Cardiac tumors represent 0.1-0.2% of all tumors and can be divided into two broad categories: primary tumors (which can be malignant or benign) and metastatic tumors (always of a malignant nature). The former are tumors that develop in the heart: they are very rare (less than 1 case in 2000 people) and are generally benign in nature. To the second category belong all those tumors that originate in other parts of the body (primary tumor) and spreading their cells at a distance (metastases) reach the heart where they develop (secondary tumor).
Meaning of myxoma
Myxoma, a benign primary tumor, is the most widespread form of heart cancer in the population. It develops following the transformation process of the endothelial cells that make up the inner lining of the heart, the endocardium. In 80% of cases the myxoma is formed in the left atrium, the heart chamber predisposed to receive oxygen-rich blood from from the lungs. In a percentage between 15 and 30% the myxoma can develop in the right atrium, while the localization at the ventricular level is very rare (5-8%). Myxomas occur mainly in adult women with a peak incidence in the age group ranging from 40 to 60 years.
In children, myxoma has a low frequency, around 10% of all cardiac cancers.Although in the vast majority of cases the appearance of myxomas does not depend on having family members already affected, there are some forms, uncommon, of a hereditary nature. The presence of myxomas of a familial nature is one of the characteristics of Carney's syndrome, characterized by the development of different types of benign tumors in multiple heart chambers This syndrome mainly develops in young males, aged between 20 and 30 years.
The main disorders (symptoms) caused by myxoma are:
- heart failureassociated with leg swelling, shortness of breath and general fatigue
- arrhythmias, which cause palpitations, weakness and fainting
- low blood pressure (hypotension)
Difficulty breathing, the sense of fatigue and fainting are clinical manifestations (symptoms) closely related to the shape, nature and localization of the myxoma: in an upright position, in fact, myxoma, which is generally a peduncle attached to the inner lining of the heart, can be "squeezed" towards the mitral valve, which separates the left ventricle from the "left atrium. This squeezing can cause a blockage of blood flow resulting in a temporary drop in blood pressure. Clinical manifestations (symptoms) are reduced in a lying position with the stomach facing up (supine position). In the vast majority of cases, due to the particular location of the tumor, people experience heart murmurs.
In addition to these main clinical manifestations, other secondary manifestations may appear such as fever, fatigue, petechiae (red dots) on the skin, signs of poor blood circulation, Raynaud's syndrome (syndrome characterized by pain and cooling of the extremities of hands and feet).
It can happen that the myxoma undergoes degeneration, becomes fragile and fragments into tiny pieces.If the fragments pass from the heart into the bloodstream (arteries) they can clog a vessel and block circulation. This is a fearful occurrence with consequences of varying severity depending on the location and extent of the blockage of blood flow. If it blocks a vessel in the brain, for example, it can cause a stroke.
The detection (diagnosis) of myxoma can be complicated because the disorders with which it occurs are often common to other diseases. The doctor may come to suspect a heart tumor following the detection of disorders (anemia, heart murmur, arrhythmia, nonspecific heart failure) not attributable to any other disease.
Usually, the person undergoes an echocardiogram (instrumental examination which consists in "placing an ultrasound probe on the chest to see the images and the functioning of the heart muscle). If necessary, the echocardiogram can be accompanied by an ultrasound transesophageal (in this case the probe is passed through the throat and esophagus until it is positioned directly behind the heart). Further investigations involve the use of computed tomography (CT) or magnetic resonance imaging (MRI).
Unlike what is generally the case with all other cancers, doctors do not perform a biopsy as it could be dangerous due to the location of the tumor.
The definitive therapy for myxoma is the surgery to remove the tumor mass. The person who has had a myxoma must undergo constant checks to verify the possible reappearance of the tumor (relapse). The possibility of recurrence, more frequent in the case of hereditary forms, it is very rare in the case of sporadic myxoma and can be consequent to an "incomplete removal of the initial tumor.
Niguarda Hospital. Heart tumors: myxoma