What is cardiac rhabdomyoma?

What is cardiac rhabdomyoma?

A cardiac rhabdomyoma is a non-cancerous tumor. • A tumor can interfere with the way the heart works, blocking blood flow to vital organs and causing an abnormal heart rhythm (arrhythmia). • Cardiac rhabdomyomas naturally shrink during childhood.

What causes cardiac rhabdomyoma?

What causes rhabdomyoma. Tuberous sclerosis, a genetic disorder that causes tumors to form in many different organs, has been reported in up to 60-80% of patients diagnosed with rhabdomyomas.

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.

How is Rhabdomyoma treated?

Patients with adult rhabdomyoma should be treated with surgical resection of head and neck lesions, especially those lesions that compress or displace the tongue and those that may protrude and partially obstruct the pharynx or larynx. Fetal rhabdomyomas are usually located in the subcutaneous tissues.

What is the hallmark microscopic feature of a cardiac Rhabdomyoma?

Microscopic Findings Microscopically, cardiac rhabdomyomas are well-circumscribed nodules that lack a capsule but are distinct from the normal surrounding myocardium. The cells are round or polygonal in shape and are enlarged, with clear cytoplasm (see the image below).

How is rhabdomyoma treated?

Where are rhabdomyomas found?

Rhabdomyoma is the most common form of heart tumor. These tumors can vary greatly in size and number. Rhabdomyomas are usually multiple in number and can affect either side of the heart. In 30 percent of cases, these tumors occur in both sides of the heart.

What are symptoms of RMS?

Rhabdomyosarcoma in the head may cause headaches, bulging of an eye, or a droopy eyelid. In the urinary system, RMS affects urination (peeing) and bowel movements, and can lead to blood in the pee or stool (poop). If a muscle tumor is pressing on a nerve, a child might feel tingling or weakness in that area.

Can alveolar rhabdomyosarcoma be cured?

Research that looks at the causes and treatment of rhabdomyosarcoma has greatly improved the care of patients over the last few decades. Approximately 80% of patients with low-risk rhabdomyosarcoma can now be cured of their disease.

What are the symptoms of RMS?

If RMS becomes more advanced, it can cause symptoms such as lumps under the skin (often in the neck, under the arm, or in the groin), bone pain, constant cough, weakness, or weight loss.

Is a rhabdomyoma encapsulated?

These lesions are well delineated but not encapsulated. Depending on the cellularity, the degree of myxoid matrix and the degree of differentiation, one can discriminate between a classic immature fetal rhabdomyoma and an intermediate fetal rhabdomyoma.

How is rhabdomyoma diagnosed?

How Is Rhabdomyosarcoma (RMS) Diagnosed?

1. Imaging studies. These will likely include a CT scan, MRI, and maybe an X-ray, bone scan, or ultrasound.
2. Biopsy. For a biopsy, a sample of a lump, a sore, or tissue is taken from the body for close examination.
3. Blood tests.
4. Bone marrow aspiration and biopsy.