Cardiac Masses
Content
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
Myxomas:
Primary Cardiac Tumors
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
Fibromas:
Primary Cardiac Tumors
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?
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
Myxomas:
Primary Cardiac Tumors
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
Fibromas:
Primary Cardiac Tumors
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?
