Cardiac Tumors Sharon S. Brouha, MD

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1 Cardiac Tumors Sharon S. Brouha, MD CARDIAC TUMORS Imaging techniques Sharon Sudarshan Brouha, MD, MPH Assistant Clinical Professor Cardiothoracic Imaging Section University of California San Diego Cardiac and pericardial masses Cardiac tumor mimics Overview Prevalence of % at autopsy 75% are benign Clinical i l significance ifi Cardiac physiology Embolism Arrhythmias Radiology: Volume 268: Number 1July 2013 Modality Advantages Disadvantages Echocardiography Often the first study Limited Suspected mass FOV Incidental finding Tissue characterization Noninvasive RV evaluation Accessible Large body habitus Anatomic and functional detail Pulmonary disease Operator dependent Cardiac CT PET Incidental cardiac mass Spatial resolution Tissue characterization Calcification Fat Enhancement Metabolic activity Evaluation of interval change Radiation exposure Limited temporal resolution Limited contrast resolution compared to ECG gating Limited anatomic detail Cardiac Tissue characterization Limited access No radiation exposure ECG gating Breath-holding Radiology: Volume 268: Number 1July 2013 Characteristics of cardiac tumors Cardiac protocol Anatomic considerations Tissue characteristics Standard Optional >5 cm Irregular borders Invasion Right heart involvement Pericardial or pleural involvement Effusions Nodules Multiplicity Heterogeneous signal Hemorrhagic pericardial effusion Contrast enhancement T1 FSE thorax SSFP 2, 4 and SAX and two orthogonal planes through mass T1 DIR in optimal plane T1 DIR with fat suppression in optimal plane T2 TIR in optimal plane Early post Gd T1 DIR with TI ms Early yp post Gd T1 DIR (<2 min post Gd) LGE T1 DIR in optimal plane and SAX Myocardial tagging Perfusion 516

2 Cardiac protocol Cardiac protocol T1 FSE thorax Overview of the thorax Identification of tumor Breath-hold or free breathing if necessary SSFP 2, 4 and SAX Localization of mass Anatomic detail Mobility of mass Functional significance Cardiac protocol Cardiac protocol T1 weighted images pre and post contrast Tissue characterization Fat suppression for diagnosis of fatty elements Early Gd enhancement T1 of ms post Gd renders thrombus low in signal Late Gd enhancement Myocardial tumor infiltration with increased interstitial matrix or scarring T2 weighted images Tissue characterization Edema and liquefactive necrosis=high signal Coagulative necrosis=low signal Hemorrhage or thrombus Cardiac protocol Optional techniques Tagging Pericardial adherence/invasion Pericardial inflammation First pass perfusion Tumor vascularity Imaging techniques Cardiac and pericardial masses Cardiac tumor mimics 517

3 Tumor and tumor-like conditions by location Atria Angiosarcoma (right) Lymphoma (right) Myxoma (left) Other sarcomas (left) Thrombus (left) Ventricles Fibroma Rhabdomyoma Valves Papillary fibroelastoma Vegetations Pericardium Pericardial cyst Metastasest t Cardiac tumors Relative incidence of primary cardiac tumors in adults and children Benign Myxoma (30%) Lipoma (10%) Fibroelastoma (10%) Rhabdomyoma Fibroma Hemangioma Teratoma Malignant Angiosarcoma (9%) Rhabdomyosarcoma (6%) Mesothelioma (4%) Fibrosarcoma (3%) Lymphoma (2%) Other sarcoma Teratoma Metastatic t ti disease to the heart is x more common 28F Hx: Persistent progressive shortness of breath Outside hospital CT chest showed multiple pulmonary emboli Returned to ED for SOB Repeat CT chest showed cardiac mass and thrombus in right atrium and ventricle Sarcoma Angiosarcoma is the most common malignant cardiac tumor in adults Rhabdomyosarcoma is the most common malignant cardiac tumor in children Rare but aggressive tumor with poor prognosis Patient died 1 month after tumor resection UNDIFFERENTIATED PLEOMORPHIC SARCOMA Present between the 3 rd and 5 th decades of life Clinical presentation Right heart failure Hemorrhagic pericardial effusion Metastatic disease 518

4 Sarcoma: Imaging manifestations Location varies depending on histology Angiosarcomaright g g atrium Rhadomyosarcomamultiple Undifferentiated sarcomaleft atrium Heterogeneous signal on T1 and T2 weighted images Avid inhomogeneous enhancement 42M Hx: HIV+, troponin bump and lactate elevation CT abdomen demonstrated cardiac abnormality CT chest and C subsequently ordered Lymphoma Much less common than extracardiac lymphoma with cardiac involvement Typically immunocompromised patients, such as HIV+ DIR T1 TIR T2 Clinical Failure Obstructive symptoms Tamponade Arrhythmias No significant enhancement post gad Lymphoma: Imaging manifestations Predilection for the right heart Multiple lesions Nodular infiltration of myocardium Homogeneous signal T1 and T2 isointense Little to no enhancement on LGE images 70F Hx: Chronic pericardial effusion followed with ECHO Presented to the ED with abdominal pain CT A/P revealed ovarian mass and cardiac mass 519

5 Pericadial biopsy was normal LEFT ATRIAL MYXOMA T1 DIR T1 DIR post gad IR post gad Myxoma Myxoma: Imaging manifestations Occur at interatrial septum near fossa ovalis Smooth, lobulated margins Pedunculated Symptoms Mass effect and obstruction Embolization Constitutional symptoms due to IL-6 Carney complex: skin lentigines, endocrine tumors, fibroadenomas and melanotic schwannomas Location 75% left atrium 20% right atrium 5% right or left ventricle T1 isointense T2 hyperintense Additional features Internal hemorrhage, cysts, necrosis, calcification Mobile on cine images Surface thrombus is low signal on LGE images 17M Hx: Presented in 6/2012 for routine sports physical Arrhythmia with frequent PVCs : mass in the superolateral wall of left ventricle Referred for surgical resection DIR T1 FS DIR T2 TIR T2 IR post gad 520

6 Fibroma 2 nd most common pediatric cardiac tumor Solitary tumors Subtotal resection of tumor with residual tumor at left main bifurcation FIBROMA Clinical presentation Syncope Arrhythmia Chest pain CHF Fibroma: Imaging manifestations Rhabdomyoma Intramural in the ventricles T1 isointense T2 hypointense Hyperenhancement on LGE images Homogeneous +/- calcification Most common pediatric cardiac tumor Multiple in 90% Present in the first year of life Tuberous sclerosis in 50% Typically asymptomatic ti Undergo regression by age 4 Rhabdomyoma: Imaging manifestations Intramural in the ventricles but multiple 82F Hx: Pulmonary artery mass T1 isointense T2 hyperintense Little to no enhancement 521

7 Papillary fibroelastoma: Imaging manifestations ti Typically < 1.5 cm Clinical i l presentation Often asymptomatic Embolization of surface thrombus/tumor No valvular abnormality Surgery if symptomatic or >1cm and left-sided Papillary fibroelastoma: Imaging manifestations ti Pedicle adherent to downstream side of valve unlike vegetation Mobile Isointense T1 Hyperintense T2 No enhancement Relative incidence of primary pericardial tumors Pericardial tumors Benign Pericardial cyst Pericardial lipoma Malignant Mesothelioma Hemangioma Sarcoma Lymphoma Lipoblastomap Paraganglioma Germ cell tumors Fibroma Inflammatory pseudotumor/igg4- related disease PNET Primary pericardial tumors much less common than primary cardiac tumors Direct invasion by neoplasm or metastatic disease is x more common than primary pericardial tumor 41M Hx: Melanoma Referred for CT thorax PERICARDIAL METASTASIS 522

8 Cardiac metastases 20-40x more common than primary cardiac tumors 10-12% of patients with known malignancy have cardiac metastases at autopsy 39F Hx: epigastric pain Referred for CT abdomen Pericardial involvement is most common (~95%) Modes of spread (*most common) Hematogenouslymphoma*, melanoma*, leukemia Direct invasionlung*, breast*, esophagus TransvenousRCC, HCC Metastatic melanoma high T1/low T2 PERICARDIAL CYST T1 DIR SSFP (T2/T1) DIR T1 post gad Lipoma Well-marginated and encapsulated Most arise from the epicardial surface and extend into pericardial space Subendocardial less common Typically incidental and asymptomatic Images courtesy of Dr. Eric Goodman 523

9 Lipoma: Imaging manifestations CT: fat attenuation ti T1 hyperintense Low signal on fat suppressed sequences No enhancement 62F Hx: bilateral carotid bifurcation paragangliomas (carotid body tumors) PET scan and MIBG scan demonstrate mediastinal abnormality 62F Hx: bilateral carotid bifurcation paragangliomas (carotid body tumors) PET scan and MIBG scan demostrate mediastinal abnormality Paraganglioma: Imaging manifestations Rare tumor Typically nonfunctioning Bilateral carotid body tumors PARAGANGLIOMA Clinical presentation Functioning: F i elevated catecholamines, tremors, sweating, palpitations, hypertension 524

10 Paraganglioma: Imaging manifestations Adjacent to left atrium or anterior to aortic root 65M Hx: epicardial mass CT: hypervascular mass Feeding vessel may originate from coronary artery : T2 hyperintense Enhancement post Gd T1 DIR T1 TIR PostgadT13DFSPGR LGE Hemangioma Usually solitary Epicardial mass with extrinsic compression No invasion of cardiac structures HEMANGIOMA Predilection for the ventricles 525

11 Hemangioma: Imaging manifestations Cardiac tumor mimics Heterogeneous T1 hyperintense T2 hyperintense Avid enhancement although calcification or fibrous septations may be present Normal structures Coumadin ridge Between the LAA and the left superior pulmonary vein (Q tip sign) Eustachian valve Cit Crista terminalis Pseudotumors Thrombus Caseous calcification of the mitral annulus Lipomatous hypertrophy > 2cm Nonencapsulated Spares the fossa ovalis giving it a dumbbell shape Vegetation Typically on low pressure side of valve 71F Hx: DOE ECHO: 3 x 2 cm right atrial mass attached to interatrial septum Referred for CCTA Crista terminalis LIPOMATOUS HYPERTROPHY OF THE INTERATRIAL SEPTUM Muscular extending from SVC to IVC within the right atrium Represents the junction of the anterior trabeculated and posterior smooth walled (sinus venosus) portions of the primitive right atrium May be seen on chest CTnot a tumor or thrombus RadioGraphics 2005; 25:

12 References Radiology Jul;268(1):26-43 Radiographics Oct;33(6): Radiographics Jul- Aug;20(4): M metastatic renal cell carcinoma Right atrial thrombus 527

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