Table 2.

Clinicopathologic Features of Primary Heart Tumors

Histologic Type	Age			Site in Heart		Multiplicity	Syndromic Association
	Fetuses, Iinfants	Children	Adults	Layer	Location
Benign congenital tumors
Rhabdomyoma	++	+		Myocardium	Ventricles	Usual	Tuberous sclerosis
Fibroma	+	++	+	Myocardium	Ventricle, ventricular septum	Rare	Gorlin syndrome
Histiocytoid cardiomyopathy	++	+/1		Endocardium, myocardium	Ventricles, atrial, AV SA nodes	Always
Benign acquired tumors
Myxoma		+/-	++	Endocardium	LA, atrial septum	Rare	Carney complex
					RA, atrial septum
Papillary fibroelastoma			++	Endocardium	Valves >atria >ventricles	Occasional
Hemangiomab	+	+	+	Myocardium Endocardium	Atria >ventricles	Unusual
Lipomatous hypertrophy			++	Myocardium of atrial septum
Lipoma			++	Myocardium, epicardium, endocardium	All sites	Rare
Inflammatory myofibroblastic tumord	++	+	+/-	Endocardium	Valves >atria	Occasional
Germ cell tumors
Teratoma	++	+	+/-	Pericardial cavity		No
				Ventricular septum (rare)
Yolk sac tumor	++	+		Pericardial cavity		No
				Ventricular septum (rare)
Malignant tumors
Angiosarcoma		+/-	++	All layers	Right atrium, pericardium	Occasional
UPS/myxofibrosarcoma	+/-	+/-	++	Endocardium	Left atrium	Rare
				Other sites
Rhabdomyosarcoma	+/-	++	+	Myocardium	Ventricles	No
Leiomyosarcoma		+/-	++	Endocardium	Left atrium	No
Lymphoma		+/-	++	Myocardium	Right atrium, others	Occasional

WHO, World Health Organization; AV, atrioventricular; SA, sinoatrial; LA, left atrium; RA, right atrium; UPS, undifferentiated pleomorphic sarcoma.