs, CNS common

-poor Px despite chemo

-Cardiac sarcomas- rare in alla ges

SECONDARY CARDIAC TUMORS

-more freq than primary tumors

-fr non-Hodgkin lymphoma, leukemia, neuroblastoma

-can p/w arrhythmias, effusions, CHF

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