Cardiac neoplasms

Question 1

Classify cardiac neoplasms

Answer 1

Primary Tumors – 75% are benign and 25% malignant

Metastatic/Secondary tumors–metastatic spread (lymphoma, breast, lung, melanoma, sacroma) and direct spread (esophagus, lung breast, thymus) to the heart. 30 x more common than primary cardiac tumors

Sub-diaphragmatic tumors– originate from the kidneys, uterus, liver and adrenals, may invade the atrium via the IVC.

Question 2

What are the most frequent primary benign cardiac tumors

Answer 2

Myxoma
Lipoma 
Fibroelastoma--on cardiac valves (especially aortic and mitral) 
Rhabdomyoma--commonest benign primary cardiac tumor in children
Others
       Fibroma
       Hemangioma
       Teratoma
       Neurofibroma
       Lymphangioma

Question 3

List most frequent primary malignant cardiac tumors

Answer 3

Angiosarcoma--(30%) 
Rhabdomyosarcoma (20%) 
Fibrous histiocytoma
Fibrosarcoma
Leiomyosarcoma
Liposarcoma
Mesiothelioma

Most sarcomas incurable but cardiac transplantation can be considered if no distant spread

Question 4

What is epidemiology of cardiac myxomas

Answer 4

mesenchymal tumors, which can occur at any age, however, they mainly exist between the 30th and 60th year of life with a female predominance
Occur 1: 500 000 with 5% familial
account for 40% of all benign heart lesions
majority are in the left atrium (75%) usually originating from the interatrial septum, with 20% in the right atrium and 5% in the ventricles or attached to valves.

Question 5

What are common metastatic diseases which lead to cardiac tumors. List in order of occurrence

Answer 5

Leukemia
Lymphoma
Melanoma
Lung
Breast

Question 6

What are features of malignant cardiac tumors

Answer 6

20% of all cardiac tumors
most sarcomas 
most > 4th decade
 Male = Female
Most common location is RA
Excision can be performed but with usually only short term survival benefit 
Cardiac Transplant has been performed
Chemo offered

Question 7

What is pathology of Cardiac Myxomas

Answer 7

Neoplasms of endocardial origin derived from either pluripotent mesenchymal cells or endocardial nerve cells
Ovoid, pedunculated masses, which are friable, mucoid, gray-white in color and smooth or granular in appearance, have a stalk, mobile
Histological anlaysis reveals an acid mucopolysaccharide matrix with polygonal cells, capillary-like structures and hemorrhagic areas

Question 8

What are presenting clinical features of cardiac myxomas

Answer 8

A. obstruction to blood flow causing heart failure
B. interference with valvular function
C. systemic emolisation
D. constitutional symptoms–fever, weight loss,anemia,
E Pericardial effusion/tamponade
F. Diastolic murmur (mimics mitral)
G. Early diastolic tumor “plop”
H. Pan-systolic murmur of mitral or tricuspid regurgitation (wrecking ball effect)

Question 9

What is the most common location for a pheochromocytoma

What percentage of intrapericardial pheochromocytomas are malignant

What are the most sensitive and specific biochemical markers in the blood and urine in a patient with a secreting tumor

Answer 9

Left atrial dome behind the aorta and the pulmonary artery

25%

metanephrines

Question 10

35 year old patient admitted with a right ventricle myoxma. Strong family history? what does this suggest?

Answer 10

Carneys syndrome—Autosomial dominant syndrome with variable penetrance that account for 7% of all myxomas. The tumors are often multiple and have a predilection to recur at distant intracardiac and extra cardiac sites

Question 11

List common location of cardiac myxoma

Answer 11

Left atrium = 75%
Right atrium = 20%
Ventricles = 10%
multicentric = 5%

Question 12

What is presentation of rhabdomyoma

Answer 12

most common benign cardiac tumor in children
90% < 15 years of age
30-50% associated with tuberous sclerosis
90% multiple
most occur in ventricles
common in neonates with incessant VT

Question 13

What is the pathology of rhabdomyoma

Answer 13

Benign, yellow-gray tumor

“spider cell” central cytoplasmic mass suspended by fine fibrillar processes

pts present with obstruction or tachyarrhythmias

Question 14

What is different about a fibroma

Answer 14

also usually occurs in children
Ventricular septum or myocardium
Most occur in the AV node region
pacemaker

Question 15

Describe papillary fibroelastoma

Answer 15

7% of all cardiac tumors

Develop on aortic or mitral vale leaflet

Question 16

Describe a Lipoma

Answer 16

Occur most commonly in the atrial septum

Also known as lipomatous hypertropy of the interatrial septum

Question 17

What is natural history of Rhabdomyosarcoma

Answer 17

very aggressive, often presents as acute cardiac failure.
By the time cardiac symptoms become evident, metastatic lesions are usually diffuse. Although survival of up to 5 years has been reported, 9 prognosis is very poor: patients usually survive less than 1 year, in spite of excision of the primary tumor and subsequent radiation and chemo-therapy.

Question 18

What are histological features of Rhabdomyosarcoma

Answer 18

mass of roundish tumor cells with pleomorphic nuclei and large eosino-philic cytoplasm resembling myoblasts.

Monoclonal antibody tests will be positive for desmin, vimentin, and muscle actin and can confirm the embryonic differentiation.

Question 19

What is typical life span and history of angiosarcoma

Answer 19

Very aggressive, 6 to 11 month survival
Removal can be performed
Transplant has been attempted but with poor long term results
about 30% of all malignant cardiac tumors

Question 20

What is Carneys syndrome?

Answer 20

autosomal dominant inheritable disease characterized by myxomas, schwannomas, germ cell tumors, abnormal skin pigmentation and endocrine hyperactivity.
average age at the time of diagnosis is around the age of 20
life expectancy for is reduced.
pts die from complications of cardiac myxomas, metastasizing or intra cranial psammomatous melanotic schwannomas, thyroid carcinomas and metastasising pancreatic or germ cell tumors

Question 21

What is Carney Complex

Answer 21

Carney complex and its subsets LAMB syndrome and NAME syndrome are autosomal dominant conditions comprising myxomas of the heart and skin, hyperpigmentation of the skin (lentiginosis), and endocrine overactivity.

Approximately 5% of all cardiac myxomas are associated with Carney complex

Question 22

What are LAMB and NAME

Answer 22

LAMB acronym refers to lentigines, atrial myxomas, and blue nevi.

NAME refers to nevi, atrial myxoma, myxoid neurofibromas, and ephelides.

Question 23

What are skin lesions of cardiac myxoma

Answer 23

A lentigo is a small pigmented spot on the skin with a clearly-defined edge, surrounded by normal-appearing skin. It is a benign hyperplasia of melanocytes which is linear in its spread.

Question 24

What is gene associated with Carney Complex

Answer 24

Mutations in the PRKAR1A gene on chromosome 17q23-q24,[5] which may function as a tumor-suppressor gene.

Question 25

What is are endocrine manifestation of Carney Syndrome

Answer 25

May manifest as disorders such as Cushing syndrome.
Most common endocrine gland manifestation is an ACTH-independent Cushing’s syndrome due to primary pigmented nodular adrenocortical disease (PPNAD).

Question 26

What is clinical presentation of myxoma

Answer 26

1. complications are embolism (about 30-40%, both peripheral and central, whereas central embolisms are more frequent),
2. intra cardiac obstruction and obstruction of the ventricular outflow tract (seen in left or right 3. heart failure with symptoms of dyspnea, syncope, supra ventricular cardiac arrhythmia).
3. ‘myxoma disease’ with fever, arthralgies, polymyositis, weight loss and hypergammaglobulinaemia

Question 27

Describe Pheochromocytoma

Answer 27

soft, fleshy, tumors, flattened by pressure of the pericardium on the surface of the heart
cannot usually be enucleated, but must be dissected away from the surrounding structures

Question 28

Describe Carcinoid heart disease

Answer 28

Carcinoid syndrome occurs in 10% of pts with carcinoid tumors
GI tract====argentiffen cells===seritonin
Carcinoid heart disease in 50% of pts with syndrome
usually right sided valves (tricuspid most common and then Pulmonary) can left sided.
Glistening, white-yellow fibrous deposits

Question 29

Describe the features of carcinoid disease

Answer 29

Fibrotic deformity of the tricuspid and pulmonary valve
triscupid commissure fused, chorade, thickened, fused and the leaflets thickened and shortened resulting in stenosis and incompetence
microscopically, a deposition of loose or compact fibrous tissue can be seen on both surfaces of the tricuspid leaflet

Question 30

What is in differential diagnosis of cardiac mass

Answer 30

thrombus, vegetation, foreign body, intracardiac metastases, infectious and nonbacterial thrombotic or marantic endocarditis, constrictive cardiomyopathy, and bronchogenic carcinoma or mesothelioma.