Cardiac Tumors B&B Flashcards
most common:
a. primary cardiac tumor
b. primary cardiac tumor in children
c. cardiac tumor overall
a. primary cardiac tumor: myxoma (benign)
b. primary cardiac tumor in children: rhabdomyoma (benign, usually multiple present)
c. cardiac tumor overall: metastatic tumors (usually do not produce symptoms, identified post-mortum)
where are myxomas usually found in the heart? are they benign or malignant?
myxomas: most common primary cardiac tumor (adults), benign
most common in left atrium (80%), usually attached to atrial septum at the border of the fossa ovalis
what are myxomas made of?
mesenchymal cells (undifferentiated), endothelial cells, thrombus/clot, mucopolysaccharides
most common primary cardiac tumor (adults, benign)
what symptoms to patients with a myxoma develop? (most common primary cardiac tumor in adults, benign)
system or ”B symptoms” - fever, chills, sweats
may embolize and cause a stoke
may disrupt mitral valve function —> regurgitation, heart failure
myxomas of the heart most often affect which valve? what is the effect of this? (most common primary cardiac tumor in adults, benign)
recall myxomas most often grow in left atrium - may disrupt mitral valve function —> regurgitation, heart failure
may also cause mitral stenosis symptoms if myxoma sits in the valve —> risk of syncope, sudden death
if a patient dies suddenly and a cardiac myxoma is found in the autopsy, how did they most likely die?
myxomas most often grow in left atrium and can disrupt mitral valve - if they sit in the valve, can cause symptoms of mitral stenosis which may cause syncope or sudden death
[myxomas may also cause mitral regurgitation, depends where it is located]
where are cardiac rhabdomyomas most often located and in whom?
most common primary cardiac tumor in children , mostly <1yo and associated with tuberous sclerosis (90%)
usually multiple masses are present, embedded in ventricular wall (benign)
most regress spontaneously, rarely cause symptoms via obstruction of blood flow
how do cardiac rhabdomyomas appear histologically?
most common primary cardiac tumor in children, mostly <1yo (benign) - usually multiple masses are present
tumor is embedded in ventricular wall
histology shows enlarged, disorganized myocytes with clear cytoplasm + spider cells (nuclear material connects out to periphery of cell)
with what genetic syndrome are cardiac rhabdomyomas associated?
most common primary cardiac tumor in children, mostly <1yo (benign)
associated with tuberous sclerosis (90%!) - autosomal dominant mutation in TSC1 (Hamartin) or TSC2 (Tuberin), causing widespread tumor formation
how does tuberous sclerosis present?
autosomal dominant mutation in either TSC1 (hamartin) or TSC2 (tuberin), causing widespread tumor formation (including cardiac rhabdomyomas)
most common presenting feature is seizures!, may also have ”ash leaf” hypopigmentation, angiofibromas (facial skin spots), mental retardation
Pt is 4yo F presenting to their pediatrician due to recent onset of seizures. PMH is significant for cardiac rhabdomyoma at 6mo, which spontaneously resolved. PE is significant for ash leaf spots of hypopigmentation and facial angiofibromas. What is the most likely diagnosis?
tuberous sclerosis: autosomal dominant mutation in either TSC1 (hamartin) or TSC2 (tuberin), causing widespread tumor formation (including cardiac rhabdomyomas)
most common presenting feature is seizures!, may also have ”ash leaf” hypopigmentation, angiofibromas (facial skin spots), mental retardation
