Download cardiac masses - NT Cardiovascular Center

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 or pericardium
is more likely to be:
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
Anatomy
Crista
Eustatian V.
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
Will discuss further
5% multiple sites
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  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
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  Malignancy
Lateral wall of the LA  Malignancy
Atrial septum  Myxoma
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
NL Wall Motion
Leofler’s
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 primary cardiac neoplasms
not routinely considered for OHT due
to:
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?