Primary tumors of the heart are rare and benign in three-quarters of the cases and a quarter are malignant, particularly sarcomas. It shows either chest pain, syncope, heart failure, blows, arrhythmias, conduction disturbances, pericarditis or tamponade, and whose signs and symptoms are related to the location of the disease.
The most common primary cardiac tumor is myxoma.
NANE syndrome (nerves, atrial myxoid neurofibroma and ephelides);
LANB syndrome (atrial myxoma and blue nerves).
Most sporadic myxomas are solitary and are localized in the atrium, especially in the AS in the septum between the atria and even in the ventricles or can have different locations. Familial myxoma mainly occurs in young people in post-operative cases. Most myxomas are fibro vascular pedicle on a shaft having an average diameter of 4-8 cm.
Myxomas are presented clinically as mitral valve diseases, either stenosis or as regurgitation. Symptoms of atrial myxoma are dependent on the position of the tumor. On auscultation is observe a beep sound, at the beginning or mid-diastolic; “tumor poplar”.
Myxoma emboli can cause peripheral or extra-pulmonary signs and symptoms as fever, weight loss cachexia, arthralgia, rash, Raynaud’s phenomenon, hypergammaglobulinemia, anemia, polycythemia, leukocytosis, elevated ESR, thrombocytopenia or thrombocytosis. Therefore due to these reasons, myxoma can be diagnosed as endocarditis, collagenases or non-cardiac tumor.
1. Transthoracic and Transesophageal echocardiography. This method is used for the diagnosis of myxoma, making a localization and measurement of the tumor to perform the surgery. Being with a family history is recommended a screening ultrasound to first-degree relatives of the young patients.
A classification of left atrial myxomas will be:
Type I small myxoma which is prolapsing in VS;
Type II small myxoma which isn’t prolapsing;
Type III large myxoma which is prolapsing;
Type IV large myxoma which isn’t prolapsing.
2. Computed tomography and magnetic resonance. This method brings information about the size, location and the surface of the tumor.
3. Cardiac catheterization and angiography. This method is important for the surgeon being the danger of displacement of tumor emboli, it is not necessary if is not associated with other cardiac disease.
The treatment is done by surgical excision only. In case of inadequate tumor excision with multiple locations might appear a recurrence of the tumor.
Other cardiac benign tumors
Cardiac lipomas – frequent at necropsy can reach 15 cm.
Papillary fibroelastoma – is rarely symptomatic and appears on the valve or adjacent endothelium.
Rhabdomyoma and fibroids – mimicking valvular stenosis is common in infants and children, manifesting as a tumor in the ventricle.
Hemangioma mesothelioma – are small tumors, resulting in intra-myocardial conduction disturbances and sudden death.
Sarcomas – are evolving rapidly and lead to death in 3-4 months by hemodynamic compromise, local invasion and distant metastasis.
Mesothelioma affects the pericardium, and leads to hemorrhagic pericarditis, tamponade.
Infiltration the AV node leads to conduction disturbances and sudden death. For treatment biopsy is mandatory.
Metastatic tumor on the heart
Are the most common tumors and occur in 20% of tumor types, namely in malignant melanoma, leukemia and lymphoma. Also occur with lung neoplasms with an increasing incidence.
At the heart level is made tumor extension through the bloodstream, lymphatic or by direct invasion. Well-defined small nodules configure metastases, they affecting the pericardium, rarely the myocardium and more rarely the endocardium and the heart valves.