Cardiac Tumors

Question 1

What is a myxoma?

Answer 1

benign (primary) mesenchymal tumor with a gelatinous appearance and abundant ground substance on histology.

• sporadic= middle aged females
• familial= young males

Question 2

***What is the most common primary cardiac tumor in adults?

board question

Answer 2

myxoma

Question 3

Where do myxomas usually form?

Answer 3

They form a pedunculated mass (grows off a little stalk) in the left atrium if left-sided causing mitral valve stenosis, or can form in the right-side causing syncope, dyspnea, and JVD.

Question 4

What is a rhabdomyoma?

Answer 4

benign hamartoma (bc this is skeletal muscle tumor) of cardiac muscle that usually arises in the ventricle during the first decade of life. Form spider cells, which have radial cytoplasmic extensions.

Question 5

What is the most common primary cardiac tumor in CHILDREN?

Answer 5

rhabdomyoma

Question 6

***With what is rhabdomyoma associated?

high yield

Answer 6

tuberous sclerosis

Question 7

**Are metastatic cardiac tumors more or less common than primary cardiac tumors?

Answer 7

more common and reaches the heart via mediastinal lymph nodes (if carcinomas)

Question 8

What are the common metastases to the heart and where do they go?

Answer 8

breast and lung carcinoma, melanoma, and lymphoma (AIDS related; non-hodgkins). They usually go to the pericardium causing a pericardial effusion.
*kidney carcinoma and choriocarcinoma also (these are exceptions bc they are carcinomas that metastasize via hematogenous route rather than the normal lymphatic route for carcinomas).

Question 9

**What is Carney’s syndrome?

Answer 9

myxoma, adrenocortical nodular dysplasia associated with Cushing’s syndrome, Sertoli cell tumor of testis, and fibroadenomas.

Question 10

What is a cardiac fibroma?

Answer 10

a benign fibrotic tumor that is more often seen in the second decade of life and may be associated with sudden death.

Question 11

What is Gorlin’s syndrome?

Answer 11

increased incidence of basal cell carcinoma, medulloblastoma, rhabdomyosarcoma and cardiac fibroma.

Question 12

What is Sotos syndrome?

Answer 12

cerebral gigantism and cardiac fibroma.

Question 13

What is a calcified amorphous tumor of the heart?

Answer 13

nodular deposition of calcium in a background of degenerating blood cell elements and chronic inflammation. Clinical course is benign.

Question 14

***What is Cardiac MICE?

he said know this

Answer 14

Mesothelial/monocytic incidental cardiac excrescences= incidental microscopic findings at the time of cardiac surgery. BENIGN. You will see ribbons of mesothelial cells surrounded by plump histiocytes (stationary macrophage).

Question 15

What is the worry with cystic tumors of the AV nodal region?

Answer 15

can cause complete heart block. Will see cysts lined by squamous like cells.

Question 16

What is a papillary fibroelastoma?

Answer 16

small papillary growth that occurs on the surface of the valves or on other endocardial locations. Will see a core of hyalinized hypocellular stroma.

Question 17

What is paraganglioma?

Answer 17

a rare neuroendocrine neoplasm that causes hypertension and elevated urine catecholamine levels.

Question 18

Are malignant tumors of the heart rare?

Answer 18

YES, but if they do form they are usually angiosarcomas and form a large mass in the RIGHT atrium.

Question 19

What sarcomas could be found in the heart?

Answer 19

• Kaposi sarcoma
• Leiomyosarcoma (smooth muscle)
• Rhabdomyosarcoma (skeletal muscle)
• osteosarcoma
• fibrosarcoma
• remember sarcomas metastasize via hematogenous route.

Question 20

How do patients present if they have a cardiac sarcoma of some sort?

Answer 20

with CHF, arrhythmias, or superior vena cava obstructions.