Post on 14-Apr-2018
7/30/2019 Cardiac Masses
1/77
CARDIAC MASSES
Nick Tehrani, MD
7/30/2019 Cardiac Masses
2/77
Outline
General
Primary Cardiac tumorsBenign
Malignant
Secondary Cardiac TumorsBenign
Malignant
Q U I Z TIME
7/30/2019 Cardiac Masses
3/77
General
Neoplasia of the heartor pericardiumis more likely to be:
Secondary, than
Primary
7/30/2019 Cardiac Masses
4/77
General
Primary tumors
7/30/2019 Cardiac Masses
5/77
GeneralPresentation:
Embolization
SystemicPulmonary (less common)
Obstruction
Arrhythmia
TamponadeDirect compression of coronary artery
Intramyocardial
Conduction disturbances
Global dysfunction due to infiltration
7/30/2019 Cardiac Masses
6/77
What is this?
7/30/2019 Cardiac Masses
7/77
Potential Pitfalls
Reverberation artifactChange depthand gain
Hiatal hernia
Fills the Rt. Or Lt. AtriumDrink Pepsi
7/30/2019 Cardiac Masses
8/77
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
7/30/2019 Cardiac Masses
9/77
Anatomy
Crista
Eustatian V.
7/30/2019 Cardiac Masses
10/77
What is this?
7/30/2019 Cardiac Masses
11/77
Outline
General
Primary Cardiac tumorsBenign
7/30/2019 Cardiac Masses
12/77
Primary Cardiac Tumors
BenignMyxoma
Papillary fibroelastoma
Fibroma
Lipoma
Rhabdomyomas
7/30/2019 Cardiac Masses
13/77
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:
7/30/2019 Cardiac Masses
14/77
Benign Primary Cardiac Tumors
Grossly
Typically pedunculated
Gelatinous consistency
Friable
Histologically
Copious mucopolysaccharide stroma
Scattered solitary or clustered polygonal
cells.
Myxomas:
7/30/2019 Cardiac Masses
15/77
Left atrial Myxoma
7/30/2019 Cardiac Masses
16/77
BenignPrimary Cardiac Tumors
Extra cardiac manifestations suggestive ofCollagen Vascular dz:
Fever
ESR elevation
Anemia
Thrombocythemia
Circulating autoantibodies
Myxomas:
7/30/2019 Cardiac Masses
17/77
BenignPrimary Cardiac Tumors
Etiology of immunologic manifestations
? ? ?
? Tumor necrosisAnitimyocardial antibody titers declinepost resection
Myxomas:
7/30/2019 Cardiac Masses
18/77
BenignPrimary Cardiac Tumors
High propensity for embolization
Surgical results excellent
Decision to operate:
Lt. versus Rt. Sided tumor
Sx.
Age
Co morbidities
Managementof Myxomas:
7/30/2019 Cardiac Masses
19/77
BenignPrimary Cardiac Tumors
Familial pattern of Myxomas with
autosomal dominant featuresAge < 30
Bad actor
Any guesses?
Carney Complex
7/30/2019 Cardiac Masses
20/77
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
7/30/2019 Cardiac Masses
21/77
BenignPrimary Cardiac Tumors
Carney Complex:
Genes located on chromosomes
2p
17q2
Family members should be screenedwhen an index case is identified
7/30/2019 Cardiac Masses
22/77
Primary Cardiac Tumors
Benign
Myxoma
Papillary fibroelastoma
7/30/2019 Cardiac Masses
23/77
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)
7/30/2019 Cardiac Masses
24/77
BenignPrimary Cardiac Tumors
Small tumors (
7/30/2019 Cardiac Masses
25/77
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:
7/30/2019 Cardiac Masses
26/77
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
7/30/2019 Cardiac Masses
27/77
Primary Cardiac Tumors
Benign
Myxoma
Papillary fibroelastoma
Fibroma
7/30/2019 Cardiac Masses
28/77
BenignPrimary Cardiac Tumors
Encapsulated, solitary tumors
Frequently in the septal myocardium
Often encroach on the conductionsystem as they grow
Fibromas:
7/30/2019 Cardiac Masses
29/77
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:
7/30/2019 Cardiac Masses
30/77
BenignPrimary Cardiac Tumors
Lipomas:
Affect both myocardium andpericardium
Can reach several centimeters in size
7/30/2019 Cardiac Masses
31/77
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
7/30/2019 Cardiac Masses
32/77
Outline
GeneralPrimary Cardiac tumors
Benign
Malignant
7/30/2019 Cardiac Masses
33/77
Malignant Primary Cardiac Tumors
AngiosarcomasRhabdomyosarcomas
Mesotheliomas
LymphomaIntrapericardial Pheochromocytomas
7/30/2019 Cardiac Masses
34/77
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:
7/30/2019 Cardiac Masses
35/77
Malignant Primary Cardiac Tumors
Diffuse, irregularly shaped
Mean survival one year
Successful Rx with Chemo and XRTfollowed by transplant reported
Angiosarcomas:
7/30/2019 Cardiac Masses
36/77
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:
7/30/2019 Cardiac Masses
37/77
Undifferentiated Sarcoma
7/30/2019 Cardiac Masses
38/77
Malignant Primary Cardiac Tumors
Diffuse pericardial tumor
Involve both parietal and visceralpericardium
Superficially invade the myocardium
Rarely invade the cardiac chambers
Mesotheliomas:
7/30/2019 Cardiac Masses
39/77
Malignant Primary Cardiac Tumors
Sx of pericarditis or tamponade
Poor prognosis
XTR or chemo only offer temporaryimprovement
Mesotheliomas:
7/30/2019 Cardiac Masses
40/77
Malignant Primary Cardiac Tumors
Lymphoma:Heart and pericardium are the onlyaffected organs
No predilection for any particular site
7/30/2019 Cardiac Masses
41/77
Malignant Primary Cardiac Tumors
Soft, fleshy, highly vascular
Anatomic location
AV groove
Atrium
Interatrial septumCoronary, Pulmonary, Aorticopulmonaryregions
Generally very difficult to resect
Intrapericardial Pheochromocytomas:
7/30/2019 Cardiac Masses
42/77
Malignant Primary Cardiac Tumors
Generalizations:
Pulmonary vein mass Malignancy
Lateral wall of the LA Malignancy
Atrial septum Myxoma
7/30/2019 Cardiac Masses
43/77
Outline
General
Primary Cardiac tumors
Benign
Malignant
Secondary Cardiac Tumors
Malignant
7/30/2019 Cardiac Masses
44/77
MalignantSecondary Cardiac Tumors
Incidenceof Solid Tumors involving the heart:
LungBreast
Soft tissue sarcomas
Renal carcinomaMelanoma
Leukemia and Lymphomas also common culprits
7/30/2019 Cardiac Masses
45/77
7/30/2019 Cardiac Masses
46/77
What is this?
7/30/2019 Cardiac Masses
47/77
MalignantSecondary Cardiac Tumors
IVC tumors in General
Renal carcinoma
80% 5 year survival for surgicalresection of tumors migrating up
the IVCHepatoma
Ovarian CA
7/30/2019 Cardiac Masses
48/77
Outline
General
Primary Cardiac tumorsBenign
Malignant
Secondary Cardiac TumorsMalignant
Benign
7/30/2019 Cardiac Masses
49/77
BenignSecondary Cardiac Tumors
Hints:
IVC tumor
Long stringy appearance
Seen many years post hysterectomy
LEIOMYOMATOSIS
7/30/2019 Cardiac Masses
50/77
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:
7/30/2019 Cardiac Masses
51/77
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
7/30/2019 Cardiac Masses
52/77
Another Benign Secondary
CardiacTumor
7/30/2019 Cardiac Masses
53/77
How about this one?
7/30/2019 Cardiac Masses
54/77
APICAL MASSES
Differential Dx for Apical Masses
Thrombus
Leoflers
Endomyocardial fibrosis (seen intropics)
NL Wall Motion
7/30/2019 Cardiac Masses
55/77
Outline
General
Primary Cardiac tumors
Benign
Malignant
Secondary Cardiac Tumors
Malignant
Benign
Surgical Options
7/30/2019 Cardiac Masses
56/77
Surgical Options
Primary Cardiac Tumors
Rare entity,
7/30/2019 Cardiac Masses
57/77
Surgical Options
If surgical resection is contemplated,thorough metastatic evaluation is necessary:
CT
Head
Chest
AbdBone scan if indicated
Bone marrow Bx if indicated
7/30/2019 Cardiac Masses
58/77
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
7/30/2019 Cardiac Masses
59/77
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
7/30/2019 Cardiac Masses
60/77
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
7/30/2019 Cardiac Masses
61/77
Surgical Options
OHT is promising for patientswith unresectable benign
neoplasias
7/30/2019 Cardiac Masses
62/77
Surgical Options
Malignant primary cardiac neoplasms
not routinely considered for OHT dueto:
Concern for tumor recurrence
Immunosuppression stimulation oftumor growth
7/30/2019 Cardiac Masses
63/77
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
7/30/2019 Cardiac Masses
64/77
Q U I Z TIME
7/30/2019 Cardiac Masses
65/77
Any Guesses??
7/30/2019 Cardiac Masses
66/77
The answer Is
Ruptured Papillary Muscle Abcess
Inflamatory tissue
7/30/2019 Cardiac Masses
67/77
Any Guesses??
7/30/2019 Cardiac Masses
68/77
The answer Is
Non-specific Inflamatory Tissue
7/30/2019 Cardiac Masses
69/77
What is this?
7/30/2019 Cardiac Masses
70/77
PE in Transit across PFO
7/30/2019 Cardiac Masses
71/77
What is this?
7/30/2019 Cardiac Masses
72/77
Hiatal Hernia
7/30/2019 Cardiac Masses
73/77
What is this?
7/30/2019 Cardiac Masses
74/77
Mediastinal Lymph node
7/30/2019 Cardiac Masses
75/77
7/30/2019 Cardiac Masses
76/77
Myxoma with recent thrombosis
7/30/2019 Cardiac Masses
77/77
What do you really see here?