Flashcards in Cardiac neoplasms Deck (30):
Classify cardiac neoplasms
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.
What are the most frequent primary benign cardiac tumors
Fibroelastoma--on cardiac valves (especially aortic and mitral)
Rhabdomyoma--commonest benign primary cardiac tumor in children
List most frequent primary malignant cardiac tumors
Most sarcomas incurable but cardiac transplantation can be considered if no distant spread
What is epidemiology of cardiac myxomas
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.
What are common metastatic diseases which lead to cardiac tumors. List in order of occurrence
What are features of malignant cardiac tumors
20% of all cardiac tumors
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
What is pathology of Cardiac Myxomas
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
What are presenting clinical features of cardiac myxomas
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)
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
Left atrial dome behind the aorta and the pulmonary artery
35 year old patient admitted with a right ventricle myoxma. Strong family history? what does this suggest?
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
List common location of cardiac myxoma
Left atrium = 75%
Right atrium = 20%
Ventricles = 10%
multicentric = 5%
What is presentation of rhabdomyoma
most common benign cardiac tumor in children
90% < 15 years of age
30-50% associated with tuberous sclerosis
most occur in ventricles
common in neonates with incessant VT
What is the pathology of rhabdomyoma
Benign, yellow-gray tumor
"spider cell" central cytoplasmic mass suspended by fine fibrillar processes
pts present with obstruction or tachyarrhythmias
What is different about a fibroma
also usually occurs in children
Ventricular septum or myocardium
Most occur in the AV node region
Describe papillary fibroelastoma
7% of all cardiac tumors
Develop on aortic or mitral vale leaflet
Describe a Lipoma
Occur most commonly in the atrial septum
Also known as lipomatous hypertropy of the interatrial septum
What is natural history of Rhabdomyosarcoma
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.
What are histological features of Rhabdomyosarcoma
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.
What is typical life span and history of angiosarcoma
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
What is Carneys syndrome?
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
What is Carney Complex
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
What are LAMB and NAME
LAMB acronym refers to lentigines, atrial myxomas, and blue nevi.
NAME refers to nevi, atrial myxoma, myxoid neurofibromas, and ephelides.
What are skin lesions of cardiac myxoma
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.
What is gene associated with Carney Complex
Mutations in the PRKAR1A gene on chromosome 17q23-q24, which may function as a tumor-suppressor gene.
What is are endocrine manifestation of Carney Syndrome
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).
What is clinical presentation of myxoma
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).
4. 'myxoma disease' with fever, arthralgies, polymyositis, weight loss and hypergammaglobulinaemia
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
Describe Carcinoid heart disease
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
Describe the features of carcinoid disease
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