|Dx:||Heart, benign neoplasm|
|Pre-ICD10 counterpart:||none assigned|
|APACHE Como Component:||none|
|APACHE Acute Component:||none|
|External ICD10 Documentation|
This diagnosis is a part of ICD10 collection.
Cardiac tumors may be primary (benign or malignant) or metastatic (malignant).
Examples of benign primary tumors are: myxomas, papillary fibroelastomas, rhabdomyomas, fibromas, hemangiomas, teratomas, lipomas, paragangliomas, and pericardial cysts.
- Myxoma is most common, accounting for 50% of all primary cardiac tumors. Incidence in women is 2 to 4 times that in men. In uncommon familial forms (Carney complex), men are affected more often. About 75% of myxomas occur in the left atrium, and the rest occur in the other chambers as a solitary tumor or, less commonly, at several sites. Myxomas may be up to 15 cm in diameter. About 75% are pedunculated and may prolapse through the mitral valve and obstruct ventricular filling during diastole. The remainder of the tumors are broad-based and sessile. Myxomas may be myxoid and gelatinous; smooth, firm, and lobular; or friable and irregular. Friable, irregular myxomas increase risk of systemic embolism.
- Carney complex is a familial, autosomal dominant syndrome of recurrent cardiac myxomas with some combination of cutaneous myxomas, myxoid mammary fibroadenomas, pigmented skin lesions (lentigines, ephelides, blue nevi), multiple endocrine neoplasia (primary pigmented nodular adrenocortical disease causing Cushing syndrome, growth hormone and prolactin-producing pituitary adenoma, testicular tumors, thyroid adenoma or carcinoma, and ovarian cysts), psammomatous melanotic schwannoma, breast ductal adenoma, and osteochondromyxoma. Patients are often young at presentation (median age, 20 yr), have multiple myxomas (particularly in the ventricles), and have a higher risk of myxoma recurrence.
- Papillary fibroelastomas are the 2nd most common benign primary tumor. They are avascular papillomas that occur on heart valves in > 80% of cases. The papillomas are more likely to occur on the left side of the heart, predominantly on the aortic and mitral valves. Men and women are affected equally. They have papillary fronds branching from a central core, resembling sea anemones. About 45% are pedunculated. They do not cause valvular dysfunction but increase risk of embolism.
- Rhabdomyomas account for 20% of all primary cardiac tumors and 90% of those in children. Rhabdomyomas affect mainly infants and children, 80% of whom also have tuberous sclerosis. Rhabdomyomas are usually multiple and located intramurally in the septum or free wall of the left ventricle, where they affect the cardiac conduction system. They are firm white lobules that typically regress with age. A minority of patients develop tachyarrhythmias and heart failure due to left ventricular outflow tract obstruction.
- Fibromas occur mainly in children and are associated with adenoma sebaceum of the skin and kidney tumors. They occur primarily on the left side of the heart, are often located within the ventricular myocardium, and may develop in response to inflammation. They can compress or invade the cardiac conduction system, causing arrhythmias and sudden death. Some fibromas occur as part of a syndrome with generalized body overgrowth, jaw keratocytes, skeletal abnormalities, and various benign and malignant tumors (Gorlin, or basal cell nevus syndrome).
- Hemangiomas account for 5 to 10% of benign tumors. They cause symptoms in a minority of patients. Most often, they are incidentally detected during examinations done for other reasons.
- Teratomas of the pericardium affect mainly infants and children. They are often attached to the base of the great vessels. About 90% are located in the anterior mediastinum; the rest, mainly in the posterior mediastinum.
- Lipomas can develop at a wide range of ages. They originate in the endocardium or epicardium and have a large pedunculated base. Many are asymptomatic, but some obstruct flow or cause arrhythmias.
- Paragangliomas, including pheochromocytomas, rarely occur in the heart; when they do, they are usually localized to the base of the heart near vagus nerve endings. They may manifest with symptoms due to catecholamine secretion (eg, increased heart rate and BP, excessive sweating, tremor). Paragangliomas may be benign or malignant.
- Pericardial cysts may resemble a cardiac tumor or pericardial effusion on chest x-ray. They are usually asymptomatic, although some cause compressive symptoms (eg, chest pain, dyspnea, cough).
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