Term
| what makes up 50% of the primary tumors found in the heart? |
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Definition
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Term
| who are sporadic myxomas found in most commonly? where are sporadic myxomas usually found? do they often have associated conditions? |
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Definition
| sporadic myxomas are usually found in middle aged females. they are usually found in the L atrium and usually single w/o associated conditions |
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Term
| who are familial myxomas found in most commonly? where are familial myxomas usually found? do they often have associated symptoms? |
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Definition
| familial myxomas are usually found in young males, less commonly in the L atrium, they are multicentric in 1/3 of cases and are associated with extracardiac abnormalities in 20% of cases |
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Term
| how might pts with myoxmas present? |
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Definition
| with syncope - b/c it produces a ball-valve-like change that can occlude blood flow until the person changes position and moves to re-establish flow |
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Term
| what is carney's syndrome? |
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Definition
| myxoma, adrenocortical nodular dysplasia associated with cushing's syndrome, sertoli cell tumor of the testes, and fibroadenomas |
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Term
| what do L sided myxomas present with? |
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Definition
| signs of mitral stenosis or insufficiency |
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Term
| what do R sided myxomas present with? |
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Definition
| dyspnea, syncope, neck vein distention and emboli w/possible infarcts |
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Term
| what does a myxoma look like macroscopically? |
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Definition
| a glob of strawberry jelly - mucinous material, less cellular, more gelatinous |
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Term
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Definition
| no they are benign and well circumscribed |
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Term
| what are the gross features of a myxoma? |
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Definition
| soft, polypoid, pale, lobulated w/ stalk to attach to septum or foramen ovale. calcification may occur over time |
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Term
| what are the histological features of a myxoma? |
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Definition
| round, polygonal cells w/a loose background of mucopolysaccharides |
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Term
| where do myxomas come from? |
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Definition
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Term
| can sx help with myxomas? |
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Definition
| it is often curative, but if some of it is left behind - it may grow back |
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Term
| what is unique about rhabdomyomas in comparison to other tumors? |
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Definition
| they tend to *regress on their own |
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Term
| when do most rhabdomyomas appear? |
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Definition
| many are congenital and are therefore seen early in life |
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Term
| if a rhabdomyoma is found in an adult, what needs to be considered? |
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Definition
| the pt may have *tuberous sclerosis (large tuberous growth of the brain) and or congenital heart disease |
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Term
| are rhabdomyomas multiple or single? |
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Definition
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Term
| are rhabdomyomas malignant? |
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Definition
| no, they are well-circumscribed benign tumors of skeletal muscle |
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Term
| what are microscopic features of rhabdomyomas? |
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Definition
| *spider cells, which are cells w/a central area w/leg-like structures radiating from the cytoplasm |
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Term
| are cardiac fibromas usually found alone or in multiple? |
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Definition
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Term
| when are cardiac fibromas found generally? what kinds of problems might they cause? |
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Definition
| in the 2nd decade - may be associated with sudden death usually due to outflow tract obstruction |
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Term
| what is gorlin's syndrome? |
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Definition
| an increase in BCC, medulloblastoma, rhabdomyosarcoma and cardiac fibroma |
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Term
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Definition
| cerebral gigantism and cardiac fibroma |
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Term
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Definition
| a proliferation of tissues that would normally be found (not necessarily a neoplasm) |
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Term
| how do hamartomas of cardiac myocytes present microscopically? |
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Definition
| microscopically, hamartomas of cardiac myocytes resemble localized hypertrophic cardiomyopathy. there is myocyte diarray, focal areas of scarring, and intramural coronary thickening |
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Term
| where are hamartomas of cardiac myocytes usually seen? |
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Definition
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Term
| what does a calcified amorphous tumor of the heart appear as? |
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Definition
| a nodular deposition of Ca in a background of degenerating *blood cell elements and *chronic inflammation |
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Term
| what is the clinical course of calcified amorphous tumors of the heart? pathogenesis? |
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Definition
| the clinical course of calcified amorphous tumors of the heart is benign, pathogenesis is obscure |
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Term
| what are cardiac MICE? *where are they found? |
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Definition
| Mesothelial/Monocytic Incidental Cardiac Excrenscences. they are found attached to the *endocardium, free floating in the *pericardium or inside an *aortic aneursym |
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Term
| how do cardiac MICE form? |
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Definition
| microscopically, mesothelial cells form strips, tubular and micropapillary formations surrounded by histiocytes |
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Term
| are cardiac MICE malignant? |
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Definition
| no, they are benign, non neoplastic w/no clinical signifigance. they are generally found as incidental microscopic findings at the time of cardiac sx |
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Term
| what is a cystic tumor of the atrioventricular nodal region? is it usually associated with other congenital abnormalities? |
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Definition
| this is more of a *developmental abnormality of *epithelial nature and *endodermal origin. it may be associated with other congenital abnormalities |
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Term
| what can a cystic tumor of the atrioventricular nodal region cause? |
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Definition
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Term
| are cystic tumors of the atrioventricular nodal region seen grossly? |
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Definition
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Term
| how do cystic tumors of the atrioventricular nodal region appear microscopically? |
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Definition
| there may be *ductal structures, *cysts, and *solid nests of epithelial-like cells (which upon electron microscopy, show desmosones and microvilli) |
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Term
| what is a papillary fibroelastoma? how are they usually discovered? |
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Definition
| a small papillary growth that occurs on the surface of the *valves or on other *endocardial locations. they are usually incidental findings at autopsy |
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Term
| how do papillary fibroelastomas appear microscopically? |
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Definition
| microscopically, a papillary fibroelastoma may appear as a lining of *hyperplastic endocardial cells covering a core of *hyalinized hypocellular stroma |
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Term
| what might a papillary fibroelastoma represent? what might cause an increase in their frequency? |
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Definition
| papillary fibroelastomas may represent the end state of the organization of a *mural thrombus, and are seen with increased frequency after *cardiac sx |
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Term
| what is a paraganglioma? where does it commonly present? are there other associated conditions? |
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Definition
| this can present as a primary cardiac neoplasm, usually in the L atrium and usually accompanying *HTN and elevated *urine catecholamine levels |
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Term
| how common are sarcomas in the heart? |
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Definition
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Term
| what is the most common kind of sarcoma to affect the heart? |
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Definition
| angiosarcomas, some may be poorly differentiated/highly pleomorphic and difficult to classify |
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Term
| where do sarcomas usually appear in the heart? |
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Definition
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Term
| what are the kinds of sarcomas that can affect the heart? |
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Definition
| kaposi (from the vasculature, HIV, HHV-8), leiomyosarcoma (from smooth muscle), rhabdomyosarcoma (from skeletal muscle), osteosarcoma (from bone), and fibrosarcoma (often times these can resemble each other) |
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Term
| how will pts usually present if they have sarcomas in their heart? |
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Definition
| often with CHF, arrhythmias, or superior vena cava obstructions/impingement (jugular vein distension, edema of the head and neck structures) |
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Term
| are fibrosarcomas found more commonly one one side of the heart? |
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Definition
| yes, the R side (vs tumors and angiosarcomas which are more common on the L side) |
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Term
| how can sarcomas result in arrhythmias? |
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Definition
| b/c sarcomas are malignant, they can infiltrate the myocardium, impinge on the conduction system & cause it to become hyper-excitable which will lead to arrhythmias |
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Term
| how else can sarcoma cell infiltration of the myocardium cause disease, other than causing problems w/conduction? |
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Definition
| malignant cells also do not have the same contractile properties as normal myocardium, and thus CHF and decreased CO may ensue |
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Term
| what are synovial sarcomas? can they affect the heart? what makes them unique? |
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Definition
| synovial sarcomas are usually found adjacent to joints (synovial) but can affect the heart and have *biphasic growth patterns: *epithelial-type of growth pattern producing primitive glandular structures as well as a *mesenchymal-growth pattern |
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Term
| how would a high grade sarcoma appear? |
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Definition
| abnormal cells, large nuclei, a large N:C ratio, pleomorphic cells (different sizes and shapes), mitoses, and poorly differentiated |
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Term
| how common are lymphomas as primary heart tumors? |
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Definition
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Term
| what are most lymphomas as primary heart tumors associated with? is there a particular type that is more common w/the heart? |
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Definition
| AIDs, and are of the diffuse large cell type |
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Term
| what is the more common cardiac involvement w/lymphoma? |
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Definition
| secondary cardiac involvement by leukemia or lymphoma is more common |
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Term
| which are more common in the heart, metastatic or primary tumors? |
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Definition
| metastatic, which can involve any portion of the heart and if the conduction system is involved, it can result in a heart block |
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Term
| where are primary tumors commonly found that throw metastatic tumors affecting the heart? how does do they usually reach the heart? |
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Definition
| primary tumors in the thoracic or contiguous regions usually reach the heart by spreading to the mediastinal lymph nodes |
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Term
| what kinds of tumors reach the heart via the hematogenous route? |
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Definition
| melanoma, kidney CA, breast CA, and choriocarcinomas |
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Term
| what is the most common metastatic tumor to the heart? |
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Definition
| lung CA then breast then melanoma |
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