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Flashcards in Cardiac Tumors Deck (12)
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Myxoma clinical presentation


ball-valve obstruction, embolization, or a syndrome with fever and malaise
-Constitutional symptoms from IL-6
-Echocardiography lets you visualize the mass
-Surgical removal is curative


Pathogenesis of myxoma

-Arise from multipotent mesenchymal cells
-Familial syndromes are associated with GNAS1 mutations or PRKAR1A mutations
-90% arise in the atria. Left to right 4:1


Morphology of myxoma

-Usually single in the region of the fossa ovalis in the atrial septum
-1 cm to 10 cm
-Sessile or pedunculated lesions
-Globular hard masses with hemorrhage to soft, translucent, papillary or villous lesions that are gelatinous
-During systole they can cause obstruction of the AV valve
-Can act as a wrecking ball
-Peculiar vessel or gland like structures are characteristic
-Hemorrhage or mononuclear inflammation are generally present


Clinical signs of lipoma

-May be asymptomatic or cause ball-valve obstructions and arrhythmias


Pathogenesis of lipoma

Often in the left ventricle, right atrium or atrial septum
-Nonneoplastic lipomatous hypertrophy may occur in the atrial septum. White and brown adipose tissue


Morphology of lipoma

-Localized, well-circumscribed, benign tumors of mature fat cells
-In the subendocardium, subepicardium or myocardium


Pathogenesis of papillary fibroelastoma

-Usually incidental, sea-anenome like lesions found on autopsy
-May embolize


Morphology of papillary fibroelastoma

-80% are located on the valves
-Cluster or hairlike projections made of surface endothelium surrounding a core of myxoid connective tissue


Lambl excrescences

remotely organized thrombus on the aortic valves of older individuals


Clinical presentation of Rhabdomyoma


-Often discovered in the first years of life from a valve or chamber obstruction
-often regress spontaneously


Pathogenesis of Rhabdomyoma

-Half are spontaneous and half are associated with TSC1 or TSC2 (tuberous sclerosis)
-TSC1 and 2 are inhibitors of the mTOR pathway


Morphology of Rhabdomyoma

-Gray-white myocardial masses that can be a couple cm long
-Usually multiple and in the ventricles
-Composed of bizarre, enlarged myocytes
-Histo: spider cells