Cardiac Masses - Jarzembowski

Question 1

Define: Thrombus

Describe: Lines of Zahn

Answer 1

Formation of a blood clot within intact vessels

laminations (gross or microscopic) produced by alternating layers of platelets, fibrin, and RBCs

Question 2

What is Virchow’s Triad of Thrombosis?

Answer 2

Three primary abnormalities that lead to thrombus formation:

Endothelial injury

Abnormal blood flow

Hypercoagulability

Question 3

Describe the embolization pattern of thrombi based on their origin in the heart

Answer 3

Left atria or ventricle: embolizaiton to organs

Right atria or ventricle: embolization to lungs

Question 4

Name 5 types of emboli. Which is the most common?

Answer 4

• thrombus (thromboembolism) -> most common
• fat
• air
• amniotic fluid
• tumor

Question 5

Where do 80% of systemic emboli originate? Where do they end up?

What is a paradoxical embolism?

Answer 5

The heart (2/3 secondary to LV infarct, 1/4 with dilated left atria). 75% lower legs, 10% brain.

Paradoxical embolism: an embolism that travels through a heart defect (ASD, VSD, PFO, etc) into systemic circulation.

Question 6

What is the most common etiology of heart tumors?

Answer 6

Metastases from other sites -> often lung carcinoma, melanoma, lymphomas, breast carcinoma, leukemias

Question 7

What is the most common primary neoplasm of the heart?

Where do they occur?

Describe them histologically.

Answer 7

Myxoma

90% in atria, L:R 4:1

Myxoma cells (spindle myocytes) embedded in abundant ground substance

Question 8

What is Carney Syndrome?

Answer 8

Multple cardiac/extracardiac myxomas, spotty pigmentation, and endocrine overactivity.

Responsible for ~10% of cardiac myxomas

Question 9

What is a pedunculated mass? Why might this be a problem for the heart?

Answer 9

A mass of tissue supported by a thin stalk from the wall of a cavity. This arrangement can form a ball-valve obstruction in the heart.

Question 10

What is the most common heart tumor of infants/children?

Are these always neoplasms?

Describe them morphologically/histologically

Answer 10

Rhabdomyoma

May also be a hamartoma or other type of malformation (not always neoplasm)

multiple firm, white nodules with spinder cells (altered myocytes with vacuolation)

Question 11

Tuberous sclerosis associated with what type of heart tumor?

Answer 11

rhabdomyoma

Question 12

What kind of heart tumor generally forms on heart valves and can embolize?

Answer 12

Papillary fibroelastoma

Question 13

Name (2) neoplasms in each of the following categories:

• Benign
• Intermediate grade (generally benign)
• Malignant

Answer 13

Benign

• Hemangioma
• Lymphangioma

Intermediate

• Kaposi sarcoma
• Hemangioendothelioma

Malignant

• Angiosarcoma
• Hemangiopericytoma

Question 14

Describe: hemangiomas

What are the two major types? Where do they occur?

Answer 14

Increased numbers of normal or abnormal blood-filled vessels

Capillary Hemangiomas:

• skin, subQ, mucous membranes, some visceral organs
• Includes Juvenile (strawberry), adult (cherry), and pyogenic granulomas

Cavernous Hemangiomas:

• larger, dilated vessels, located in deeper tissue, do not regress
• Associated with von Hippel-Lindau Disease (tumor suppression disease)

Question 15

What is a “strawberry” hemangioma. Who does it affect?

Answer 15

Juvenile hemangioma - primarily affects young children. It tends to fade around 2 years old and completely regresses by 7 years old.

Question 16

What is a “cherry” hemangioma. Who does it affect?

Answer 16

Adult hemangioma - more common with increasing age. Unlike Juvenile, does not regress.

Question 17

What is a pyogenic granuloma hemangioma? Who does it affect?

Answer 17

Hemangioma that ulcerates and bleeds. Seen in pregnancy (regresses after hormone stimulus is removed) or trauma.

Question 18

What is another name for ‘cavernous lymphangioma’

What genetic condition are these associated with?

Describe

Answer 18

cystic hygroma

Turner syndrome (XO)

Poorly circumscribed, very large vessels devoid of blood. Often found on the neck or axilla of infants and children.

Question 19

What is a Glomus Tumor?

Answer 19

Tumor composed of modified smooth muscle cells (Glomus cells)

Often found on the digits, under the nails (extremely painful)

usually cured by excision

Question 20

Bacillary angiomatosis is caused by what?

Describe

Answer 20

Bartonella infection in an immunocompromised host

Bacterial secretion of HIF-1 causes massive local capillary proliferation

Diagnosed with biopsy and/or PCR testing

Question 21

Name 4 general categories of Kaposi Sarcoma

Answer 21

• Chronic/classic - indolent
• Lymphadenopathic (African or endemic)
• Transplant-associated
• AIDS-associated (epidemic)

Question 22

What is the most common HIV-associated malignancy?

Answer 22

Kaposi sarcoma

Question 23

In what populations is Kaposi Sarcoma especially prevalent?

What is the clinical presentation?

Answer 23

Eastern Europeans, Mediterranean descent, Ashkenazi Jews

Presents with multple red-purple skin nodules on the legs

Question 24

What virus is associated with 95% of cases of Kaposi Sarcoma?

Where does it infect? What governs its degree of disease development/progression.

Answer 24

HHV-8 (a.k.a. KSHV)

Infects endothelial cells in immunocompromised individuals. Immunocompetent individuals rarely develop productive infection with HHV-8.

Question 25

Name some treatment options for Kaposi Sarcoma (KS)

Answer 25

• resection/cryotherapy (superficial and localized)
• radiation (limited area)
• chemotherapy (disseminated)
• Decrease immunosuppression
• Angiogenesis inhibitors

Question 26

What is an angiosarcoma? What are its major forms?

What are some specific associations?

What is the usual prognosis?

Answer 26

Malignant neoplasm of endothelial cells (commonly skin, soft tissues, breast, liver), usually found in older adults.

Forms:

• Well-differentiated (abdundant vascular channels)
• Less-differentiated (no apparent vascular channels)

Associations:

• Arsenic (liver)
• Thorotrast (radioactive contrast agent - not in use anymore)
• PVC (polyvinyl chloride)
• Radiation therapy
• Post-mastectomy lymphedema (arm/axilla)

** Poor prognosis ** ~30% 5-yr

Question 27

What is lyphangiosarcoma? Where is it usually seen?

Answer 27

Malignant lymphangioma

Usually secondary to chronic lymphatic obstruction (example: post-axillary dissection lymphedema after radical mastectomy)