Cardiac Tumors • Rare – autopsy <0.05% Cardiac Masses Heidi M. Connolly, MD Advanced Echocardiography Dunblane, Scotland No disclosures •  antemortem diagnosis – imaging advances • Echo – often initial imaging noninvasive, available • CT and MRI – complimentary data re tumor extension impact management Cardiac Tumors and Masses Differential Echo Features of Masses Clinical presentation • Size • CV – embolism, arrhythmia, heart failure • Location • Systemic – fever, fatigue, chest pain, weight loss • Mobility • Obstruction – chamber, valve or venous • Attachment site • Cardiac compression • Tissue characterization • Incidental • Clinical presentation and ancillary nonEcho data Primary Cardiac Tumors Benign (94%) Myxoma Lipoma Fibroelastoma Rhabdomyoma Malignant (6%) Sarcoma Benign Cardiac Tumors Mesothelioma Lymphoma Fibroma 1 Benign Cardiac Neoplasm n=319 4% 5% 4% 3% 5% 40% 11% 14% 14% Tuberous Sclerosis • Diagnostic Triad Seizures Mental retardation Facial angiofibromas Myxoma Lipoma Papillary fibro Rhabdo Fibroma Hemangio Teratoma Mesothel Misc • Autosomal Dominant • Rhabdomyoma McAllister: Atlas of Tumor Pathology, 1978 Common Benign Cardiac Tumors Rhabdomyoma Common Benign Cardiac Tumors • Most common cardiac tumor in children • LV free wall, septum or apex • Associated with tuberous sclerosis • Well demarcated • Often multiple • Grows into cavity • Regress spontaneously Fibroma • Intramyocardial May interfere with filling • Often multiple • Heart failure, arrhythmias Cardiac Myxoma • Most common 1º cardiac tumor • Most common location is LA Myxoma • Usually attached to atrial septum • Emboli, obstruction, arrhythmias, systemic ymptoms • Management – surgical Papillary Smooth • Atypical location, multiple (5%) Myxoma syndrome Courtesy of William Edwards, MD 2 41-Year-Old Female with Recurrent Spells 2 Prior Negative TEEs Complex Myxoma, Syndrome Myxoma Carney Complex • Myxoma – often multiple Skin, mucosal or cardiac • Family history Chromosomes 2 & 17, AD • Associated clinical syndrome Lentigenes, blue nevus Acromegaly, Cushing’s Thyroid ca or nodules Melanotic schwannaoma Breast ductal adenomas Testicular tumors CNS Manifestations • Emboli – related to mobility not size • Delayed myxoma-induced cerebral aneurysm • Myxomatous metastases Rodriguez et al: Neuropath and Applied Neurobio, 2006 Lee et al: Arch Neurol, 2007 Boikos: Current Opinion Card, 2007 Carney: Mayo Clin Proc, 1986 Cardiac Myxoma - Key Points Majority (95%) • Left or right atrium • Attached to the atrial septum Differential diagnosis • Thrombus • Papillary fibroelastoma • Lipoma • Metastases Benign Masses on Valves Carney syndrome – Endocrine and atypical location Benign Masses Usually on Valves Papillary Fibroelastoma Characteristics • Papillary fibroelastoma • Small mobile tumors ≤1.0 cm • Lambl’s excrescence • “Shimmering” edge • Fenestration • Embolic potential • Cysts • Attached by a stalk (90%) • Immune mediated valve disease • Any endocardial surface • Most often valvular • Associated with endocardial injury – surgery, radiation and HCM Klarich et al: JACC, 1997 3 Papillary Fibroelastoma Papillary Fibroelastoma Sea anemone Gross Appearance Electron micrograph of PFE Probe in RCA ostium Aorta PFE still wet & gelatinous LV Autopsy specimen (opened Ao valve) Baltimore Aquarium Papillary Fibroelastoma Gross Appearance Papillary Fibroelastoma PFE still wet & gelatinous Multiple fronds, MV (H&E, low power) Surgical specimen (aortic valve cusp) Courtesy of Bill Edwards, MD PFE on CT PFE by Echo Symptoms or left sided Yes No Surgical candidate Consider anticoagulation Yes Remove PFE No A/C Tests to consider • Antiphospholipid AB • Blood cultures 4 Echo Features of Blood Cyst • Thin-walled cystic lesion Another Case • Multi-lobed • Attached to valve leaflet • Echo lucent core • If channels are present, may show contrast in the cyst 1° Malignant Cardiac Neoplasms (n=533) Primary Malignant Tumors 6% 7% 31% 9% 11% 15% 21% Angiosarcoma Rhabdomyosarcoma Mesothelioma Fibrosarcoma Other sarcoma Lymphoma Misc McAllister: Atlas of Tumor Pathology, 1978 CP1267233-7 1 Cardiac Tumors Angiosarcoma Primary Cardiac Angiosarcoma • Often in the RA • Associated pericardial effusion 5 Surgical Resection Primary Cardiac Malignancies Neoplastic Heart Disease Primary Cardiac Neoplasms – Location Overall survival 1.0 Left atrium Myxoma (85%) Right atrium Angiosarcoma (90%) 0.8 0.6 Valves PFEs (90%) Resection 0.4 0.2 Ventricles Fibroma (95%) Rhabdomyoma (60%) No Resection 0.0 0 5 10 15 20 25 30 35 40 Survival months Median survival was 17 months vs 6 months in patients with complete resection Moynihan T: Cancer, 2008 Direct Extension Lymphatic Lung Leukemia Breast Lymphoma Esophageal Mediastinal Metastatic Tumors Venous Spread Renal Adrenal Secondary Cardiac Tumors Hematogenous Melanoma Hepatoma Breast Thyroid Lung Leiomyosarcoma GU tract Lung GI tract CP1267233-5 Frequencies of Cardiac Metastases n=100 Cases of Each 50 Hypernephroma Extension into the Renal Vein, IVC and RA 46 40 % 30 20 17 17 13 10 10 0 Melanoma Breast Lymphoma Esophagus Lung Roberts WC: Am J Cardiol 1997 6 28-Year-Old Female, Pericardial Effusion Metastatic Leiomyosarcoma Other Masses and Pseudomasses ©2011 MFMER | 3138928-37 Cardiac Masses Pectus Excavatum Echo for Tumors and Masses • Rapid and safe • Defines anatomic impact on heart • Clues to differential diagnosis • Use all other available information and ancillary studies Questions or Comments? [email protected] • Can not make a pathologic diagnosis 7