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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