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CARDIACTUMORS

Sharon Sudarshan Brouha, MD, MPHAssistant Clinical Professor

Cardiothoracic Imaging SectionCardiothoracic Imaging SectionUniversity of California San Diego

Imaging techniquesImaging techniques

Cardiac and pericardial massesCardiac and pericardial masses

Cardiac tumor mimicsCardiac tumor mimics

OverviewOverview

Prevalence of 0.002-0.3% at autopsy

75% are benign

Cli i l i ifiClinical significance�Cardiac physiologyp y gy�Embolism�Arrhythmias

Radiology: Volume 268: Number 1�July 2013

Modality Advantages Disadvantages

Echocardiography Often the first study LimitedEchocardiography Often the first study� Suspected mass� Incidental findingNoninvasive

Limited � FOV� Tissue characterization� RV evaluation

AccessibleAnatomic and functional detail

� Large body habitus� Pulmonary diseaseOperator dependent

Cardiac CT Incidental cardiac massSpatial resolutionTissue characterization� Calcification

Radiation exposureLimited temporal resolution Limited contrast� Calcification

� Fat� Enhancement

Limited contrast resolution compared to MRECG gating

PET Metabolic activityEvaluation of interval change

Limited anatomic detail

Cardiac MR Tissue characterization Limited access

Radiology: Volume 268: Number 1�July 2013

No radiation exposure ECG gatingBreath-holding

Characteristics of cardiac ttumors

Anatomic considerations

� >5 cm

Tissue characteristics

� Heterogeneous signal� Irregular borders� Invasion� Right heart involvement

� Hemorrhagic pericardial effusion

� Contrast enhancementg� Pericardial or pleural

involvement� EffusionsEffusions� Nodules

� Multiplicity

Cardiac MR protocolCardiac MR protocol

St d rd Opti lStandard Optional

T1 FSE thorax

SSFP 2, 4 and SAX and two orthogonal planes through mass Myocardial tagging

T1 DIR in optimal plane

T1 DIR with fat suppression in optimal plane

T2 TIR in optimal plane

Early post Gd T1 DIR with TI 450-500 ms

Early post Gd T1 DIR (<2 min post Perfusiony p ( pGd)

LGE T1 DIR in optimal plane and SAX

Cardiac Tumors Sharon S. Brouha, MD

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Cardiac MR protocolCardiac MR protocol

T1 FSE thoraxT1 FSE thorax

Overview of the thoraxOverview of the thoraxIdentification of tumorBreath-hold or free breathing if necessaryg y

Cardiac MR protocolCardiac MR protocol

SSFP 2, 4 and SAXSSFP 2, 4 and SAX

Localization of massLocalization of massAnatomic detailMobility of massyFunctional significance

Cardiac MR protocolCardiac MR protocol

T1 weighted images pre and post contrastT1 weighted images pre and post contrast

Tissue characterizationTissue characterizationFat suppression for diagnosis of fatty elementsEarly Gd enhancementy� T1 of 450-500 ms post Gd renders thrombus low in signalLate Gd enhancement�Myocardial tumor infiltration with increased interstitial matrix or�Myocardial tumor infiltration with increased interstitial matrix or

scarring

Cardiac MR protocolCardiac MR protocol

T2 weighted imagesT2 weighted images

Tissue characterizationTissue characterizationEdema and liquefactive necrosis=high signalCoagulative necrosis=low signalg gHemorrhage or thrombus

Cardiac MR protocolCardiac MR protocol

Optional techniquesOptional techniques

TaggingTagging� Pericardial adherence/invasion� Pericardial inflammation

First pass perfusion� Tumor vascularity

Imaging techniquesImaging techniques

Cardiac and pericardial massesCardiac and pericardial masses

Cardiac tumor mimicsCardiac tumor mimics

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Tumor and tumor-like conditions b l tiby location

AtriaAtria�Angiosarcoma (right)�Lymphoma (right)�Myxoma (left)�Other sarcomas (left)�Other sarcomas (left)�Thrombus (left)

VentriclesVentricles�FibromaFibroma �Rhabdomyoma

ValvesValves�Papillary fibroelastoma�Vegetations

PericardiumPericardium�Pericardial cyst

t t�Metastases

Cardiac tumorsCardiac tumors

Relative incidence of primary cardiac tumors in adults and children

Benign Malignant

� Myxoma (30%)� Lipoma (10%)� Fibroelastoma (10%)

� Angiosarcoma (9%)� Rhabdomyosarcoma

(6%)Fibroelastoma (10%)� Rhabdomyoma� Fibroma

H i

( )� Mesothelioma (4%)� Fibrosarcoma (3%)� Lymphoma (2%)� Hemangioma

� Teratoma� Lymphoma (2%)� Other sarcoma� Teratoma

M t t ti di t th h t i 20 40Metastatic disease to the heart is 20-40 x more common

28FHx: Persistent progressive shortnessprogressive shortness of breath

Outside hospital CT pchest showed multiple pulmonary emboli

Returned to ED for SOB � Repeat CT chest

showed cardiac mass and thrombusmass and thrombus in right atrium and ventricle

UNDIFFERENTIATEDPatient died 1 month after tumor resection

UNDIFFERENTIATED PLEOMORPHIC SARCOMA

SarcomaSarcoma

Angiosarcoma is the most common malignant cardiac tumor in adultsAngiosarcoma is the most common malignant cardiac tumor in adults

Rhabdomyosarcoma is the most common malignant cardiac tumor in children

Rare but aggressive tumor with poor prognosis

Present between the 3rd and 5th decades of life

Clinical presentation

�Right heart failure�Hemorrhagic pericardial effusion�Metastatic disease

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Sarcoma: Imaging manifestationsSarcoma: Imaging manifestations

Location varies depending on histology�Angiosarcoma�right atriumg g�Rhadomyosarcoma�multiple�Undifferentiated sarcoma�left atrium

MR�Heterogeneous signal on T1 and T2 weighted�Heterogeneous signal on T1 and T2 weighted

images�Avid inhomogeneous enhancement

42MHx: HIV+, troponin bump and lactatebump and lactate elevation

CT abdomen demonstrated cardiac abnormality

CT chest and CMRCT chest and CMR subsequently ordered

DIR T1 TIR T2DIR T1 TIR T2

No significant enhancement post gad

LymphomaLymphoma

Much less common than extracardiac lymphomaMuch less common than extracardiac lymphoma with cardiac involvement

Typically immunocompromised patients such asTypically immunocompromised patients, such as HIV+

Clinical

� Failure� Failure� Obstructive symptoms� Tamponade

Arrh thmias� Arrhythmias

Lymphoma: Imaging manifestationsLymphoma: Imaging manifestations

Predilection for the right heartPredilection for the right heart

Multiple lesionsMultiple lesions

Nodular infiltration of myocardium

MR�Homogeneous signal� T1 and T2 isointense� Little to no enhancement on LGE images� Little to no enhancement on LGE images

70FHx: Chronic

i di l ff ipericardial effusion followed with ECHO

Presented to the ED ith bd i lED with abdominal pain

CT A/P revealed ovarian mass and

dicardiac mass

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T1 DIR T1 DIR post gad IR post gad

LEFT ATRIAL MYXOMAPericadial biopsy was normal

LEFT ATRIAL MYXOMA

MyxomaMyxoma

Occur at interatrial septum near fossa ovalisOccur at interatrial septum near fossa ovalis

Smooth, lobulated margins

Pedunculated

Symptoms� Mass effect and obstruction� Embolization� Embolization� Constitutional symptoms due to IL-6� Carney complex: skin lentigines, endocrine tumors,

fibroadenomas and melanotic schwannomasfibroadenomas and melanotic schwannomas

Myxoma: Imaging manifestationsMyxoma: Imaging manifestations

LocationLocation� 75% left atrium� 20% right atrium� 5% right or left ventricle

MR� T1 isointense� T2 hyperintense� Additional featuresAdditional features� Internal hemorrhage, cysts, necrosis, calcification� Mobile on cine images� Surface thrombus is low signal on LGE imagesSurface thrombus is low signal on LGE images

17MHx: Presented in 6/2012 for routine6/2012 for routine sports physical� Arrhythmia with

f t PVCfrequent PVCs

MR: mass in theMR: mass in the superolateral wall of left ventricle

Referred for surgical resection

DIR T1 FS TIR T2

DIR T2 IR post gad

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Subtotal resection of tumor with residual tumor at left main

FIBROMA

Subtotal resection of tumor with residual tumor at left main bifurcation

FIBROMA

FibromaFibroma

2nd most common pediatric cardiac tumor

S lit tSolitary tumors

Clinical presentationClinical presentation� Syncope�ArrhythmiaArrhythmia�Chest pain�CHF

Fibroma: Imaging manifestationsFibroma: Imaging manifestations

I t l i th t i lIntramural in the ventricles

MR� T1 isointenseT1 isointense� T2 hypointense�Hyperenhancement on LGE images�Hyperenhancement on LGE images

Homogeneous +/- calcificationg

RhabdomyomaRhabdomyoma

Most common pediatric cardiac tumorMost common pediatric cardiac tumor

Multiple in 90%

Present in the first year of life

Tuberous sclerosis in 50%

T i ll t tiTypically asymptomatic

Undergo regression by age 4g g y g

Rhabdomyoma: Imaging manifestationsRhabdomyoma: Imaging manifestations

I t l i th t i l b tIntramural in the ventricles but multiple

MRMR

� T1 isointenseT1 isointense� T2 hyperintense� Little to no enhancementLittle to no enhancement

82FHx: Pulmonary

tartery mass

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Papillary fibroelastoma: Imaging if t timanifestations

T i ll < 1 5Typically < 1.5 cm

Cli i l iClinical presentation�Often asymptomaticOften asymptomatic�Embolization of surface thrombus/tumor�No valvular abnormality�No valvular abnormality�Surgery if symptomatic or >1cm and

left-sidedleft sided

Papillary fibroelastoma: Imaging if t timanifestations

Pedicle adherent to downstream side of valvePedicle adherent to downstream side of valve unlike vegetation

Mobile

MR

� Isointense T1� Hyperintense T2� No enhancement� No enhancement

Pericardial tumorsPericardial tumors

Relative incidence of primary pericardial tumors

Benign

�Pericardial cyst�Pericardial lipoma

Malignant

�Mesothelioma�HemangiomaPericardial lipoma Hemangioma�Sarcoma�Lymphoma�Lipoblastomap�Paraganglioma�Germ cell tumors�Fibroma� Inflammatory

pseudotumor/IgG4-related disease

�PNET�PNET

Primary pericardial tumors much less common than primary cardiac tumorstumors

Direct invasion by neoplasm or metastatic disease is 100-1000x more common than primary pericardial tumor

41MHx: Melanoma

Referred for CT thoraxthorax

PERICARDIAL METASTASISPERICARDIAL METASTASIS

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Cardiac metastasesCardiac metastases

20 40x more common than primary cardiac tumors20-40x more common than primary cardiac tumors

10-12% of patients with known malignancy have cardiac metastases at autopsyautopsy

Pericardial involvement is most common (~95%)

Modes of spread (*most common)

�Hematogenous�lymphoma*, melanoma*, leukemia�Direct invasion�lung*, breast*, esophagus�Transvenous�RCC, HCC

Metastatic melanoma high T1/low T2

39F Hx: epigastric pain

Referred for CT abdomenabdomen

T1 DIR SSFP (T2/T1) DIR T1 post gad

PERICARDIAL CYSTPERICARDIAL CYST

Images courtesy of Dr. Eric Goodman

LipomaLipoma

Well-marginated and encapsulated

Most arise from the epicardial surface and extend into pericardial spaceand extend into pericardial space�Subendocardial less common

Typically incidental and asymptomatic

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Lipoma: Imaging manifestationsLipoma: Imaging manifestations

CT f t tt tiCT: fat attenuation

MR�T1 hyperintense�T1 hyperintense�Low signal on fat suppressed

sequencessequences

No enhancement

62FHx: bilateral

tid bif ticarotid bifurcation paragangliomas(carotid body tumors)

PET dPET scan and MIBG scan demonstrate mediastinalabnormality

62FHx: bilateral

tid bif ticarotid bifurcation paragangliomas(carotid body tumors)

PET dPET scan and MIBG scan demostratemediastinalabnormality

PARAGANGLIOMABilateral carotid body tumors

PARAGANGLIOMA

Paraganglioma: Imaging manifestationsParaganglioma: Imaging manifestations

R tRare tumor

Typically nonfunctioning

Clinical presentationF ti i l t d�Functioning: elevated catecholamines, tremors, sweating, palpitations hypertensionpalpitations, hypertension

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Paraganglioma: Imaging manifestationsParaganglioma: Imaging manifestations

Adjacent to left atrium or anterior to aorticAdjacent to left atrium or anterior to aortic root

CT: hypervascular mass

� Feeding vessel may originate from coronary artery

MR: T2 hyperintense

� Enhancement post GdEnhancement post Gd

65M Hx: epicardialmass

T1 DIR T1 TIR

Post gad T1 3D FSPGR LGEPost gad T1 3D FSPGR LGE

Epicardial mass with extrinsic compression

HEMANGIOMA

Epicardial mass with extrinsic compressionNo invasion of cardiac structures

HEMANGIOMA

HemangiomaHemangioma

Usually solitaryUsually solitary

Predilection for the ventricles

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Hemangioma: Imaging manifestationsHemangioma: Imaging manifestations

H tHeterogeneous

MR�T1 hyperintense�T1 hyperintense�T2 hyperintense

Avid enhancement although�Avid enhancement although calcification or fibrous septations may be presentbe present

Cardiac tumor mimics

Normal structures Pseudotumors

� Coumadin ridge� Between the LAA and

the left superior l i ( ti

� Thrombus� Caseous calcification

of the mitral annuluspulmonary vein (Q tip sign)

� Eustachian valveC i t t i li

� Lipomatous hypertrophy� > 2cm

� Crista terminalis � Nonencapsulated� Spares the fossa

ovalis giving it a d bb ll hdumbbell shape

� Vegetation� Typically on low

id f lpressure side of valve

71FHx: DOE

ECHO: 3 x 2 cm right atrial mass attached to interatrial septum

Referred for CCTA

LIPOMATOUS HYPERTROPHY OFLIPOMATOUS HYPERTROPHY OF THE INTERATRIAL SEPTUM

� Muscular extending

Crista terminalis

from SVC to IVC within the right atrium

� Represents the junction of the anterior trabeculatedand posterior smooth

ll d ( iwalled (sinus venosus) portions of the primitive right atriumatrium

� May be seen on chest CT�not a tumor or thrombustumor or thrombus

RadioGraphics 2005; 25:441�453

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84M metastatic renal cell carcinoma

� Right atrial thrombus

84M metastatic renal cell carcinoma

ReferencesReferences

� Radiology 2013 Jul;268(1):26-43Radiology. 2013 Jul;268(1):26 43� Radiographics. 2013 Oct;33(6):1613-30.

R di hi 2000 J l� Radiographics. 2000 Jul-Aug;20(4):1073-103.