CARDIAC MASSES

Nick Tehrani, MD

Outline

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

General

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

General

Prevalence: Primary tumors <3/10,000 autopsies Over ¾ are benign Secondary 20-40 x more common Present in upto 20% of patients dying of malignancy

General

Presentation:

Embolization Systemic Pulmonary (less common) Obstruction Arrhythmia Tamponade Direct compression of coronary artery Intramyocardial Conduction disturbances Global dysfunction due to infiltration

What is this?

Potential Pitfalls

Reverberation artifact Change depth and gain Hiatal hernia Fills the Rt. Or Lt. Atrium Drink Pepsi

…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

Eustatian V.

Anatomy

Crista

What is this?

Outline

General Primary Cardiac tumors Benign

Primary Cardiac Tumors

Benign

Myxoma Papillary fibroelastoma Fibroma Lipoma Rhabdomyomas

Benign

Primary Cardiac Tumors

Myxomas:

The most common primary cardiac tumor 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

Benign

Primary Cardiac Tumors

…Myxomas:

Grossly Typically pedunculated Gelatinous consistency Friable Histologically Copious mucopolysaccharide stroma Scattered solitary or clustered polygonal cells.

Left atrial Myxoma

Benign

Primary Cardiac Tumors

…Myxomas:

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

Benign

Primary Cardiac Tumors

…Myxomas:

Etiology of immunologic manifestations

? ? ?

?

Tumor necrosis Anitimyocardial antibody titers decline post resection

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

Benign

Primary Cardiac Tumors Familial pattern of Myxomas with autosomal dominant features Age < 30 Bad actor…

Any guesses?

Carney Complex

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 (Cushing’s syndrome, pituitary adenomas, testicular Sertoli cell tumors) Multiple recurrent cardiac masses

Benign

Primary Cardiac Tumors

…

Carney Complex:

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

Primary Cardiac Tumors

Benign

Myxoma Papillary fibroelastoma

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 Age>60

Benign

Primary Cardiac Tumors

…Papillary Fibroelastoma:

Small tumors (<1cm) 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

Benign

Primary Cardiac Tumors

…Papillary Fibroelastoma:

Adults  Aortic valve Children patients  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

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

Primary Cardiac Tumors

Benign

Myxoma Papillary fibroelastoma Fibroma

Benign

Primary Cardiac Tumors

Fibromas:

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

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

Benign

Primary Cardiac Tumors

Lipomas:

Affect both myocardium and pericardium Can reach several centimeters in size

Benign

Primary Cardiac Tumors

Rhabdomyoma:

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

Outline

General Primary Cardiac tumors Benign Malignant

Malignant

Primary Cardiac Tumors

Angiosarcomas Rhabdomyosarcomas Mesotheliomas Lymphoma Intrapericardial Pheochromocytomas

Malignant

Primary Cardiac Tumors

Angiosarcomas:

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

Malignant

Primary Cardiac Tumors

…Angiosarcomas:

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

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.

Undifferentiated Sarcoma

Malignant

Primary Cardiac Tumors

Mesotheliomas:

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

Malignant

Primary Cardiac Tumors

…Mesotheliomas:

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

Malignant

Primary Cardiac Tumors

Lymphoma:

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

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

Malignant

Primary Cardiac Tumors

Generalizations:

Pulmonary vein mass  Lateral wall of the LA  Atrial septum  Myxoma Malignancy Malignancy

Outline

General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant

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

What is this?

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

Outline

General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant Benign

Benign

Secondary Cardiac Tumors Hints: IVC tumor Long stringy appearance Seen many years post hysterectomy

LEIOMYOMATOSIS

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

HIV

and cardiac tumors

Kaposi’s 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

Another

Benign

Secondary CardiacTumor

How about this one?

APICAL MASSES

Differential Dx for Apical Masses Thrombus Leofler’s NL Wall Motion Endomyocardial fibrosis (seen in tropics)

Outline

General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant Benign Surgical Options

Surgical Options

Primary Cardiac Tumors Rare entity, <3/10,000 autopsies Surgical options Benign  majority resectable 6% rate of recurrence Malignant  seldom resectable Surgery often intended for tissue diagnosis (benign vs. malignant)

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

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

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

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

Surgical Options

OHT is promising for patients with unresectable benign neoplasias

Surgical Options

Malignant

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

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

Q U I Z TIME

Any Guesses??

The answer Is

Ruptured Papillary Muscle Abcess Inflamatory tissue

Any Guesses??

The answer Is

Non-specific Inflamatory Tissue

What is this?

PE in Transit across PFO

What is this?

Hiatal Hernia

What is this?

Mediastinal Lymph node

Myxoma with recent thrombosis

What do you

really

see here?