Cardiac Masses - Jarzembowski Flashcards
Define: Thrombus
Describe: Lines of Zahn
Formation of a blood clot within intact vessels
laminations (gross or microscopic) produced by alternating layers of platelets, fibrin, and RBCs
What is Virchow’s Triad of Thrombosis?
Three primary abnormalities that lead to thrombus formation:
Endothelial injury
Abnormal blood flow
Hypercoagulability
Describe the embolization pattern of thrombi based on their origin in the heart
Left atria or ventricle: embolizaiton to organs
Right atria or ventricle: embolization to lungs
Name 5 types of emboli. Which is the most common?
- thrombus (thromboembolism) -> most common
- fat
- air
- amniotic fluid
- tumor
Where do 80% of systemic emboli originate? Where do they end up?
What is a paradoxical embolism?
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.
What is the most common etiology of heart tumors?
Metastases from other sites -> often lung carcinoma, melanoma, lymphomas, breast carcinoma, leukemias
What is the most common primary neoplasm of the heart?
Where do they occur?
Describe them histologically.
Myxoma
90% in atria, L:R 4:1
Myxoma cells (spindle myocytes) embedded in abundant ground substance
What is Carney Syndrome?
Multple cardiac/extracardiac myxomas, spotty pigmentation, and endocrine overactivity.
Responsible for ~10% of cardiac myxomas
What is a pedunculated mass? Why might this be a problem for the heart?
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.
What is the most common heart tumor of infants/children?
Are these always neoplasms?
Describe them morphologically/histologically
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)
Tuberous sclerosis associated with what type of heart tumor?
rhabdomyoma
What kind of heart tumor generally forms on heart valves and can embolize?
Papillary fibroelastoma
Name (2) neoplasms in each of the following categories:
- Benign
- Intermediate grade (generally benign)
- Malignant
Benign
- Hemangioma
- Lymphangioma
Intermediate
- Kaposi sarcoma
- Hemangioendothelioma
Malignant
- Angiosarcoma
- Hemangiopericytoma
Describe: hemangiomas
What are the two major types? Where do they occur?
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)
What is a “strawberry” hemangioma. Who does it affect?
Juvenile hemangioma - primarily affects young children. It tends to fade around 2 years old and completely regresses by 7 years old.
What is a “cherry” hemangioma. Who does it affect?
Adult hemangioma - more common with increasing age. Unlike Juvenile, does not regress.
What is a pyogenic granuloma hemangioma? Who does it affect?
Hemangioma that ulcerates and bleeds. Seen in pregnancy (regresses after hormone stimulus is removed) or trauma.
What is another name for ‘cavernous lymphangioma’
What genetic condition are these associated with?
Describe
cystic hygroma
Turner syndrome (XO)
Poorly circumscribed, very large vessels devoid of blood. Often found on the neck or axilla of infants and children.
What is a Glomus Tumor?
Tumor composed of modified smooth muscle cells (Glomus cells)
Often found on the digits, under the nails (extremely painful)
usually cured by excision
Bacillary angiomatosis is caused by what?
Describe
Bartonella infection in an immunocompromised host
Bacterial secretion of HIF-1 causes massive local capillary proliferation
Diagnosed with biopsy and/or PCR testing
Name 4 general categories of Kaposi Sarcoma
- Chronic/classic - indolent
- Lymphadenopathic (African or endemic)
- Transplant-associated
- AIDS-associated (epidemic)
What is the most common HIV-associated malignancy?
Kaposi sarcoma
In what populations is Kaposi Sarcoma especially prevalent?
What is the clinical presentation?
Eastern Europeans, Mediterranean descent, Ashkenazi Jews
Presents with multple red-purple skin nodules on the legs
What virus is associated with 95% of cases of Kaposi Sarcoma?
Where does it infect? What governs its degree of disease development/progression.
HHV-8 (a.k.a. KSHV)
Infects endothelial cells in immunocompromised individuals. Immunocompetent individuals rarely develop productive infection with HHV-8.
Name some treatment options for Kaposi Sarcoma (KS)
- resection/cryotherapy (superficial and localized)
- radiation (limited area)
- chemotherapy (disseminated)
- Decrease immunosuppression
- Angiogenesis inhibitors
What is an angiosarcoma? What are its major forms?
What are some specific associations?
What is the usual prognosis?
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
What is lyphangiosarcoma? Where is it usually seen?
Malignant lymphangioma
Usually secondary to chronic lymphatic obstruction (example: post-axillary dissection lymphedema after radical mastectomy)
