-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
...