cardiac tumors: the role of echocardiography · cardiac masses classification * pfe arising de-novo...
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Καρδιακοί όγκοι: Ο ρόλος του Υπερηχογραφήματος
Cardiac Tumors: The role of echocardiography
Σοφία Μ. ΑράπηΕπιμελήτρια Α’ Καρδιολογικής Κλινικής
ΓΝΑ ‘Γ. Γεννηματάς’
Disclosure:
Nothing to declare
Primary cardiac tumors:
Autopsy studies: 0.01- 0.1%
Surgeries : 0.3%
75%(-85%) benign (>50% myxomas)
(15%-) 25% malignant (95% sarcomas)
Cardiac metastases:
20-fold to 40-fold more common
about 10-15% of all tumor patients (only
rarely clinically manifested-10%):
- in general population autopsy studies: 0.7- 3.5%
- in pts with diagnosed cancer: 9.1-20%
- in pts with multiple metastases: 14.2%
Burke A, et al: Heart 2008; 94: 117- 123, Bruce C: Heart 2011; 97: 151- 160, Goldberg A, et al: Circulation 2013; 128: 1790- 1794
Hoffmeier A et al. Dtsch Arztebl Int 2014; 111(12): 205-11
Cardiac Tumors
Cardiac Tumors—Diagnosis and Surgical TreatmentAndreas Hoffmeier, Jürgen R. Sindermann, Hans H. Scheld, Sven Martens
Cardiac masses classification
* PFE arising de-novo
+ PFE arising in setting of hypertrophic obstructive cardiomyopathy or following endocardial injury
Bruce C: Heart 2011; 97: 151- 160
Primary
Diagnostic Algorithm for Evaluation of a Cardiac Mass
Bruce C: Heart 2011; 97: 151- 160
H
Age
Location
Imaging
Relative incidence of primary benign heart tumors
% of Group
TUMOR Adults Children
Infants
MyxomaLipomaPapillary fibroelastomaRhabdomyomaFibromaHemangiomaTeratomaMesothelioma of AV nodeGranular cell tumorNeurofibromaLymphangiomaHamartoma
462116235131110
1500
461551340101
000
651241820000
Children
1) Age
Relative incidence of primary malignant heart tumors
% of Group
TUMOR TYPE Adults Children
Infants
AngiosarcomaRhabdomyosarcomaMesotheliomaFibrosarcomaMalignant lymphomaExtraskeletal osteosarcomaThymomaNeurogeic sarcomaLeiomyosarcomaLiposarcomaSynovial sarcomaMalignant teratoma
3321161164331110
03301100011000
44
06603300000000
95% sarcomas 5% lymphomas
1) Age
Dujardin KS, et al: J Am Soc Echocardiogr 2000; 13: 1080- 1083, Burke A, et al: Heart 2008; 94: 117- 123
2) Location
Clinical Presentation
General symptoms fever, weight loss, exhaustion, muscle pain, night sweats, coughing, leukocytosis, arthralgia, rush, Raynaud phenomenon (secretion of various factors i.e. IL6, endothelin)
EmbolismsPulmonary or peripheral artery embolism by detached tumor tissue or thrombotic deposit (particular myxomas due to gelatinous structure, small friable tumors)
Cardiac manifestations - obstruction
tumors in atria or AV valves: mimick MVS, TVSMobile, pediculated neoplasms lead to paroxysmal dyspnea or syncope (depending on posture), HF
- Tumor infiltration /expansionSymptoms of hypertrophic or restrictive CMP, HFSVC syndrome, pericardial effusion
- arrhythmiasInfiltration of neural pathways of myocardium, AV block (fibromas), SCD
Symptoms from metastases i.e. sarcoma to lung, brain, bones
Burke A, et al: Heart 2008; 94: 117 123, Elbardissi AW, et al: Stroke 2009; 40: 156Hoffmeier A et al. Dtsch Arztebl Int 2014; 111(2):205-11
Nonspecific symptoms depending on tumor location, size and infiltration,regardless of tumor type
Salcedo EE, et al: Curr Probl Cardiol 1992; 17: 73- 137
3) Imaging: + Non-invasive tissue characterization
Echocardiographic evaluation of cardiac masses
SVC, IVC, PV,
direct access through a wall
Plana JC. MDCVJ, VI(3) 2010
(sessile or
pedunculate)
Echocardiographic evaluation of cardiac masses
Plana JC. MDCVJ, VI(3) 2010
(Hypereosinophilic syndrome)
+ Clinical milieu
+ 3D-Echo, contrast echocardiography, deformation imaging
Non-invasive tissue characterisation(composition of the mass)
echogenicity of the mass and whether calcification is present
Vascularity can also be assessed using colour flow Doppler and echocardiographic contrast
Strain imaging also has potential in identifying the non-contractile nature of masses such as fibromas
Myocardial Contrast
Echocardiography
better delineation of endocardial
tumor
Vascularity assessment (contrast
perfusion imaging):
hyperenhancement of malignant & highly
vascular tumors
myxomas: partial enhancement (<adjacent
myocardium)
thrombi: no enhancement
targeted microbubbles (tumor binding peptides for ultrasound
imaging of tumor angiogenesis/ molecular
imaging
Kirkpatrick JN, et al: J Am Coll Cardiol 2004; 43: 1412- 1419, Weller GE, et al: Cancer Res 2005; 65: 533- 539
poorly differentiated adenocarcinoma
LV apical thrombus
LV haemangioma
better delineation of endocardial
tumor
better delineation of endocardial tumor
…
Vascularity assessment (contrast perfusion imaging)
better delineation of endocardial tumor
Vascularity assessment (contrast perfusion imaging):
hyperenhancement of malignant & highly vascular tumors
Vascularity assessment (contrast perfusion imaging):
hyperenhancement of malignant & highly vascular tumors
Deformation Imaging
Ganame J, et al: Eur J Echocardiogr 2005; 6: 461- 64, Peters PJ, et al: J Am SocEchocardiogr 2006; 19: 230- 240
Rhabdomyoma
Fibroma
LV rhabdomyoma: tumor of cardiac myocytes with
vacuoles containing glycogen (soft mass).
TDI for tissue characterization of the tumor:
Rhabdomyoma deformed in the opposite direction of the normal myocardium
Fibroma did not deform at allRV fibroma: tumor composed of fibroblasts and
collagen. Hard mass, difficult to deform
3D Echocardiography in the assessment of cardiac tumors: the added value of the extra dimension
Detailed description of the location, shape, attaching interface & relationship to adjacent structures. Unparalleled anatomic detailed achieved with 3D-MTEE
2D TEE/TTE underestimate the max diameter of irregularly shaped structures (by 19.8% and 24.6% respectively), while RT3DE measurements are fast, with excellent intra- & interobserver variability
More comprehensive assessment of the inner structure of the mass, that correlates better with pathologic findings (necrosis, hemorrhage, cystic areas or fibrotic bands)
Added value in the evaluation of embryonic remnants and normal variants (i.e. false chords, prominent Eustachian valve or chiarinetwork, prominent IVC ridge or crista terminalis)
Image acquisition is less operator-dependent
Accurate estimation of LV volumes and EF (cardiotoxicity of therapy)
Plana JC MDCVJ VI(3)2010, Nanda NC et al. Echocariography 1995, Asch FM e al. Echocardiography 2006, Mehmood F et al Echocardiography 2005
Paraskevaidis et al. ISRN Oncology 2011
Algorithm for the detection and differential diagnosis of a cardiac tumor
Yuan SM, et al: Cardiology J 2009; 16: 26– 35, Cooper LT, et al: J Am Coll Cardiol 2007; 50: 1914- 1931
+ staging
Endomyocardial Biopsy
• Diagnosis cannot be established by noninvasive modalities (such as cardiac MR) or less invasive (non-cardiac) biopsy
• Tissue diagnosis can be expected to influence the course of therapy
• Chances of successful biopsy are believed to be reasonably high
• The procedure is performed by an experienced operator
Role of Echocardiography for cardiac tumor
assessement & treatment
To guide interventions:
biopsy (TTE/ΤΕΕ, ICE)
pericardiocentesis
surgical Tx
(TEE, epicardial echo)
Malignant lymphoma
Tumor
Tumor
Tumor
ICE- guided biopsy
Abramowitz Y, et al: Int J Cardiol 2007; 118: e39 – e40, Higo T, et al: Circ J 2009; 73: 381 – 383
Biopsy catheter
Primary benign cardiac tumors
Myxoma
Rhabdomyoma
Papillary Fibroelastoma
Lipoma
Fibroma
rare: hemangioma, mesothelioma, teratoma, pericardial cyst
Myxoma
25% of all cardiac tumorsup to 50-70% of all primary benign cardiac tumors
Mainly middle age (30-60 yrs), women: 2/3In children only 10% of benign tumors
Histopathology:Benign neoplasms of multipotent mesenchymal cells in
the subendocardial tissue
Polygonal, possibly multinucleate cells with eosinophiliccytoplasm, surrounded by myxoid stroma
Degenerative changes:cystic formation, hemorrhages, fibroses, calcifications(10-20%), gland formation (lithomyxoma)
Paraskevaidis AI. Et al. ISRN Oncology 2011, Hoffmeier A et al. Dtsch Arztebl Int 2014; 111(12):205-11Burke A, et al: Heart 2008; 94: 117- 123
Macropathology/ consequences:
Usually 5-6cm (as large as 15cm)
Soft, gelatinous consistency - surface often coveredwith thrombotic material
Embolization of fragments of tumor may also occur(30- 40% (50% initial clinical presentation))
Systemic effects i.e. fever (20%, IL-6)
Myxoma
Hoffmeier A et al. Dtsch Arztebl Int 2014; 111(12):205-11Burke A, et al: Heart 2008; 94: 117- 123
Bruce C: Heart 2011; 97: 151- 160
Macropathology/consequences:
Usually grow on pedicles – may behighly mobile
may prolapse through AV valve ("ballvalve" effect by intermittentlyoccluding the atrioventricular valveorifice – 30%) (tumor plop (1/3 ofpts), dyspnea, syncope)
globular with regular & smooth surface with stalk not homogenous echogenicity,
with areas of echolucency or calcification (d.d. thrombus)
multilobular irregular, friable
surface no apparent stalk
Myxoma
Sporadic Familial or Syndrome Myxoma
• Solitary• More common• Usually located in left
atrium (75%) (18% in right atrium)
• Arise from inter-atrialseptum in vicinity of fossa ovalis
• May also occur in the ventricles (LV:3%, RV:4%) or multiple locations (including valves)
Hoffmeier A et al. Dtsch Arztebl Int 2014; 111(12):205-11Burke A, et al: Heart 2008; 94: 117- 123
• Carney syndrome• Subforms: NAVE and
LAMB syndromes• Autosomal dominant pattern
of transmission• Mutation of tumor suppressor
gene PRKAR1A (chromosome 17q22-24)
• Younger individual (<3rd decade)
• Often multiple/atypical location
• Less common (7-10%)
• Associated with freckling,endocrine neoplasms,non-cardiac tumors
• Recurrent after surgery
recurrence 2%- 13%, esp in young individuals: 22% in familial vs 3% in sporadic cases
ΤΤΕ every 6mos (esp for the 4 years postoperatively)
Papillary Fibroelastomas
• Endocardial papilloma:
- benign tumor of the (valvular) endocardium
- 10% of primary benign cardiac tumors
- 3/4 of all tumors of the cardiac valves
• average age: 60 years (4th-8th decade)
• strong association with HOCM, surgical or
haemodynamic trauma, radiation
• solitary (or multiple) location: >95% left
heart
• small [<1cm (7-12mm)], pedicled
VALVES: 77% (middle portion of leaflet)
- AV: 44% (mainly aortic side)
- MV: 35% (mainly atrial side)
- TV: 15%
- PV: 8%
mural non-valvular endocardium: 23%Gowda R, et al: Am Heart J 2003; 146: 404- 10
•White, gelatinous structure reminiscent of a sea anemone, usually small (1-2cm)
•Highly mobile tumors with a frond-like appearance (echo: shimmering effect)
• Potential for systemic or pulmonary embolism
• AV PFE: syncope, MI, SCD (impingement on coronary ostia)
•Tumor mobility = independent predictor of death or nonfatal embolization
•30-47% incidental finding
Gowda R, et al: Am Heart J 2003; 146: 404- 10, Koniari I, et al: Interact CardioVasc Thorac Surg 2009; 9: 922- 923,
Papillary Fibroelastomas
....
PFE on AV: 44% (mainly aortic side)
PFE on VALVES: 77%
PFE on MV: 35% (mainly atrial side)PFE on MV: 35% (mainly atrial side)
PFE on VALVES: 77%
PFE on mural non-valvular endocardium (23%)- here LVOT
PFE on mural non-valvular endocardium (23%)- here LVOT
Wolber T, et al: Circulation 2001; 104: e87- e88
LAA Papillary Fibroelastoma
Well-defined head with echolucencies and stippled pattern near the edges-‘shimmering’ or ‘vibrating’ effect
ΤΤΕ/ΤΕΕ (method of choice):
superior resolution of TEE makes it
the definitive imaging modality
MRI (motion artifact)
CT scanning (low temporal resolution, 3D reconstruction) - preoperatively CT angiograms are generally preferred in order to avoid manipulating the tumours into the coronary ostia during CAA
Lembcke A, et al: Circulation 2007; 115: e3- e6
Papillary Fibroelastomas:
Diagnosis
pedunculated, spherical mass (arrows) located in both the left and noncoronary sinus slightly above the aortic valve
• Other cardiac tumors (myxoma, fibroma, rhabdomyoma, metastatic)
• Thrombi: laminated appearance, irregular of lobulated border, microcavitations, absence of pedicle
• Vegetations: valvular destruction, clinical signs of endocarditis
• Mitral annular calcification
• Lambl’s excrescences: filliform fronds that occur on valvular contact margins (at sites of valve closure), smaller, multiple, shared pathogenesis (;)
Bruce C: Heart 2011; 97: 151- 160
Papillary Fibroelastomas
Differential diagnosis
LAA thrombus
MV vegetations
Lambl’s
excrescences
• surgical excision:
symptomatic patients
asymptomatic patients when large, highly mobile tumor / in RC with PFO R-L shunt
• Oral anticoagulation
small, immobile, asymptomatic
Follow-up (treat at emergence of symptoms or mobility)
• no recurrence
Gowda R, et al: Am Heart J 2003; 146: 404- 10
Gopaldas RR et al. TexasHeart Institute Journal 2009
Papillary Fibroelastomas
Treatment algorithm
homogeneous, relatively rounded, well-encapsulated masses
ususally subepicardial tumors in LV, RA and IAS – hypoechoic when in pericardial space
If endomyocardial: broad base, hyperreflective
Arise from benign neoplastic proliferation of mature adipocytes
Often asymptomatic (may cause arrhythmias, conduction system disturbances, obstruction / HF when large / If subepicardial: compression of the heart, pericardial effusion)
diagnosis: CT scanning (low- attenuation), MRI
Zhang J, et al: Singapore Med J 2009; 50: e342, Stephant E, et al: Circulation 2008; 118; e71- e72
Lipomas
hyperplasia and accumulation of adipose tissue on the IAS, except for the fossa ovalis membrane
brown fat, width: > 2.0 cm, high echogenicity
characteristic dumbbell shape
Elderly and obese male patients
rare superior vena cava syndrome, atrial arrhythmias;
Takayama T, et al: Circ J 2007; 71: 986 – 989
Lipomatous hypertrophy of the
interatrial septum
Commonest primary cardiac tumor in children (40-60%) (1st year of life)
Focal hamartomatous accumulation of striated
cardiomyocytes – not actually a neoplasm
Usually in LV, RV, IVS or AV junction, often multiple (90%)
Usually intramural – intracavitary extension up to 50%
50% associated with tuberous sclerosis
50% spontaneous regression
Mechanical complications (obstruction), arrhythmias (associated with pre-excitation: WPW)
Echo: round, well circumscribed, homogenous masses, brighter than the surrounding normal myocardium, with luminal extensions
Echo: tool of choice to monitor the haemodynamic significance of the mass and its evolution
Wage R, et al: Circulation 2008; 117: e469- e470 Costello J, et al: Circulation 2003; 107: 1066- 1067,
Rhabdomyomas
Yang HS, Circulation 2008 Nov 11; 118: e692- 6
2nd most common in pediatric age group (2/3 < 1st year of life)
Usual in Gorlin syndrome
Echo: solitary, homogeneous echogenic lesion, rounded – 1-10cm
Location: intramural, IVS or LV free wall (d.d. HCM, apical thrombus)
non-contractile mass
Calcification of the central portion= pathognomonic
potential for HF or arrhythmias (SCD) (surgical resection in symptomatic cases)
Echo monitoring for recurrence
Fibroma
only 2% to 3% of all benign primary cardiac tumors
•Generally small tumors (multiple: 1/3)
•Most often intra-myocardial in location- subendocardial nodules,
2-4cm, mainly in IVS
•May cause AV conduction disturbances & SCD due to predilection
for region of AV node
Echo (contrast), CT scanning, MRI, CAA (tumor “blush”)
Hemangiomas and Mesotheliomas
Roser M, et al: Circulation. 2008; 117: 2958-2960
Hemangioma
Ou P, et al: Circulation 2006; 113: e17- e18
Teratomas
• may arise within the pericardium
• serious consequences: either by causing tamponade or through direct pressure on the heart.
• high risk of death in-utero or immediately after birth.
•Treatment requires either fetal tumor excision, or caesarean section and immediate operation on the newborn
pulmonary obstruction by the sessile part of the tumorRV teratoma characterized by cysts separated by solid areas
Cardiac Calcified Amorphous Tumor - CAT
• Calcium deposits in a matrix of amorphous degenerating fibrinous material
• Symptoms due to obstruction or to the embolization of calcific fragments (retinal arterial embolism)
• d.d: Calcified myxomas, fibromas or tuberculoma, thrombi, emboli, vegetations, tophaceous pseudogout, tumoral calcinosis(end-stage chronic renal failure)
• Surgical excision if the lesion is symptomatic, large or mobile
Fujiwara M et al. Circulation 2012Nazli Y, et al: Tex Heart Inst J 2013; 40: 453- 8
mobile MAC-related CAT echocardiographic appearance of the revolving movement
Sarcomas – 95%
Malignant lymphomas – 5%
Primary malignant cardiac tumors
Sarcomas
• Most common primary cardiac malignancy: 95%
• Common in male (3:1)
• Characterized by rapidly downhill course leading to patient’s death weeks to months from time of presentation due to:1. Hemodynamic compromise2. Local invasion3. Distant metastases
• Characterized by rapid growth:At presentation, often spread extensively for surgical excision
Bruce C: Heart 2011; 97: 151- 160
Sarcomas
• Histologic types:
Angiosarcomas – most common: 30%
predilection for males, RA / adjacent structures
invasion, metastases (lung, brain, bone, colon)
Rhabdomyosarcoma- 20%, adult males,
children, adolescents/ single or multiple, rarely
beyond parietal pericardium
Fibrosarcoma -10%, adults, multiple, firm, RA
Osteosarcoma, Leiomyosarcoma (9%),
malignant fibrous histiocytoma e.t.c.
• Commonly involve RA & pericardium right-
sided failure, pericardial disease, vena cava
obstruction
• May occur in left side mistaken for myxoma
Echocardiographic appearance of a complex multilobulated intracavitary LV mass with haemorrhagic pleural and pericardial effusions:highly suspicious for malignancy
Li-Fraumeni syndrome
Patients <45 years with previous cardiac tumor disease= SBLA syndrome: sarcoma
breast / brainleukemiaadrenal gland
(in 9% of rhabdomyosarcomas)
400 individuals from 64 families reported in literature
Autosomal dominant conditionMutation of the PR53 gene on chromosome 17p13.1Frequency of de novo mutations at least 7% (- 20%)
Risk of cancer: 50% by 30 years (vs 1% in general population)
90% by 70 years
Schneider SRB et al. Z Herz-Thorax-Gefaβchir 2009; 23: 23-6
Hoffmeier A et al. Dtsch Arztebl Int 2014; 111(12): 205-11
Myxofibrosarcoma
Lazaros GA, et al: Angiology 2008; 59:632- 5
• multilobular LA mass
• Infiltration of posterior mitral annulus and
leaflet
• Surgical resection and MV replacement
• Despite chemotherapy, tumor recurrence and death 3months after surgery
Binder J, et al: Circulation 2004; 110: e451- e452
5% of primary cardiac malignancies
Rising incidence due to AIDS, transplanted population
RA primary cardiac lymphoma extending to SVC
Lymphomas
Metastatic cardiac tumors
• 20-40x more common than primary tumors
• Occurs in 1-20% of all tumor types
• Rare tumors with highest predilection for cardiac metastasis: Malignant melanoma (50-65%), malignant germ cell neoplasm, malignant thymoma, pleural mesothelioma
• Most cardiac metastases originate from lung (36-39%), breast (10-12%) and hematologic malignancies (10-21%) (also: renal, stomach, liver, ovary, colon)
• Echocardiography should be performed to rule out cardiac involvement in patients with malignancy and cardiac symptoms
Metastatic stomach adenocarcinoma
Goldberg AD et al. Circulation
2013; 128: 1790-1794
• Haematogenous, lymphatic, venous spread or direct invasion of the heart
• Location:1. Pericardium – most common (64- 69%)2. Epicardial involvement (25– 34%)3. Myocardium (29– 32%)4. Rarely, endocardium and cardiac valves (3-5%)
• Almost always occur in the setting of widespread primary disease
• May be the initial presentation of tumor elsewhere
• For right-sided tumors, TOE or ICE-guided transvenous biopsy can be helpful and safe
Metastatic cardiac tumors
In the presence of a floating mass extending through the IVC within the RC,
suspicion for intracardiac extension of pelvic leiomyoma/leiomyosarcoma,
D.D.renal cell tumor, hepatocellular carcinoma D.D. thrombus-in-transit
- evaluation of abdomen & pelvis is necessary
Gu X et al. JASE 2014
Intracardiac leiomyomatosis in 10% of intravenous leiomyomatosisUsually women in 5th decade with history of myomectomy, hysterectomy, uterine fibroid or endometriosis
variable echocardiographic appearances: solid tumor like, myxomalike, serpentine like and convoluted
Echo follow-up for recurrence
Pericardial tumors
Primary tumors: rare (0.001-0.007%)
(6.7-12.8% of all primary cardiac tumors)
- 2/3: benign (teratoma, fibroma, angioma, lipoma, mesothelial cyst, myxoma, lymphangioma)
- 1/3: or malignant (mesothelioma, sarcoma): dim prognosis (6- 15 months)
(male/female: 2/1, 3rd decade)
Secondary tumors: more common (100-1000x)
- metastasizing mostly from the lung, breast, melanomas, lymphoma, or leukaemia
incidence of malignant pericardial involvement :
0.15%- 21% of all patients with an underlying malignancy
85% of all patients with malignant cardiac involvement
Diagnosis: Echo (TTE/TEE), CT scanning, MRI,
open pericardial biopsy >90% of cases, pericardioscopy
Prognosis: poor - survival after diagnosis 6weeks to 15 months
Matsakas EP, et al: Clin Cardiol 2002;25: 83-5Suman S et al. Hear 2004
The most common benign pericardial ‘tumor’
Rare congenital, usually right-sided
Usually small, incidental finding – excellent prognosis
Rarely: tamponade
Pericardial cysts
....
Lagrotteria D, et al: Can J Cardiol 2005; 21: 185- 187, Suman S, et al: Heart 2004; 90: e4
Pericardial malignant mesothelioma:
most common primary malignant pericardial tumor
echo-bright, thickened layers of pericardium interposed by fibrin-like material in the pericardial space
Matkakas EP, et al: Clin Cardiol 2002;25: 83-5
pericardial fibrosarcoma:
large mass within the pericardial sac, attached by a broad base to the parietal pericardium and lying along the right ventricular free wall
In summary…
Primary cardiac tumors are rare (most often benign: myxomas, PFEs)
Metastatic cardiac tumors are 20-40x more common
Intracardiac masses are frequently detected during routine echocardiogram
The echocardiographer has to gather and properly interpret the full range of data
provided by cardiac ultrasound (plus 3D & contrast echocardiography) to
assess a cardiac tumor (d.d. normal variants, non-neoplastic mass) –always in
clinical context
Echocardiography evaluates haemodynamic consequences of cardiac tumors and
is a useful follow-up tool
Other imaging modalities such as CT, MRI, PET : further information (staging,
extracardiac extension)
The neoplastic nature & histotype of a cardiac mass can be established only by
histology
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