Cardiac tumors differential diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

• The cardiac tumors have some common features like fever, weight loss, and other constitutional symptoms. Each tumor has its peculiar characteristics that sets it apart from other tumors.

Differenting Cardiac Tumors from Other Diseases

• Primary cardiac tumors have been divided into two categories: benign and malignant.
• Both benign and malignant tumors can be differentiated based on age of presentation, location, morphological features on echocardiography and MRI findings.
• Cardiac MRI is the most useful assessment tool.

Primary Cardiac Tumors

Benign	Malignant
Myxoma	Rhabdomyosarcoma
Rhabdomyoma	Angiosarcoma
Fibroelastoma	Fibrosarcoma
Cardiac Lipoma	Lymphoma
Cardiac Hemangioma
Cardiac Fibroma
Atrioventricular tumors
Paragangliomas

Differential Diagnosis of Cardiac Mass

Cardiac tumors present as a cardiac mass on imaging. The following are the differentials of cardiac mass:

Site of Tumor	Malignant Potential	Type of Tumor	Tissue of Origin	Age of Presentation	Location	Morphology	Signs and Symptoms			MRI Findings
							Systemic Manifestations	Cardiac Manifestations	Embolic Manifestations
Primary Cardiac Tumor	Primary Benign	Myxoma	Mesenchymal	Between third to sixth decade of life	Left atrium (75%) Right atrium (15-20%) Arise from border of fossa ovalis	1-15 cm in diameter Pedunculated Polypoid Smooth and lobulated Villous and pappillary appearance associated with embolization	Constitutional symptoms Carney complex: Spotty skin pigmentation Endocrinal tumors such as sertoli testicular tumors, pituitary adenomas, thyroid tumors Schwannoma NAME syndrome: Nevi Atrial myxoma Myxoid neurofibroma Ephelides LAMB syndrome: Lentigo (small brown patches on the skin) Atrial myxoma Blue nevi	Dyspnea Mitral regurgitation (LA) Right heart failure (RA) Cardiomegaly	Pulmonary hypertension and pulmonary emboli (RA)	Oval mobile left atrial lesion Heterogenous enhancement Attachment to fossa ovalis
		Rhabdomyoma	Striated Muscle	Majority (80%) in infants (<12 months) Most common cardiac mass in childhood 50-70% of all pediatric tumors	Ventricles Interventricular septum	1-3 cm in size Yellow-gray color Firm Circumscribed lobulated Majority multiple if associated with tuberous sclerosis (of those with no association, 50% are single)	Associated with tuberous sclerosis: Developmental delay Renal tumors (angiomyolipomas) Phakomas (white retinal spots) Hypomelanic macules (ash leaf spots) Ungal fibromas Facial angiofibromas Headache Blurred vision Arrhythmias Benign brain tumors (cortical tubers, subependymal nodules)	Outflow obstruction (both right and left ventricles) Arrhythmias (ventricular pre-excitation, Wolff-Parkinson White syndrome) Spontaneous regression in young cases (< 4 years); seldom regress in adult cases	Pulmonary embolism Ischemic stroke	Multiple masses isointense to muscle tissue on T1 images Hyperintense on T2 images
		Fibroma	Fibrous	Children (1/3rd in infants)	Ventricles Ventricular septum >Left ventricular free wall > Right ventricle > Atria Central calcification	Solitary	Asymptomatic Cyanosis	Atypical chest pain Heart failure Cardiomegaly Biventricular hypertrophy Atrioventricular nodal block	Pulmonary embolism Ischemic stroke	Solitary mass Low intensity on T2 weighted image
		Fibroelastoma	Valvular endocardium		Valvular endocardium (Adults-Aortic, children-Tricuspid) Ventricular surface of aortic and pulmonary valves and atrial surface of atrioventricular valves	< 1 cm in diameter Solitary Papillary Flower-like appearance with multiple attachments to valve Short pedicle	Asymptomatic Association with hypertrophic obstructive cardiomyopathy (HOCM)	Subacute bacterial endocarditis	Pulmonary embolism	T1 and T2 weighted images show uniform intermediate signal intensity similar to myocardium Homogeneous late gadolinium contrast enhancement
		Hemangioma	Vascular	< 1 year to 70 years < 2 % of primary cardiac tumors	Right Ventricle Left ventricle Right atrium Small percentage in interatrial septum and left atrium	Polypoid Encapsulated	Cyanosis Kasabach-Merritt syndrome (giant hemangiomas): Thrombosis Thrombocytopenia Coagulopathy	Pericardial effusions CHF Arrhythmias	Pulmonary embolism Ischemic stroke	Intermediate density on T1 images Hypointense on T2 images Multicystic enhancing lesion Involvement of epicardium or pericardium
		Lipoma	Adipose	Between fourth to sixth decade of life	Left ventricle (most commonly subendocardium) Right atrium	Sessile and small (specially subendocardial that protrude into cardiac chambers) Broad pedicle Growth into pericardial space	Asymptomatic	Dyspnea Local compression (subepicardial) Arrhythmia	Ischemic stroke	Epicardial or intramural lesion High intensity on T1 weighted image Drop out on fat saturation images
		Paraganglioma	Nervous (chromaffin cells of the sympathetic ganglia)	Average age of presentation is 11-13 years	Left atrium (under the aorta and the pulmonary artery) Interatrial septum Left ventricle Right ventricular outflow tract	3-8 cm Well-defined Broad base Encapsulated Heterogeneous Hypervascular	Majority asymptomatic May present with symptoms of catecholamine excess: Tachycardia Hypertension Palpitations Fever Diaphoresis Positive for chromogranin and synaptophysin Association with succinate dehydrogenase (SDH) mutation	Dyspnea Valvular obstruction (murmurs) Acute coronary syndrome (anginal pain)	Enascement of coronary arteries by tumor	Well defined lesion arising from atrial walls or septum Bright on T2 weighted imaging
		Atrioventricular Node Tumor	Endodermal	Average age of diagnosis is 38 years Female to male ratio 3:1	Triangle of Koch in the AV nodal region of the atrial septum	2 mm to 2 cm Multiple Cystic Thickening of the atrial septum	Most common cardiac tumor leading to sudden death Emery-Dreifuss muscular dystrophy Midline developmental defects along the central vertical body axis: Thyroglossal duct cysts Cysts in the ovaries, breasts Ventricular septal defect Encephalocele Positive for: Cytokeratin CAM5.2 Cytokeratin AE1/AE3 Cytokeratin 34βE12 Cytokeratin 5/6 (CK5/6) Cytokeratin 7 (CK7) Epithelial membrane antigen (EMA) Carcinoembryonic antigen (CEA) Carbohydrate antigen (CA)19.9, p63, bcl2, galectin 3	Palpitations Dyspnea Chest pain Diziness Syncope Complete heart block Partial AV block Paroxysmal atrial tachycardia Spontaneous intermittent pre-excitation	Myocardial infarction Ischemic stroke	Hypointense cardiac mass on standard imaging Hyperintensity on late gadolinium enhancement (LGE) images with heterogeneous contrast enhancement
		Lipomatous hypertrophy of the interatrial septum	Adipose	> 60 years	Limbus of the fossa ovalis (sparing fossa ovalis membrane)	> 2 cm Interatrial septum maybe upto 7 cm in thickness	Mostly asymptomatic May present with obstructive symptoms such as syncope in rare cases of extension into superior vena cava Associated with: Increased age Obesity Arrhythmias	Palpitations Dyspnea Fatigue	-	Diffuse or nodular thickening of atrial walls Hyperintense to normal myocardium Hypointense to pericardial and posterior mediastinal fat
	Primary Malignant	Fibrosarcoma	Fibrous	20 to 80 years	Left sided (atrial mostly)	Sessile or pedunculated protruding masses in ventricular cavities Soft Lobulated Gelatinous	Fever Fatigue Malaise Weight loss Cytoplasmatic positivity for a-SMA	CHF Pericardial infiltration (effusions)	Pulmonary embolism Ischemic stroke Metastasis	Heterogenous or isointense to myocardium on T1 weighted images
		Angiosarcoma	Vascular	30 to 50 years	Right atrium	Broad-based Internal hemorrhage	Fever Fatigue Weight loss Congestion Superior vena cava syndrome Embolic stroke Endothelial marker D2-40	Dyspnea CHF Pericardial effusions Cardiac tamponade	Pulmonary embolism Ischemic stroke Metastasis	Arterial phase enhancement
		Rhabdomyosarcoma	Mesenchymal	Most common primary sarcoma of children Average age of presentation is 20 years	Left sided (atrial mostly)	Multiple Three types: Embryonic Pleomorphic Alveolar	Fever Fatigue Weight loss Metastasize to lung and lymph nodes	CHF Arrhythmias	Pulmonary embolism Ischemic stroke Metastasis	Intermediate-to-hypointensity compared with muscle on T1 images Hyperintense on T2-weighted imaging with heterogeneous contrast enhancement
		Lymphoma	Lymphoid	10 to 90 years Males > females	Right sided mostly	Majority solitary (1/3rd multiple)	Associations: Chronic immunosupression Epstein Barr virus HIV Diffuse large B cell lymphoma	May involve epicardium and pericardium	Pulmonary embolism Ischemic stroke Metastasis	Hypointense on T1-weighted images and hyperintense on T2-weighted images
Secondary Cardiac Tumor	Metastastatic Malignant	Metastasis	Skin (Melanoma) Lung carcinoma Lymph (leukemia and lymphoma) Breast carcinoma Smooth muscle (Esophageal carcinoma)	Any age	Tumors metastasizing via hematogenous route present as multiple intramyocardial masses: Melanoma Leukemia Sarcoma Tumors metastasizing via venous system as right sided mass: Renal cell carcinoma Hepatocellular carcinoma Lung tumor metastasizes to left atrium	Multiple	Fever Fatigue Weight loss Dysphagia Lymphadenopathy Night sweats	Pericardial effusions Dyspnea Arrhythmia Outflow obstruction	Pulmonary embolism Ischemic stroke Metastasis	Hyperintense relative to myocardium Cardiac mass displaces epicardial fat and pericardial effusion away from heart