Cardiac And Vascular Tumors

Front 1

What is the most common tumor of the heart?

Back 1

metastatic tumor

Front 2

Myxoma

Back 2

most common primary tumor of the heart (in adults)
Mutation in PRKAR1 on chrom 17; benign
febrile, pass out, tumor in L ventricle (usu) that is mobile and sessile
can become embolus and cause stroke
can cause hyperactive endocrine glands, spotty pigmentation
10% associated with Carney's syndrome: multiple cardiac myxomas
have fever and malaise due to IL6
microscope: spindled mesenchymal-type cells in a loose mucopolysac stroma

Front 3

Rhabdomyoma

Back 3

primary benign tumor common in peds; second most common cardiac tumor
really a hamartoma; can be in L or R ventricle
apoptosis defect and have redundant tissue left over
associated with Tuberous Sclerosis

Front 4

Tuberous Sclerosis

Back 4

have astrocytic scars forming tubers in neocortex (looks like dripped candle wax), SEGAs (subependymal giant cell astromas), angiomyolipomas of the kidney, angiofibromas of the face, usu mentally retarded

Front 5

Metastatic Carcinomas to Heart

Back 5

5% of patients with widespread cancer will develop carcinomas to heart
Malignant; from lung (SVC), breast, leukemia, lymphoma (SVC), melanoma, renal carcinoma (IVC)
will see white spots on pericardiumsince lymphatic drain to outside. Symptoms are related to cardiac filling by effusion, tamponade, or tumor bulk
Can spread through lymphatics, blood, direct contact, veins
decreased contractility can result from myocardial involvement

Front 6

Angiosarcoma

Back 6

The most common primary malignant heart tumor, but rare. Identical to a vascular tumor.
Seen in older M and F in skin, soft tissue, breast, and liver.
*Angiosarcomas of the liver are assoc w PVC, Thortrast, and arsenical pesticides
TO find out what it is, use immunostains for surface R: CD31, CD34, or vWF
The tumor will not effect the epicardium. Only metastatic cancers will affect epicardium

Front 7

Rhabdomyosarcoma

Back 7

malignant version of Rhabdomyoma; second most common primary malignant cardiac tumor
stain for skeletal markers MSA, desmin, and myosin

Front 8

Carcinoma stain

Back 8

stain for keratin

Front 9

What is the most common vascular tumor?

Back 9

hemangioma

Front 10

hemangioma

Back 10

localized proliferation of vessels, most common in H adn N, occur on skin mucous membranes and subcutaneous tissue, liver, spleen, kidney
vulnerable to bleeding and ulceration

Front 11

Pyogenic granuloma

Back 11

not a granuloma, not infectious
rapidly growing polyoid hemangiomas on the skin, gingival and oral mucosa
1/3 dev after trauma. Bleed easily and may ulcerate
Common in pregnancy and then goes away after

Front 12

Lymphangioma/ cystic hygroma

Back 12

webbing of the neck that scars down in teens
TURNER'S
have subcut mass of neck, axilla, and retroperitoneal
It is large lymphatic channels, surgically removed

Front 13

Glomus tumor

Back 13

painful leasion in distal fingertips usu; develops from glomus body, which is specialized SM around vessels that either become pericytes of SM cells
It is branching channels surrounded by nests of glomus cells
tumors are cuboidal cells lining both sides of vessels

Front 14

Vascular ectasias

Back 14

nonneoplastic; they are dilated bv
nevus flammeous (birth mark that will go away) and Sturge-Weber Syndrome

Front 15

Sturge-Weber Syndrome

Back 15

benign lesions in bad locations
a type of vascular ectasias (dilated bv)
Has 3 stages: patch, plaque, and nodule
has sheets of spindle cells, slit like vessels, extravasated RBCs and hyaline globules
port wine stains along the trigeminal nerve; can have vascular malformations of the leptomeninges (arachnoid and pia mater)- if they bleed you will die; assoc with mental retardation

Front 16

Kaposi Sarcoma

Back 16

intermediate grade and low grade malignant- features are bt benign hemangioma and angiosarcoma
2 types: transplant assoc and AIDs assoc
To diagnose, stain for HHV8 markers (95% of infections are HHV8 +)

Front 17

Transplant assoc Kaposi Sarcoma

Back 17

in patients on immunosuppressive therapy, fatal if infects an organ
can be localized or widespread

Front 18

AIDS associated Kaposi Sarcoma

Back 18

precedes AIDs, used to be in 1/3 AIDs patients, but now in 1% due to new drugs
can infect skin, lymph nodes, and viscera