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 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
Anatomy
Crista
Eustatian V.
What is this?
Outline
General Primary Cardiac tumors Benign
Benign
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
Myxomas:
Extra cardiac manifestations suggestive of Collagen Vascular dz : Fever ESR elevation Anemia Thrombocythemia Circulating autoantibodies
Benign
Myxomas:
Etiology of immunologic manifestations
Benign
Management of Myxomas: High propensity for embolization Surgical results excellent Decision to operate: Lt. versus Rt. Sided tumor Sx. Age Co morbidities
Benign
Familial pattern of Myxomas with autosomal dominant features Age < 30 Bad actor
Any guesses?
Carney Complex
Benign
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)
Benign
Carney Complex:
Genes located on chromosomes 2p 17q2 Family members should be screened when an index case is identified
Benign
Benign
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
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
All fibroelastomas, any size, any age are to be resected Seward, ACC 2000
Benign
Fibromas:
Encapsulated, solitary tumors Frequently in the septal myocardium Often encroach on the conduction system as they grow
Benign
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
Lipomas: Affect both myocardium and pericardium Can reach several centimeters in size
Benign
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
Pericardium
Hemorrhagic effusion Thrombus
Undifferentiated Sarcoma
Sx of pericarditis or tamponade Poor prognosis XTR or chemo only offer temporary improvement
Lymphoma: Heart and pericardium are the only affected organs No predilection for any particular site
Pulmonary vein mass Malignancy Lateral wall of the LA Malignancy Atrial septum Myxoma
Outline
General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant
What is this?
Outline
General Primary Cardiac tumors Benign Malignant Secondary Cardiac Tumors Malignant Benign
LEIOMYOMATOSIS
APICAL MASSES
Differential Dx for Apical Masses Thrombus NL Wall Motion Leoflers 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
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
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
Any Guesses??
The answer Is
What is this?
What is this?
Hiatal Hernia
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