Cardiac Masses

Question 1

• Definition of thrombosis:
• General characteristics of a thrombus:

Answer 1

formation of a blood clot within intact vessels

– Begin at a site of endothelial injury, or a site of turbulence of flow, or site of blood stasis
– Point of attachment to wall
– Lines of Zahn: laminations apparent grossly +/or microscopically produced by alternating layers of platelets, fibrin, & RBCs
– May fragment and create emboli

Question 2

Three primary abnormalities that lead to thrombus formation in Virchows Triad

Answer 2

Abnormal blood flow, hypercoagulability, endothelial injury

Question 3

Risk factors for cardiac thrombi

Answer 3

• Abnormal blood flow = abnormal myocardial contraction from: Arrhythmia/ Dilated cardiomyopathy/ Myocardial infarction
• Hypercoagulability
• Endothelial injury from Myocarditis or endocarditis

Question 4

Cardia mural thrombi attach to wall usually at site of:

Answer 4

decreased contraction where blood flow is turbulent (secondary to MI)

Question 5

Significance of Cardiac thrombi

• In left atrium or left ventricle :
• In right atrium or right ventricle:

Answer 5

• In left atrium or left ventricle can embolization to various organs and decreased cardiac output
• In right atrium or right ventricle embolization to lungs and decreased cardiac output

Question 6

Definition of embolus/emboli and causes

Answer 6

detached intravascular mass carried by blood to site distant from origin
– Thrombus (thromboembolism): most common
– Fat
– Air
– Amniotic fluid
– Tumor

Question 7

Consequence of emobli

Answer 7

ischemic necrosis to target organ

Question 8

Origin of systemic embolisms:

Answer 8

– Heart: atrium, ventricle, valve (80%)
• 2/3 with LV infarcts
• ¼ with dilated left atria
– Atherosclerotic plaque (abdominal aorta, carotid artery)

Question 9

Systemic embolism travel in arterial circulation
– What is their destination:
***** Results in infarction of area supplied by vessel

Answer 9

lower legs (75%), brain (10%), intestines, kidneys, spleen, upper extremities

Question 10

What is a paradoxical embolism?

Answer 10

• Paradoxical embolism: travels through heart defect into systemic circulation (patent foramen
ovale)

Question 11

Heart tumors come from where?

Answer 11

Metastases – more common than primary neoplasms
– Primary sites: lung carcinoma, melanoma, lymphomas, breast carcinoma, leukemias

Question 12

Clinical presentation of patients with heart tumors

Answer 12

dependent upon site of tumor within heart (pericardium, conduction system, ventricle)

The masses may be diffuse, mulitnodular, or a single dominant mass

5% of pts dying of cancer have mets to heart

Question 13

What is true of primary neoplasms in the heart:

Answer 13

Most are benign!!

Question 14

What is the most common cardiac tumor and where is it in the heart?

Answer 14

– Myxoma (90% in atria, L:R = 4:1), most common cardiac
tumor, mean age at presentation = 50 yrs
• Sessile or pedunculated (ball-valve obstruction)

Question 15

Histologically what do we see in myxoma’s in the heart?

Answer 15

myxoma cells embedded in abundant ground substance, the tumor cells are splindled and in this amorphous substance

Question 16

Syndrome associated with myxomatous cardiac tumors

Answer 16

Carney syndrome in 10%

Multiple cardiac/extracardiac myxomas (skin, breast, uterus), spotty pigmentation, endocrine overactivity
– Familial (AD and AR

Question 17

Most common heart tumor in infants or children is:

Usually assoicated with:

Answer 17

Rhabdomyoma; may be hamartomas or malformations rather then neoplasms~~ these guys are BENIGN and slow growth, just in a bad place

ASsoicated with tuberous sclerosis

Question 19

Clincal presentation of child with rhabdomyoma in the heart?

Gross morphology of tumor?

Answer 19

Clincal presentation depends on size and location; can be in either or both sides of heart; usally causes congestive sysmptoms and congestive heart fail.

Gross: see multple firm white nodules

Question 20

What are these spider cells assoicated with?

Answer 20

Rhabdomyomas, altered muyocytes with vacuolation

Question 21

– often asymptomatic, may cause ball-valve effect or arrhythmia

– form on valves, can embolize

Answer 21

Lipomas

Papillary fibroelastomas

Question 22

Hemangiomas, lymphagiomas, glomus tumor and bacillary antiomatosis are examples of

Answer 22

Benign vascular malformations and neoplasms

Question 24

– Kaposi sarcoma and hemangioendothelioma are examples of:

Answer 24

Intermediate grade vascular malformations and neoplasms

Question 25

Angiosarcoma and hemagiopericytoma are examples of

Answer 25

Malignant vascular malformations and neoplasms

Question 26

– Very common; 5-10% of benign pediatric tumors; often congenital – Increased numbers of normal or abnormal, blood-filled vesels

Answer 26

Hemangiomas

Question 27

Difference between capillary and cavernous hemangiomas

Answer 27

**Capillary**:Skin, subcutaneous tissue, mucous membranes, some visceral organs --includes juvenile (strawberry)/ Adult (cherry)/ and Pyogenic granulomas **Cavernous**:• Larger, dilated channels; deeper tissue; do not regress. • Associated with von Hippel-Lindau disease

Question 28

Hemangioma that grows for a few months, fades around 2 yo, then usually regresses by age 7

Answer 28

Juvenile "stawberry" hemiangioma... type of Capillary hemangioma

Question 29

Below are two types of capillary hemangioimas, describe them Adult (“cherry”) hemangioma – Pyogenic granulomas –

Answer 29

more common with increasing age; does not regress ulcerate and bleed; seen w/pregnancy or trauma

Question 30

Larger, dilated channels; deeper tissue; do not regress. • Associated with von Hippel-Lindau disease

Answer 30

Cavernous hemangiomas (think **deep** into the **cave**)

Question 31

type of lymphangioma seen on neck or axilla of infants/children • Poorly circumscribed, very large vessels devoid of blood (bc full of lymph) • Associated with Turner syndrome

Answer 31

Cavernous lymphangiomas

Question 32

Bacillary angiomatosis looks like a vascular lesion... but it's not. What is it and how do you diagnosis it?

Answer 32

– Actually Bartonella infection in immunocompromised host – Causes capillary proliferation (bacteria secrete HIF-1) – Diagnosed on biopsy and/or PCR testing

Question 33

clear, small nodules on the head/neck/ axilla

Answer 33

Simple lymphangiomas

Question 34

– Composed of modified smooth muscle cells – Small, on digits, often under nails – Painful, easily cured by excision

Answer 34

Glomus tumor (glomangioma) \*Bening vascular lesion

Question 36

Kaposi Sarcoma is associated or caused in what 4 ways?

Answer 36

**Chronic, classic:** indolent, see multiple, red to purple skin nodules, legs • Eastern European or Mediterranean descent (Ashkenazi Jews) **• Lymphadenopathic, African or endemic: .** African Bantu children and is aggressive **• Transplant – associated KS; **Localized or widely metastatic, mostly internal in lymphs and viscera; not necessarily have cutaneous lesions, regresses with decreased immunosuppresion **• AIDS – associated (epidemic) KS** • 25% of AIDS patients / 40% of homosexual men, 5% of others/ Most common HIV-related malignancy

Question 37

Why is KS so high in homosexual men with AIDS?

Answer 37

Human Herpes Virus type 8 (HHV 8): found in 95% of the lesions – Also called KS-associated herpes virus (KSHV) and infects the endothelial cells \*\*Transmission not limited to sexual contact

Question 38

What type of cells do we see histologically in KS?

Answer 38

Later stages: spindle cells predominant \*Spindle cells are believed to be of endothelial cell origin

Question 39

Disease expression of KS is affected by degree of

Answer 39

immunosuppresion and 95% of time we see HHV 8

Question 40

Tx recommendation for pts with KS that are not immunosuppressed:

Answer 40

– Resection/cryotherapy: if superficial and localized – Radiation: if in limited area – Chemotherapy: if disseminated

Question 41

In pt immunocompromised, what is the tx for KS?

Answer 41

* Decrease immunosuppression if possible * Add chemotherapy and/or radiation if needed * Angiogenesis inhibitors have had success

Question 42

What is Angiosarcoma, who do we see it in and where in the body?

Answer 42

Malignant neoplasm of endothelial cell seen in older adults • Anywhere there are endothelial cells, but commonly: – Skin, soft tissues, breast, liver

Question 43

What are spefic associations do we see in angiosarcomas?

Answer 43

– Liver and arsenic, Thorotrast\*, polyvinyl chloride – Arm in patient with lymphedema, most commonly post mastectomy (often d/t disrupted lymph drainage) – Post radiation

Question 44

Describe the histology you see in angiosarcoma well differentiated: poorly differentiated:

Answer 44

Well differentiated: abundant vascular channels ▫ Less differentiated forms: solid spindle cell proliferation

Question 45

Prognosis of angiosarcoma

Answer 45

5 year survival is 30%

Question 46

Malignant variant of lymphangioma • Occurs in the setting of chronic lymphatic obstruction (such as post-axillary dissection lymphedema after radical mastectomy)

Answer 46

Lymphangiosarcoma