Vascular and Cardiac Neoplasms

Question 1

Origin of glomus tumors:

Answer 1

Smooth muscle cell of the glomus body

Question 2

Sturge-Weber syndrome (3):

Answer 2

• Port wine stain in distribution of the trigeminal nerve
• Venous angiomatous mass in leptomeninges
• Mental retardation, seizures, hemiplegia, radiopacities

Question 3

Osler-Weber-Rendu disease (4):

Answer 3

Hereditary Hemorrhagic Telangiectasia

• Autosomal dominant
• Dilated capillaries and veins present from birth
• Wide distribution
• May rupture (nose bleeds, GI bleeds or hematuria)

Question 4

Bacillary angiomatosis tx:

Answer 4

Macrolides

Question 5

Forms of kaposi sarcoma (4):

Answer 5

• Chronic
• Lymphadenopathic
• Transplant-associated
• AIDS-associated

Question 6

Virus kaposi sarcoma is associated with:

Answer 6

HHV8

Question 7

Three stages of kaposi sarcoma:

Answer 7

1. Patch
2. Plaque
3. Nodule

Question 8

Hepatic angiosarcoma is associated with exposure to (3):

Answer 8

• Arsenic
• Thorotrast (former x-ray dye)
• Polyvinyl chloride (PVC)

Question 9

Angiosarcoma gross morphology (3):

Answer 9

• Begin as small, sharply demarcated, multiple red nodules
• Eventually, large fleshy masses of pale gray-white soft tissue
• Necrosis and hemorrhage frequent

Question 10

Most common primary cardiac tumor in adults:

Answer 10

Myxoma

Question 11

Microscopic morphology of a myxoma (2):

Answer 11

• Composed of stellate or globular myxoma

- Covered by endothelium

Question 12

Most common primary cardiac tumor in infants and children:

Answer 12

Rhabdomyoma

Question 13

Rhabdomyoma:

Answer 13

A hamartoma or malformation rather than neoplasm

Question 14

Superior vena cava syndrome (2):

Answer 14

• Tumor may invade or compress SVC

- Obstruct blood flow from head and upper extremities