Which primary cardiac tumor is associated with tuberous sclerosis?
Most cardiac rhabdomyomas are associated with tuberous sclerosis (TS) and appear in the ventricular myocardium, the atria, the cavoatrial junction, or the epicardial surface.
Which of the following are classic manifestations associated with TSC?
Symptoms
- Skin abnormalities.
- Seizures.
- Cognitive disabilities.
- Behavioral problems.
- Kidney problems.
- Heart issues.
- Lung problems.
- Eye abnormalities.
What causes cardiac rhabdomyoma?
What causes cardiac rhabdomyomas? Cardiac rhabdomyomas are likely caused by genetic changes that happen before birth. These changes lead to tuberous sclerosis. People with tuberous sclerosis have mutations in their TSC1 or TSC2 gene.
What causes a myxoma?
Although there is not a well-defined underlying cause for myxomas, it is suspected to be the result of a combination of environmental and genetic risk factors. Cardiac myxomas can cause valvular obstruction, leading to episodes of fainting, pulmonary edema, symptoms of right heart failure, or embolisms.
What is the difference between tuberous sclerosis and tuberous sclerosis complex?
Tuberous sclerosis, also known as tuberous sclerosis complex, is a rare genetic condition that causes mainly non-cancerous (benign) tumours to develop in different parts of the body. The tumours most often affect the brain, skin, kidneys, heart, eyes and lungs.
Where is rhabdomyoma located?
A rhabdomyoma is a noncancerous (benign) tumor that typically grows in clusters in the heart. Rhabdomyomas are the most common type of cardiac tumors seen in infants and children. Cardiac rhabdomyomas usually grow in the muscles of the left and right ventricles (the lower chambers of the heart).
What is adenoma Sebaceum?
Adenoma sebaceum refers to the reddish-brown papular rash found characteristically in a “butterfly” distribution over the face. This rash is a pathognomonic hallmark of tuberous sclerosis and is very sensitive, occurring in over 85% of patients.
What is an Angiofibroma?
(AN-jee-oh-fy-BROH-muh) A benign (not cancer) tumor that is made up of blood vessels and fibrous (connective) tissue. Angiofibromas usually appear as small, red bumps on the face, especially on the nose and cheeks.
Do myxomas grow back?
The recurrence rate of sporadic myxoma is 2% to 3% (2). Gerbode et al described the first case of myxoma recurrence, which occurred several years after surgical removal. Recurrence is usually seen during the first 3 to 4 years, although it can emerge within a few months to several years after surgical excision.