Cardiac paragangliomas can be secretory or non-secreting tumors.
Image of the heart that was replaced in a Puerto Rican patient. Photos: Provided by Dr. Iván González Cancel to the Journal of Medicine and Public Health.
The cardiac paraganglioma It is extremely rare, according to the medical literature, its frequency is only 0.001 to 0.003%. It occurs more frequently in women, between the fourth and fifth decade of life. lifehas an uncertain behavior, and may be malignant in 10% of cases.
On this occasion, in Puerto Ricothe Dr. Iván González Cancel, Cardiovascular Transplant Surgeon y Heart, together with the team of the Cardiovascular Center of Puerto Rico y from the Caribbeanperformed a heart transplant whose tumordespite being benign, threatened the life of the patient.
We had to remove the tumor… well, we removed the heart and thanks to some heroes we put a new one… gift of life @llpr_DoneVida @CentroCardioPR pic.twitter.com/3o53uSLHpM
– IVAN F GONZALEZ-CANC (@drcorazon2016) July 5, 2022
Behind the posting to your personal account of Twitterthe doctor and his team received comments regarding the curious case, which although it is known by specialized personnel, causes large size impact specifically for this tumor and the impact on this vital organ.
scientific concept
According to research reported by specialists in the world, paragangliomas are tumors derived from extraadrenal chromaffin cells found in paravertebral sympathetic ganglia of the thorax, abdomen, and pelvis.
In the heart, the tumor arises from paraganglionic cells located in relation to the great arteries, the coronary arteries, or the atria. Its most frequent location is in the left atrium (55%), followed by the interatrial septum (16%), the anterior surface of the heart (10%) and the right atrium, aortic root or left ventricle in 5% of cases.
Cardiac paragangliomas can be secretory or non-secreting tumors. Secreting tumors produce catecholamines endogenous that lead to an excessive sympathetic discharge, causing arterial hypertension, tachycardia, anxiety, tremors and palpitations, as in the case of our patient.
In the nonsecretory type, patients may present with symptoms such as dyspnea and angina, related to the compression of the heart chambers or the coronary arteries. It is one of the rarest primary cardiac tumors, with <50 cases reported in the literature. 1
These types of tumors are usually found during the study of secondary arterial hypertension, generally in young patients. The prevalence in patients with arterial hypertension varies between 0.2% and 0.6%.
Untreated cardiovascular morbidity and mortality are high, and these tumors may eventually compress cardiac structures and invade adjacent organs.
The available medical literature indicates that cardiac tumors are rare. The incidence of primary heart tumors (PCT) reported in necropsies only reaches 0.05%. Metastatic tumors, on the other hand, are 20 times more common (1% in necropsies1,2)
Between 70 and 90% of primary heart tumors in adults are benign, the most common being atrial myxoma (50%), followed by lipoma (20%), papillary fibroelastoma (15%) and angioma (5). %). In the case of children, the most frequent tumor is the rhabdomyoma.
When they present symptoms, the most common is dyspnea, but they can also present with embolisms, arrhythmias, chest pain, constitutional symptoms and even sudden death.
consulted source here.
