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Καρδιακοί όγκοι : Ο ρόλος του Υπερηχογραφήματος Cardiac Tumors: The role of echocardiography Σοφία Μ. Αράπη Επιμελήτρια Α’ Καρδιολογικής Κλινικής ΓΝΑ ‘Γ. Γεννηματάς’

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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 (

  • 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 (

  • 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 [

  • •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

  • 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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