CARDIAC MASSES

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CARDIAC MASSES

• Nick Tehrani, MD

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Outline

• General
• Primary Cardiac tumors
• Benign
• Malignant
• Secondary Cardiac Tumors
• Benign
• Malignant
• Q U I Z TIME

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General

• Neoplasia of the heart or pericardium is more
  likely to be
• Secondary, than
• Primary

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General

• Primary tumors
• lt3/10,000 autopsies
• Over ¾ are benign
• Secondary
• 20-40 x more common
• Present in upto 20 of patients dying of
  malignancy

Prevalence
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General

• Presentation
• Embolization
• Systemic
• Pulmonary (less common)
• Obstruction
• Arrhythmia
• Tamponade
• Direct 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 artifact
• Change depth and gain
• Hiatal hernia
• Fills the Rt. Or Lt. Atrium
• Drink Pepsi

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Normal Anatomic Variants

• Crista terminalis
• Normal anatomic ridge
• Eustatian valve
• Attaches to the anterior lip of the IVC
• Post transplant suture lines
• All kinds of funny masses
• Intraoperative inversion of the Lt atrial
  appendage

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Anatomy
Crista
Eustatian V.
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What is this?
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Outline

• General
• Primary Cardiac tumors
• Benign

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Primary Cardiac Tumors

• Benign
• Myxoma
• Papillary fibroelastoma
• Fibroma
• Lipoma
• Rhabdomyomas

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Benign Primary Cardiac Tumors

• The most common primary cardiac tumor in the Age
  gt 35
• 75 in the Lt. Atrium near fossa ovalis
• 15 Rt. Atrium
• 5-10 Lt. Ventricle
• 5 multiple sites

Myxomas
Will discuss further
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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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Benign Primary Cardiac Tumors
Myxomas

• Extra cardiac manifestations suggestive of
  Collagen Vascular dz
• Fever
• ESR elevation
• Anemia
• Thrombocythemia
• Circulating autoantibodies

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Benign Primary Cardiac Tumors

• Etiology of immunologic manifestations
• ? ? ?
• ? Tumor necrosis
• Anitimyocardial antibody titers decline post
  resection

Myxomas
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Benign Primary Cardiac Tumors
Management of Myxomas

• High propensity for embolization
• Surgical results excellent
• Decision to operate
• Lt. versus Rt. Sided tumor
• Sx.
• Age
• Co morbidities

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Benign Primary Cardiac Tumors

• Familial pattern of Myxomas with autosomal
  dominant features
• Age lt 30
• Bad actor

Any guesses?
Carney Complex
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Benign Primary 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 cell
  tumors)
• Multiple recurrent cardiac masses

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Benign Primary Cardiac Tumors

• Carney Complex
• Genes located on chromosomes
• 2p
• 17q2
• Family members should be screened when an index
  case is identified

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Primary Cardiac Tumors

• Benign
• Myxoma
• Papillary fibroelastoma

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Benign Primary Cardiac Tumors
Papillary Fibroelastoma (aka Papilloma)

• The most common valvular tumor, followed by
• Sarcoma
• Melanoma
• Present in all age groups, but most commonly in
  Agegt60

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Benign Primary Cardiac Tumors
Papillary Fibroelastoma

• Small tumors (lt1cm) with homogeneous speckeled
  pattern
• Commonly pedunculated with multiple fronds
• Affect the Lt and Rt sides with same frequency
• Attach to
• Atrial surface of AV valves, and
• Ventricular surface of semilunar valves

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Benign Primary Cardiac Tumors
Papillary Fibroelastoma

• Adults ? Aortic valve
• Children ? Tricuspid valve
• Rarely on endocardial surfaces
• Symptomatic only in the aortic position (other
  than embolic Sx)
• Ostial occlusion ? angina, sudden death
• Rarely valvular dysfunction
• Source of embolization in up to 30 of patients

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Benign Primary Cardiac Tumors
Management of Papillary Fibroelastoma

• Surgical resection is not uniformly necessary
• Anticoagulation may be sufficient in many
  patients
• Surgery indicated for
• Embolic events
• Ostial occlusion
• Topol Textbook of Cardiovascular Medicine
• All fibroelastomas, any size, any age are to be
  resected Seward, ACC 2000

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Primary Cardiac Tumors

• Benign
• Myxoma
• Papillary fibroelastoma
• Fibroma

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Benign Primary Cardiac Tumors
Fibromas

• Encapsulated, solitary tumors
• Frequently in the septal myocardium
• Often encroach on the conduction system as they
  grow

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Benign Primary Cardiac Tumors
Fibromas

• With septal involvement V.Fib is often the first
  presentation
• Indications for surgical resection
• Mechanical problems due to size
• Arrhythmogenic nidus
• Resection of septal fibromas is not always
  possible

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Benign Primary Cardiac Tumors

• Lipomas
• Affect both myocardium and pericardium
• Can reach several centimeters in size

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Benign Primary Cardiac Tumors

• Rhabdomyoma
• Most common tumor of the heart for Age lt 30 yo
• Almost exclusively in children
• Associated with tuberous sclerosis
• Regression of tumor in infancy has been reported

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Outline

• General
• Primary Cardiac tumors
• Benign
• Malignant

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Malignant Primary Cardiac Tumors

• Angiosarcomas
• Rhabdomyosarcomas
• Mesotheliomas
• Lymphoma
• Intrapericardial 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. Heart
• Rt. Atrium
• Pericardium
• Hemorrhagic effusion
• Thrombus

Angiosarcomas
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Malignant Primary Cardiac Tumors

• Diffuse, irregularly shaped
• Mean survival one year
• Successful Rx with Chemo and XRT followed by
  transplant reported

Angiosarcomas
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Malignant Primary Cardiac Tumors
Rhabdomyosarcomas

• Most commonly seen in adults
• No chamber selectivity
• No pericardial involvement
• Multiple sites of cardiac involvement is common
• Poor prognosis
• Limited success with resection and adjuvant Rx.

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Undifferentiated Sarcoma
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Malignant Primary Cardiac Tumors
Mesotheliomas

• Diffuse pericardial tumor
• Involve both parietal and visceral pericardium
• Superficially invade the myocardium
• Rarely invade the cardiac chambers

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Malignant Primary Cardiac Tumors
Mesotheliomas

• Sx of pericarditis or tamponade
• Poor prognosis
• XTR or chemo only offer temporary improvement

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Malignant Primary Cardiac Tumors

• Lymphoma
• Heart and pericardium are the only affected
  organs
• No predilection for any particular site

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Malignant Primary Cardiac Tumors
Intrapericardial Pheochromocytomas

• Soft, fleshy, highly vascular
• Anatomic location
• AV groove
• Atrium
• Interatrial septum
• Coronary, Pulmonary, Aorticopulmonary regions
• Generally very difficult to resect

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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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Malignant Secondary Cardiac Tumors

• Incidence of Solid Tumors involving the heart
• Lung
• Breast
• Soft tissue sarcomas
• Renal carcinoma
• Melanoma
• Leukemia and Lymphomas also common culprits

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What is this?
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Malignant Secondary Cardiac Tumors

• IVC tumors in General
• Renal carcinoma
• 80 5 year survival for surgical resection of
  tumors migrating up the IVC
• Hepatoma
• Ovarian CA

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Outline

• General
• Primary Cardiac tumors
• Benign
• Malignant
• Secondary Cardiac Tumors
• Malignant
• Benign

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Benign Secondary Cardiac Tumors

• Hints
• IVC tumor
• Long stringy appearance
• Seen many years post hysterectomy
• LEIOMYOMATOSIS

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Benign Secondary Cardiac Tumors
Leiomyomatosis

• Akin to Fibroids
• Controlled by hormone suppression
• Can degenerate into very low grade sarcoma
• Histologically, similar structures are present in
  the venous channels of the uterus

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HIV and cardiac tumors

• Kaposis sarcoma can involve the myocardium or
  pericardium
• Non-Hodgkin's lymphoma
• Can present as primary cardiac lymphoma
• When involving the heart usually diffusely
  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 in tropics)

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, lt3/10,000 autopsies
• Surgical options
• Benign ? majority resectable
• 6 rate of recurrence
• Malignant ?seldom resectable
• Surgery often intended for tissue diagnosis
  (benign vs. malignant)

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Surgical Options

• If surgical resection is contemplated, thorough
  metastatic evaluation is necessary
• CT
• Head
• Chest
• Abd
• Bone scan if indicated
• Bone marrow Bx if indicated

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Surgical Options

• Total of 28 patients have undergone orthotopic
  heart transplantation for inoperable primary
  cardiac tumors
• 7 had benign histology
• Mean survival of 46 months
• 21 had malignant histology
• Mean survival 12 months

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Surgical Options

• 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

• OF the 21 with malignant histology
• Sarcoma 15
• Malignant fibrohistiocytoma 3
• Lymphoma 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 patients with unresectable
  benign neoplasias

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Surgical Options

• Malignant primary cardiac neoplasms not routinely
  considered for OHT due to
• Concern for tumor recurrence
• Immunosuppression stimulation of tumor growth

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Surgical Options

• Patients with Primary malignant neoplasias need
  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?