Cardiac Masses

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    CARDIAC MASSES

    Nick Tehrani, MD

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    Outline

    General

    Primary Cardiac tumorsBenign

    Malignant

    Secondary Cardiac TumorsBenign

    Malignant

    Q U I Z TIME

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    General

    Neoplasia of the heartor pericardiumis more likely to be:

    Secondary, than

    Primary

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    General

    Primary tumors

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    GeneralPresentation:

    Embolization

    SystemicPulmonary (less common)

    Obstruction

    Arrhythmia

    TamponadeDirect compression of coronary artery

    Intramyocardial

    Conduction disturbances

    Global dysfunction due to infiltration

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    What is this?

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    Potential Pitfalls

    Reverberation artifactChange depthand gain

    Hiatal hernia

    Fills the Rt. Or Lt. AtriumDrink Pepsi

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    Normal Anatomic Variants

    Crista terminalis

    Normal anatomic ridgeEustatian valve

    Attaches to the anterior lip of the IVC

    Post transplant suture lines

    All kinds of funny masses

    Intraoperative inversion of the Lt atrialappendage

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    Anatomy

    Crista

    Eustatian V.

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    What is this?

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    Outline

    General

    Primary Cardiac tumorsBenign

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    Primary Cardiac Tumors

    BenignMyxoma

    Papillary fibroelastoma

    Fibroma

    Lipoma

    Rhabdomyomas

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    BenignPrimary Cardiac Tumors

    The most common primary cardiactumor in the Age > 35

    75% in the Lt. Atrium near fossa ovalis

    15% Rt. Atrium

    5-10% Lt. Ventricle

    5% multiple sites Will discuss further

    Myxomas:

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    Benign Primary Cardiac Tumors

    Grossly

    Typically pedunculated

    Gelatinous consistency

    Friable

    Histologically

    Copious mucopolysaccharide stroma

    Scattered solitary or clustered polygonal

    cells.

    Myxomas:

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    Left atrial Myxoma

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    BenignPrimary Cardiac Tumors

    Extra cardiac manifestations suggestive ofCollagen Vascular dz:

    Fever

    ESR elevation

    Anemia

    Thrombocythemia

    Circulating autoantibodies

    Myxomas:

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    BenignPrimary Cardiac Tumors

    Etiology of immunologic manifestations

    ? ? ?

    ? Tumor necrosisAnitimyocardial antibody titers declinepost resection

    Myxomas:

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    BenignPrimary Cardiac Tumors

    High propensity for embolization

    Surgical results excellent

    Decision to operate:

    Lt. versus Rt. Sided tumor

    Sx.

    Age

    Co morbidities

    Managementof Myxomas:

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    BenignPrimary Cardiac Tumors

    Familial pattern of Myxomas with

    autosomal dominant featuresAge < 30

    Bad actor

    Any guesses?

    Carney Complex

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    BenignPrimary Cardiac Tumors

    Carney Complex:

    Multiple lentigines and blue nevi

    Peripheral myxoid tumors (cutaneous myxoma,myxoid mamary fibroadenoma)

    Psamommatous melanotic schwannoma

    Endocrine over activity (Cushings syndrome,pituitary adenomas, testicular Sertoli celltumors)

    Multiple recurrent cardiac masses

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    BenignPrimary Cardiac Tumors

    Carney Complex:

    Genes located on chromosomes

    2p

    17q2

    Family members should be screenedwhen an index case is identified

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    Primary Cardiac Tumors

    Benign

    Myxoma

    Papillary fibroelastoma

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    BenignPrimary Cardiac Tumors

    The most common valvular tumor,followed by

    Sarcoma

    MelanomaPresent in all age groups, but mostcommonly in Age>60

    Papillary Fibroelastoma:(aka Papilloma)

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    BenignPrimary Cardiac Tumors

    Small tumors (

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    BenignPrimary Cardiac Tumors

    Adults Aortic valve

    Children Tricuspid valveRarely on endocardial surfaces

    Symptomatic only in the aortic position (otherthan embolic Sx)

    Ostial occlusion angina, sudden death

    Rarely valvular dysfunction

    Source of embolization in up to 30% of

    patients

    Papillary Fibroelastoma:

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    BenignPrimary Cardiac Tumors

    Surgical resection is not uniformly necessary

    Anticoagulation may be sufficient in manypatients

    Surgery indicated for

    Embolic eventsOstial occlusionTopol Textbook of Cardiovascular Medicine

    All fibroelastomas, any size, any age are to be

    resected Seward, ACC 2000

    Management ofPapillary Fibroelastoma

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    Primary Cardiac Tumors

    Benign

    Myxoma

    Papillary fibroelastoma

    Fibroma

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    BenignPrimary Cardiac Tumors

    Encapsulated, solitary tumors

    Frequently in the septal myocardium

    Often encroach on the conductionsystem as they grow

    Fibromas:

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    BenignPrimary Cardiac Tumors

    With septal involvement V.Fib is oftenthe first presentation

    Indications for surgical resection:

    Mechanical problems due to sizeArrhythmogenic nidus

    Resection of septal fibromas is notalways possible

    Fibromas:

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    BenignPrimary Cardiac Tumors

    Lipomas:

    Affect both myocardium andpericardium

    Can reach several centimeters in size

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    BenignPrimary Cardiac Tumors

    Rhabdomyoma:

    Most common tumor of the heart forAge < 30 yo

    Almost exclusively in children

    Associated with tuberous sclerosis

    Regression of tumor in infancy hasbeen reported

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    Outline

    GeneralPrimary Cardiac tumors

    Benign

    Malignant

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    Malignant Primary Cardiac Tumors

    AngiosarcomasRhabdomyosarcomas

    Mesotheliomas

    LymphomaIntrapericardial Pheochromocytomas

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    Malignant Primary Cardiac Tumors

    Most common primary malignancy of the heart

    Malignant cells that form vascular channels

    Most commonly affect the

    Rt. HeartRt. Atrium

    PericardiumHemorrhagic effusion

    Thrombus

    Angiosarcomas:

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    Malignant Primary Cardiac Tumors

    Diffuse, irregularly shaped

    Mean survival one year

    Successful Rx with Chemo and XRTfollowed by transplant reported

    Angiosarcomas:

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    Malignant Primary Cardiac Tumors

    Most commonly seen in adults

    No chamber selectivity

    No pericardial involvement

    Multiple sites of cardiac involvement is

    commonPoor prognosis

    Limited success with resection andadjuvant Rx.

    Rhabdomyosarcomas:

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    Undifferentiated Sarcoma

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    Malignant Primary Cardiac Tumors

    Diffuse pericardial tumor

    Involve both parietal and visceralpericardium

    Superficially invade the myocardium

    Rarely invade the cardiac chambers

    Mesotheliomas:

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    Malignant Primary Cardiac Tumors

    Sx of pericarditis or tamponade

    Poor prognosis

    XTR or chemo only offer temporaryimprovement

    Mesotheliomas:

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    Malignant Primary Cardiac Tumors

    Lymphoma:Heart and pericardium are the onlyaffected organs

    No predilection for any particular site

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    Malignant Primary Cardiac Tumors

    Soft, fleshy, highly vascular

    Anatomic location

    AV groove

    Atrium

    Interatrial septumCoronary, Pulmonary, Aorticopulmonaryregions

    Generally very difficult to resect

    Intrapericardial Pheochromocytomas:

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    Malignant Primary Cardiac Tumors

    Generalizations:

    Pulmonary vein mass Malignancy

    Lateral wall of the LA Malignancy

    Atrial septum Myxoma

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    Outline

    General

    Primary Cardiac tumors

    Benign

    Malignant

    Secondary Cardiac Tumors

    Malignant

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    MalignantSecondary Cardiac Tumors

    Incidenceof Solid Tumors involving the heart:

    LungBreast

    Soft tissue sarcomas

    Renal carcinomaMelanoma

    Leukemia and Lymphomas also common culprits

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    What is this?

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    MalignantSecondary Cardiac Tumors

    IVC tumors in General

    Renal carcinoma

    80% 5 year survival for surgicalresection of tumors migrating up

    the IVCHepatoma

    Ovarian CA

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    Outline

    General

    Primary Cardiac tumorsBenign

    Malignant

    Secondary Cardiac TumorsMalignant

    Benign

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    BenignSecondary Cardiac Tumors

    Hints:

    IVC tumor

    Long stringy appearance

    Seen many years post hysterectomy

    LEIOMYOMATOSIS

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    BenignSecondary Cardiac Tumors

    Akin to FibroidsControlled by hormone suppression

    Can degenerate into very low gradesarcoma

    Histologically, similar structures arepresent in the venous channels of the

    uterus

    Leiomyomatosis:

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    HIVand cardiac tumorsKaposis sarcoma can involve themyocardium or pericardium

    Non-Hodgkin's lymphoma

    Can present as primary cardiaclymphoma

    When involving the heart usuallydiffusely infiltrative

    Variable results with chemo and XRT

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    Another Benign Secondary

    CardiacTumor

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    How about this one?

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    APICAL MASSES

    Differential Dx for Apical Masses

    Thrombus

    Leoflers

    Endomyocardial fibrosis (seen intropics)

    NL Wall Motion

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    Outline

    General

    Primary Cardiac tumors

    Benign

    Malignant

    Secondary Cardiac Tumors

    Malignant

    Benign

    Surgical Options

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    Surgical Options

    Primary Cardiac Tumors

    Rare entity,

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    Surgical Options

    If surgical resection is contemplated,thorough metastatic evaluation is necessary:

    CT

    Head

    Chest

    AbdBone scan if indicated

    Bone marrow Bx if indicated

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    Surgical Options

    Total of 28 patients have undergoneorthotopic heart transplantation for

    inoperable primary cardiac tumors:

    7 had benign histology

    Mean survival of 46 months21 had malignant histology

    Mean survival 12 months

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    SurgicalOptions

    OF the 7 with benign histology

    Fibroma 5 patients

    Rhabdomyoma 1

    Pheochromocytoma 1

    Survival range: 8 to 105 months

    Survival mean: 46 months

    No patients had recurrence of tumor

    Two deaths due to rejection

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    Surgical Options

    OFthe 21 with malignant histologySarcoma 15

    Malignant fibrohistiocytoma 3Lymphoma 3

    14/21 died between 1 and 36 months(mean 12 months)

    13/14 died of recurrent mets

    1/14 cause of death unknown

    7 others; follow up 6 66 months

    one had recurrence malignancy

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    Surgical Options

    OHT is promising for patientswith unresectable benign

    neoplasias

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    Surgical Options

    Malignant primary cardiac neoplasms

    not routinely considered for OHT dueto:

    Concern for tumor recurrence

    Immunosuppression stimulation oftumor growth

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    SurgicalOptions

    Patients with Primary malignant neoplasiasneed to be assessed on a case by case basis

    due to:Limited experience

    Heterogeneous nature of the malignancies

    Yet to be defined, role of:Adjuvant chemo

    10/21 had received chemo

    XRT

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    Q U I Z TIME

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    Any Guesses??

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    The answer Is

    Ruptured Papillary Muscle Abcess

    Inflamatory tissue

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    Any Guesses??

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    The answer Is

    Non-specific Inflamatory Tissue

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    What is this?

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    PE in Transit across PFO

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    What is this?

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    Hiatal Hernia

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    What is this?

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    Mediastinal Lymph node

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    Myxoma with recent thrombosis

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    What do you really see here?