Pathology of Cardiac and Vascular Tumors

Question 1

Hemanhioma 
Age
Location
Types
Prognosis

Answer 1

Infancy/Childhood
Skin>Visceral organs
Types
Capillary Hemangioma - Pyogenic granuloma - Inflammed capillary hemangioma - granuloma gravidarum - pregnant women gums
Cavernous Hemangioma - large cavities fill with blood

Regresses Spontaneously

Question 2

Hemangioma
Gross Appearance
Histologic Appearance

Answer 2

Red to blue nodule

Closely packed thin-walled capillaries - No capsule!! Hard to surgically remove

Question 3

Cavernous Hemangioma
Location
Histology
Significance

Answer 3

Visceral Distribution
Large cavernous channels filled with RBCs - NO CAPSULE
Bleeding especially in the liver –> hypovolemic shock
Do not regress as spontaneously

Question 4

Von Hippel Lindau VHL syndrome

Answer 4

AD
Tumor Supressor Gene
Cavernous Hemangioma/Hemangioblastoma - cerebellum, retina

High risk of renal cell carcinoma **

Question 5

Lymphangioma
Age
Location
Types

Answer 5

Children 
Skin and Subcutis - neck, head, axillae 
Morphologic Types 
     Capillary
     Cavernous - cystic hygroma - usually recurs after surgery - congenital syndrome seen in pt with turner's syndrome

Question 6

Lymphangioma
Gross
Histology

Answer 6

Poorly demarcated soft nodule without a capsule

Histology - NO RBCs

Question 7

Glomus Tumor (Glomangioma)

what is a glomus body? what are glomus cells?

Answer 7

Benign Tumor of Glomus body

Glomus body - arteriovenous anastomosis involved in thermoregulation

Glomus cells - Modified SMC

Question 8

Glomus Tumor
Location
Histology

Answer 8

Distal digits - under finger nails
PAINFUL !!!!

Histology
vascular channels
Perivascular aggregates of glomus cells

Question 9

Vascular Ectasia

What is it?
Is it a neoplasm?
Types?

Answer 9

Localized dilation of preexisting BV - not a neoplasm!!

Types
Teleangiectasia - single vessel - spider teleangiectasia
Nevis flammeus - large anatomic are
Nevus fammeus nuchae - located on neck
Port wine Stain
Struge-Weber Syndrome

Question 10

Spider Telangiectasia

Answer 10

Face, neck and upper chest
Hyperestrinism in pregnancy or liver cirrhosis

Compression = disappears

Question 11

Nevus Flammeus

Color
Location
Prognosis
Types

Answer 11

birth mark 
Congenital capillary malformation 
neck and head 
Pink purple flat 
Usually regresses 
Subtypes 
    Nevys Flammeus Nuchae - stork Bite
   Port wine stain - does not regress

Question 12

Sturge Weber Syndrome

Answer 12

Port wine but in trigeminal nerve distribution

you can develop mental retardation

Question 13

Bacillary Angiomatosis

What ?
Who?
2 Species?
Organs effected?

Answer 13

Vascular proliferation

Immunocompromised hosts, oppurtunistic infection - Bartonella
B henselae - cat scratch
B. quintana - trench fever , body lice

Skin bone brain

Question 14

Hemangioendothelioma

Answer 14

Endothelium in origion

Intermediate malignancy - local excision

*not high yield

Question 15

Hemangiopericytoma

Answer 15

Origin - pericytes

Intermediate malignancy

Question 16

Kaposi Sarcoma (KS

Mode of transmission

Answer 16

Malignant tumor of lymphatic endothelium
HHV-8 - opportunistic infection in AIDS patients

Transmission - Sexual Intercourse, Saliva, Blood transfusions
Skin is primarily affected

Question 17

Variants of KS

Answer 17

Chronic (European/Classic) KS
Lymphadenopathic (African/Endemic) KS
Transplant-associated KS
AIDS associated KS

Question 18

KS gross appearance

Answer 18

Skin lesion - pink-red-purple patch –> plaque –> nodule
Patch - flat
Plaque - elevated flat topped
Nodule - elevated dome shaped lesion

Question 19

Histology

Answer 19

Spindle Shaped cells - resemble fibroblasts but do not produce collagen
Small vessels and slit like spaces - rows of RBCs
Interstitium - RBCs, hemosiderin, Macrophages, and plasma cells

Question 20

(Hem)angiosarcoma 
Age 
Location 
What causes it?
Prognosis

Answer 20

Adults
Skin, soft tissue, breast and liver

Irradiation! Hepatic angiosarcoma- Arsenic, thorotrast and PVC **
Highly metastatic - poor prognosis

Question 21

Hemangiosarcoma
Gross
Histology

Answer 21

large fleshy mass - no borders - hemorrhages and necrosis

Sheets of pleomorphic endothelial cells
CD31 + endothelial marker

Question 22

Lymphangiosarcoma

Answer 22

Chronic Lymphedema

Usually after radical mastectomy

Latency period ~10 years

Morphology - resembles hemangiosarcoma except presence of RBC/s

Question 23

Cardiac Myxoma

Where?
Histology?
Complications?

Answer 23

LEFT Atrium
Stellate or spindle shaped cells embedded in a myxoid stroma

Weight loss, fatigue, fever, anemia, elevated ESR - release of cytokines, particularly IL6

Complications
Obstruction of mitral valve orifice
Systemic embolization with brain infarcts

Question 24

Cardiac Rhabdomyoma

Who?
Where?
Prognosis?
Histology?

Answer 24

Children
Ventricles
Regresses spontaneously

Spider cells