Tumors of CV System- Melissa (3)

Question 1

What is a hemangioma?

Where do they occur (2)

Answer 1

• Benign tumor of blood vessels that resemble capillaries

- Occur in skin, subQ tissues, mucous membranes, and internal organs (liver, spleen, kidney)

Question 2

Describe the gross morphology of a hemangioma:

Answer 2

• Vary in size, elevation, and color (blue-red)

- Blood filled, thin walled capillaries (blanching)

Question 3

What is a strawberry hemangioma?

By when do they regress?

Answer 3

• Hemangioma found on skin of newborns

- Common; regress by 7yoa

Question 4

What is a glomus tumor?
What do they look like?
From what type of cells do they originate?
Where are they found?

Answer 4

• PAINFUL, benign vascular tumor
• Small, elevated, red-blue color, firm nodules
• Originates in smooth muscle of glomus body
• Commonly found on tips of fingers and toes

Question 5

What are glomus bodies?

Answer 5

AV shunts in skin/ soft tissue at tips of fingers and toes; function to conserve heat/ shunt blood proximally when body is cold

Question 6

Components of glomus tumor (2)

Answer 6

• Branching vascular channels

- Aggregates of glomus cells

Question 7

List the 4 vascular ectasias.

Are these benign or malignant tumors?

Answer 7

Benign:

1. Nevus Flammus
2. Port Wine Stain
3. Spider Telangiectasia
4. Osler Weber Rendu Disease

Question 8

What is a nevus flames?
Where do they most commonly occur?
What is their prognosis?

Answer 8

Light pink-purple “Birth mark”

• occurs on head and neck
• most fade or regress

Question 9

What is a port wine stain and with what disease are they associated?

Answer 9

• special type of nevus flames that may thicken and not fade
• associated with Sturge Weber Syndrome

Question 10

What are the three findings associated with Sturge Weber Syndrome?

Answer 10

Port wine stain in distribution of CN V
Leptomeninges- venous angiomas
CNS- Mental retardation, seizures, hemiplegia, radiopacities in skull

Question 11

Spider telangectasias:

• Describe the lesion
• Where are they commonly found?
• In what type of patients do these most often occur (2)?

Answer 11

• Benign, radial, pulsatile vascular lesion w/ blanching central core
• Head and neck
• Associated with ^ Estrogen:
  1. Preggos
  2. Liver failure!

Question 12

What is Osler-Weber-Rendu Disease?
What is its inheritance pattern?

Symptoms:

Answer 12

“Hereditary Hemorrhagic Telangiectasia”

• AD inheritance
• Dilated capillaries, vv’s in mucous membranes - Lesions are benign, present at birth, and distributed across body in well defined regions

*bleeding everywhere: nose, GI, urine, everywhere!!!

Question 13

Bacillary Angiomatosis:

What what are these lesions like and what organism causes them?

Answer 13

• Vascular proliferation in immunosuppressed

- Associated with Bartonella infections

Question 14

4 forms of Kaposi Sarcoma:

Answer 14

1. Chronic (older european men)
2. Lymphadenopathic (endemic-african children)
3. Transplant-associated
4. AIDS-Assocated

Question 15

Describe AIDS Assocated Kaposi Sarcoma:
How common is it?
What is the prognosis?

Answer 15

• # 1 AIDS associated malignancy
• AIDS defining illness
• HAART decreased incidence to under 1%
• Involves lymph nodes, viscera, disseminates widely, but NOT FATAL

Question 16

Which virus causes AIDS related Kaposi Sarcoma? How does this work?

Answer 16

• KS associated herpesvirus: HHV8

- Tumor progression requires cofactor provided by HIV

Question 17

Three stages of Kaposi Sarcoma Development: how aggressive are these tumors?

Answer 17

1. Patch
2. Plaque
3. Nodule
   * Intermediate grade malignancy*

Question 18

Characteristics of Kaposi’s sarcoma patch phase (2):

Answer 18

• flat, red-purple macules on lower extremities

- Lool like granulation tissue

Question 19

Characteristics of Kaposi’s sarcoma plaque phase (2):

Answer 19

• Dilated vascular channels

- Plump spindle cells

Question 20

Characteristics of Kaposi nodule phase (6)

Answer 20

1-2.sheets* of plump spindle cells; scattered small vessels
3-4. slits w/ red cells + hyaline droplets; mitotic figures
5. pink cytoplasmic globules
6. lymph node + viscera involved

Question 21

Describe the clinical course of Kaposi Sarcoma: Is it acute or indolent?
Which forms are more aggressive?
How doe we treat AIDS associated?

Answer 21

• Typically indolent, but presentation and course vary widely
• Lymphadenopathic + transplant associated= most aggressive
• AIDS associated clears up with HAART

Question 22

Describe the gross morphology of angiosarcoma; are these tumors benign or malignant?

Answer 22

• Small, clear, red nodules–> Large, fleshy, gray-white mass
• necrosis + hemorrhage are common
• OVERTLY MALIGNANT*

Question 23

What are the three causes of HEPATIC angiosarcoma? How quickly do they cause cancer?

Answer 23

1. arsenic
2. thorotrast (former Xray dye)
3. polyvinyl chloride
   * long latency period for all toxins*

Question 24

List two causes of general angiosarcoma:

What is the prognosis for this disease?

Answer 24

1. lymphedema (breast cancer surgery, etc)
2. radiation therapy
   5 year survival = 30%

Question 25

Describe the microscopic morphology of angiosarcoma

Answer 25

- multiple degrees of differentiation

Question 26

What is an angioplasty and why is it done? | What are two associated risks with this procedure?

Answer 26

Balloon dilation of artery to stretch media and fracture plaques Complications: 1. Localized hemorrhagic dissection--> abrupt closure/ thrombosis and reclosure 2. Intimal thickening--> proliferative re-stenosis (Occurs in up to 50% of patients w/in 4-6mos)

Question 27

Endovascular stents: What are they and what is their purpose? What are some immediate and late complications associated with this procedure?

Answer 27

Expandable mesh tubes used to preserve lumens potency - Can cause ACUTE thrombosis: put patient on anticoagulant - Can cause LATE intimal thickening + proliferative restenosis: coat in anti proliferative drugs

Question 28

Which arteries fair better with synthetic grafts? | Do grafts work well for arteries, veins, or both?

Answer 28

- Larger arteries; small diameter grafts often fail due to thrombosis and intimal thickening - Work well for ARTERIES and not veins; they do not collapse well enough to replace veins

Question 29

Which vessels are implemented for autologous grafts (2)? | For what procedure are these used?

Answer 29

#1: Internal mammary artery (patency 90% @ 10 yrs) 2. Reversed Saphenous vein (patency 50% @ 10 yrs) Used for CABG

Question 30

What are three complications associated with using the Reversed Saphenous vein as an autologous graft?

Answer 30

- more early thrombosis - more late intimal thickening - athroscleorosis w/ rupture, thrombi, aneurysm after 2-3 years

Question 31

What is the #1 primary cardiac tumor in adults? Are they benign or malignant? Where do they typically occur? What causes them?

Answer 31

- Myxoma (benign) - Most commonly single tumor in Left Atrium - 10% hereditary

Question 32

Describe gross morphology of a cardiac myxoma; what are 3 associated complications?

Answer 32

Single tumor that ranges in size Pedunculated tumors may: 1. Obstruction of AV valve ("Ball valve") 2. Cause "wrecking ball" effects on AV leaflets 3. All tumors can embolize or cause flu like syndrome

Question 33

Describe the microscopic morphology of a cardiac myxoma: (3)

Answer 33

- stellate or globular myxoma - covered with endothelium - contains structures resembling poorly formed glands or vessels

Question 34

How are cardiac myxomas diagnosed? Treated?

Answer 34

- Dx with echo | - Surgery is curative

Question 35

``` #1 cardiac tumor in kiddos/ infants? Benign or malignant? What do we look for morphologically with these (2)? ```

Answer 35

Rhabdomyoma (benign) - Spider cells (large, polygonal, ^ glycogen) - Resemble a hamartoma more than neoplasm

Question 36

What are two ways a malignancy can directly invade the heart?

Answer 36

hemorrhagic pericardial effusion; myocardial mets

Question 37

Describe superior vena cava syndrome: | What type of tumors typically cause this?

Answer 37

Tumor invades/ compresses SVC--> obstruction of venous return from head and upper extremities - Common associated with lymphomas of the mediastinal area

Question 38

Describe Inferior vena cava syndrome: | What type of tumors typically cause this?

Answer 38

Renal cell carcinoma grows in renal vein--> infiltrates and obstructs IVC--> Stops venous return from lower extremity

Question 39

4 negative effects of radiation to treat cardiac/ non cardiac chest neoplasms:

Answer 39

1. Serous/serofibrinous pericarditis +/- pericardial effusion 2. Fibrosis of basically any heart tissue 3. ^ CAD 4. Restrictive cardiopathy (probably due to pericarditis)

Question 40

What are the indications for cardiac transplantation?

Answer 40

severe intractable heart failure, typically due to dilated cardiomyopathy or IHD

Question 41

3 factors lending to success of cardiac transplants:

Answer 41

1. careful selection of candidates 2. better maintenance (immunosuppressive drugs) 3. sequential endomyocardial biopsies--> early histopathic dx of rejection

Question 42

What are 4 major complications associated with cardiac transplants?

Answer 42

1. Allograft rejection (^ lymphocytes, myocyte damage on biopsy) 2. Graft CAD: late, progressive, diffuse-->silent MI, CHF, sudden cardiac death 3. Infection 4. Malignancy (lymphoma asstd. EBV) *Grafts typically don't last more than 15 years

Question 43

Why do cardiac allografts typically only last 10-15 years?

Answer 43

CAD

Question 44

Which tumor has spider cells and what do they look like?

Answer 44

Rhabdomyoma-- they are large, polygonal, and have lots of glycogen