Cardiac Tumors

Question 1

Myxoma clinical presentation

Answer 1

Adults

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

Question 2

Pathogenesis of myxoma

Answer 2

• 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

Question 3

Morphology of myxoma

Answer 3

• 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

Question 4

Clinical signs of lipoma

Answer 4

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

Question 5

Pathogenesis of lipoma

Answer 5

Often in the left ventricle, right atrium or atrial septum

-Nonneoplastic lipomatous hypertrophy may occur in the atrial septum. White and brown adipose tissue

Question 6

Morphology of lipoma

Answer 6

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

Question 7

Pathogenesis of papillary fibroelastoma

Answer 7

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

Question 8

Morphology of papillary fibroelastoma

Answer 8

• 80% are located on the valves

- Cluster or hairlike projections made of surface endothelium surrounding a core of myxoid connective tissue

Question 9

Lambl excrescences

Answer 9

remotely organized thrombus on the aortic valves of older individuals

Question 10

Clinical presentation of Rhabdomyoma

Answer 10

Kids

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

Question 11

Pathogenesis of Rhabdomyoma

Answer 11

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

Question 12

Morphology of Rhabdomyoma

Answer 12

• 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