Prevalence
-rhabdo is #1
-0.14% of fetal referrals for echo are for tumor
-rhabdo 89%, then fibromas and hemangiomas; another report found 64% where rhabdo, then teratoma, fibroma, hemangiomas
-referred often for tumor on OB scan, dysrhythmias, hydrops, pericardial effusion, or TS FHx
-check for CHF, adjusted cardiac Q, check for masses in chambers, septae, pericardial space, external...
-check if it is hetero or homogeneous, vascular, or cystic
-check if margins are smooth, irregular, lobulated
-check size of masses
-check heart size for CM, effusions, Doppler inflow/outflow for obst check for AV vlv regurg..., venous Dopplers...
Fetal MRI
-limited by motion...
Additional considerations
-check fullOB scan for cardiac tumors in fetuses
-amnio c/s if other xx
-eval for signs of TS- cerebral tubers - can check w MRI
-note that TS expression varies...
-rhabdo doesnt correlate w degree of other TS xx
Prenatal Mgt
-follow for CHF/hydrops, arrhyth, obstruction
-tumor location/obst was more important than size
-might have brady bc of compression of SA nd (?)
-obstruction can occur at any pt in gestation, so follow serially, espec post age of viability..., might deliver early...
-if mild Sx postnatally, might delay surg bc many regress...
-can delay delivery w a drain for pericardial effuson
-Tx arrhythmias
-c/s delivery to ECMO if needed...
Outcome
-survival is improving, but it d/o tumor type and HD xx
-n=224 fetus/neonate w cardiac tumor, 55% survival. 89 of these had prenatal dx, and 66^ of these survived, vs 47% if postnatal survival.
-hemangiomas did best- 83% survival; rhabdo had 67% survival; fibromas did worst 50% survival
Rhabdo
- = 89% of fetal cardiac tumors in 1 study, but others report 64%
-often incidental finding or bc of dysrhythmia on OB US, or bc FHx of TS
-usually sessile, at IVS or free wall of ventricles, but might be at AV groove, in pap muscles, or atria
-smooth, multiple, lobulated
-c/s rhabdomyosarcoma if they are clustered, irregular, or fragmented
-homogeneous, diffusely echogenic, more than myocardium
-1/2 are intracavitary
-may --> dysrhythmia, or WPW
-incr in size prenatally, then regress postnatally
-might have Nl US at 20 weeks, then later show tumor
-in 1 study, tumors grew in proportion to GA until 30-32 weeks GA, then were stable in size
0the rest of pts it grew larger, disproportionately to the heart size, and new tumors arose
-81% of these pts had +TS (ref 46)
Pathology
-benign tumors, well circumscribed, nonencapsulated
-made of large, round, vacuolated cells w strands extending outward--> spider cell look
Tuberous Sclerosis
-found in all pts w multiple tumors, 1/2 of pts w single tumors + no FHx in one study
-Sx
-MR, sz, hamartomatous nodular malformations of brain/viscera, skin hypopigmentation,
-no clear assoc w the rhabdos and other TS Sx
-TOF & HLHS has been seen in TS
-TS is auto dom - TSC1 and TSC2 genes --> timor suppressors hamartin and tumerin, chrom 91 and 16p...
-can do prenatal genetic testing for family, or CVS at 10-12 wks GA, or amnio at 15-18weeks gA
Mgt & Outcome
-early study found 50% mortality by 6mo old and 80% mortality by 1 yr
-but recent reports only minimal to no HD xx prenatally, even some in utero improvement
-?bc now we detect less severe tumors...
-?bc early Tx/surgery to improve outcome
-recent study showed 67% survival after prenatal dx
-Most centers are conservative- no intervention unless HD xx
Fibromas
-5% of fetal cardiac tumors
-usually incidental finding - mass on OB US, or dysrhythmia
-~always single, usually at LV myocardium or IVS, but maybe RA/RV
-uniform echogenicity, so cannot ddx fr rhabdo
-may hve central cystic degen'n and Ca'n, and then appear heterogeneous
-+/- assoc pericard effusion seen
-+/- in/out obstructoin, CHF, SVT/VT
-no assoc genetic xx
-round, nonencapsulated, firm, white tumors. benign - w fibroplasts, collagen fibers, elastic tissue, Ca'ion
-Px- poor Px if Dx in fetus- 50% survival to delivery, 29% survival p neonate
-surgery done in some, no one without surgery survived
-death is fr arrhyth or HF
Hemangiomas
-uncommon, 5% or less of masses
-base of the heart or near RA, or at pericardium, myocardium, ventricle
-intracavitary obstruction possible, +/- PCE too
-often displace heart to left hemithorax by the tumor
-might invade the AV nd--> hrt block
-mixed echogenicity bc of endothelial cells at diff stages of organization/thrombosis
-Path- benign, with either capillary or cavernous form
-Px- better than most tumors- but may --> HD signif PCE, so might need surg
-83% fetal survival in 1 report, expect it to regress postnatally
Teratomas
-usually extracardiac, intrapericardial, attached to Ao rt or PA
-rare inside the heart
-2nd most common tumor = 23% of fetal card tumor
-mixed echogenicity, often + PC effusion
-most --> Sx of hydrops or resp distress as a neonate
-Path- benign usually, but can be malignant; have cysts w mucoid stroma, early embryonic tissue fr 3 germ layers--> mesoderm yeilds muscle, haline, cartilage, endoderm --> bronhial/panc/GI/salivary, ectodermal--> neuroeiptheial tissue/choriod plexus, eyes
-Px- 2nd best survival rate, 75% in benign teratomas, but only 65% w prenatal dx, many of them die prenatally,
-many get resection, usually curative
-might need to drain PCE
Echogenic foci
-often normal, but might be a true tumor
-benign echogenic foci nearly always are in LV within the pap muscles
-one study found it in 20% of 2nd/3rd tri fetuses, usually smaller and much more echogenic than true tumors
-Path- mineralization within pap muscle, some w calcification
-Px- no change in size, no Sx