Tumors of CV System- Melissa (3) Flashcards
What is a hemangioma?
Where do they occur (2)
- Benign tumor of blood vessels that resemble capillaries
- Occur in skin, subQ tissues, mucous membranes, and internal organs (liver, spleen, kidney)
Describe the gross morphology of a hemangioma:
- Vary in size, elevation, and color (blue-red)
- Blood filled, thin walled capillaries (blanching)
What is a strawberry hemangioma?
By when do they regress?
- Hemangioma found on skin of newborns
- Common; regress by 7yoa
What is a glomus tumor?
What do they look like?
From what type of cells do they originate?
Where are they found?
- 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
What are glomus bodies?
AV shunts in skin/ soft tissue at tips of fingers and toes; function to conserve heat/ shunt blood proximally when body is cold
Components of glomus tumor (2)
- Branching vascular channels
- Aggregates of glomus cells
List the 4 vascular ectasias.
Are these benign or malignant tumors?
Benign:
- Nevus Flammus
- Port Wine Stain
- Spider Telangiectasia
- Osler Weber Rendu Disease
What is a nevus flames?
Where do they most commonly occur?
What is their prognosis?
Light pink-purple “Birth mark”
- occurs on head and neck
- most fade or regress
What is a port wine stain and with what disease are they associated?
- special type of nevus flames that may thicken and not fade
- associated with Sturge Weber Syndrome
What are the three findings associated with Sturge Weber Syndrome?
Port wine stain in distribution of CN V
Leptomeninges- venous angiomas
CNS- Mental retardation, seizures, hemiplegia, radiopacities in skull
Spider telangectasias:
- Describe the lesion
- Where are they commonly found?
- In what type of patients do these most often occur (2)?
- Benign, radial, pulsatile vascular lesion w/ blanching central core
- Head and neck
- Associated with ^ Estrogen:
1. Preggos
2. Liver failure!
What is Osler-Weber-Rendu Disease?
What is its inheritance pattern?
Symptoms:
“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!!!
Bacillary Angiomatosis:
What what are these lesions like and what organism causes them?
- Vascular proliferation in immunosuppressed
- Associated with Bartonella infections
4 forms of Kaposi Sarcoma:
- Chronic (older european men)
- Lymphadenopathic (endemic-african children)
- Transplant-associated
- AIDS-Assocated
Describe AIDS Assocated Kaposi Sarcoma:
How common is it?
What is the prognosis?
- # 1 AIDS associated malignancy
- AIDS defining illness
- HAART decreased incidence to under 1%
- Involves lymph nodes, viscera, disseminates widely, but NOT FATAL
Which virus causes AIDS related Kaposi Sarcoma? How does this work?
- KS associated herpesvirus: HHV8
- Tumor progression requires cofactor provided by HIV
Three stages of Kaposi Sarcoma Development: how aggressive are these tumors?
- Patch
- Plaque
- Nodule
* Intermediate grade malignancy*
Characteristics of Kaposi’s sarcoma patch phase (2):
- flat, red-purple macules on lower extremities
- Lool like granulation tissue
Characteristics of Kaposi’s sarcoma plaque phase (2):
- Dilated vascular channels
- Plump spindle cells
Characteristics of Kaposi nodule phase (6)
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
Describe the clinical course of Kaposi Sarcoma: Is it acute or indolent?
Which forms are more aggressive?
How doe we treat AIDS associated?
- Typically indolent, but presentation and course vary widely
- Lymphadenopathic + transplant associated= most aggressive
- AIDS associated clears up with HAART
Describe the gross morphology of angiosarcoma; are these tumors benign or malignant?
- Small, clear, red nodules–> Large, fleshy, gray-white mass
- necrosis + hemorrhage are common
- OVERTLY MALIGNANT*
What are the three causes of HEPATIC angiosarcoma? How quickly do they cause cancer?
- arsenic
- thorotrast (former Xray dye)
- polyvinyl chloride
* long latency period for all toxins*
List two causes of general angiosarcoma:
What is the prognosis for this disease?
- lymphedema (breast cancer surgery, etc)
- radiation therapy
5 year survival = 30%
Describe the microscopic morphology of angiosarcoma
- multiple degrees of differentiation
What is an angioplasty and why is it done?
What are two associated risks with this procedure?
Balloon dilation of artery to stretch media and fracture plaques
Complications:
1. Localized hemorrhagic dissection–> abrupt closure/ thrombosis and reclosure
- Intimal thickening–> proliferative re-stenosis (Occurs in up to 50% of patients w/in 4-6mos)
Endovascular stents:
What are they and what is their purpose?
What are some immediate and late complications associated with this procedure?
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
Which arteries fair better with synthetic grafts?
Do grafts work well for arteries, veins, or both?
- 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
Which vessels are implemented for autologous grafts (2)?
For what procedure are these used?
1: Internal mammary artery
(patency 90% @ 10 yrs)
2. Reversed Saphenous vein
(patency 50% @ 10 yrs)
Used for CABG
What are three complications associated with using the Reversed Saphenous vein as an autologous graft?
- more early thrombosis
- more late intimal thickening
- athroscleorosis w/ rupture, thrombi, aneurysm after 2-3 years
What is the #1 primary cardiac tumor in adults?
Are they benign or malignant?
Where do they typically occur?
What causes them?
- Myxoma (benign)
- Most commonly single tumor in Left Atrium
- 10% hereditary
Describe gross morphology of a cardiac myxoma; what are 3 associated complications?
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
Describe the microscopic morphology of a cardiac myxoma: (3)
- stellate or globular myxoma
- covered with endothelium
- contains structures resembling poorly formed glands or vessels
How are cardiac myxomas diagnosed? Treated?
- Dx with echo
- Surgery is curative
#1 cardiac tumor in kiddos/ infants? Benign or malignant? What do we look for morphologically with these (2)?
Rhabdomyoma (benign)
- Spider cells (large, polygonal, ^ glycogen)
- Resemble a hamartoma more than neoplasm
What are two ways a malignancy can directly invade the heart?
hemorrhagic pericardial effusion; myocardial mets
Describe superior vena cava syndrome:
What type of tumors typically cause this?
Tumor invades/ compresses SVC–> obstruction of venous return from head and upper extremities
- Common associated with lymphomas of the mediastinal area
Describe Inferior vena cava syndrome:
What type of tumors typically cause this?
Renal cell carcinoma grows in renal vein–> infiltrates and obstructs IVC–> Stops venous return from lower extremity
4 negative effects of radiation to treat cardiac/ non cardiac chest neoplasms:
- Serous/serofibrinous pericarditis +/- pericardial effusion
- Fibrosis of basically any heart tissue
- ^ CAD
- Restrictive cardiopathy (probably due to pericarditis)
What are the indications for cardiac transplantation?
severe intractable heart failure, typically due to dilated cardiomyopathy or IHD
3 factors lending to success of cardiac transplants:
- careful selection of candidates
- better maintenance (immunosuppressive drugs)
- sequential endomyocardial biopsies–> early histopathic dx of rejection
What are 4 major complications associated with cardiac transplants?
- Allograft rejection
(^ lymphocytes, myocyte damage on biopsy) - Graft CAD: late, progressive, diffuse–>silent MI, CHF, sudden cardiac death
- Infection
- Malignancy (lymphoma asstd. EBV)
*Grafts typically don’t last more than 15 years
Why do cardiac allografts typically only last 10-15 years?
CAD
Which tumor has spider cells and what do they look like?
Rhabdomyoma– they are large, polygonal, and have lots of glycogen
