Vascular Path Robbins Part 4

Question 1

benign neoplasms

Answer 1

• hemangioma
• lymphangioma
• glomus tumor
• vascular ectasias
• reactive vascular proliferations (bacillary angiomatosis)

Question 2

intermediate neoplasms

Answer 2

• kaposi sarcoma

- hemangioendothelioma

Question 3

malignant neoplasms

Answer 3

• angiosarcoma

- hemangiopericytoma

Question 4

benign vs malignant tumors

Answer 4

• benign- obvious vascular channels fillwed with blood cells lined by a monolayer of normal-appearing endo cells
• malignant tumors- more cellular and proliferative; cytologic atypia; no well-organized vessels

Question 5

vascular ectasias- what are they?

Answer 5

• congenital or acquired
• not true neoplasms!!!
• ectasia- local dilation of a structure

Question 6

vascular ectasias

Answer 6

• nevus flammeus
• port wine stain
• spider telangiectasias
• hereditary hemorrhagic telangiectasia

Question 7

nevus flammeus

Answer 7

(“birthmark”)

• most common form of vascular ectasias
• light pink to deep purple flat lesion on head or neck- dilated vessels
• most regress spontaneously

Question 8

port wine stain

Answer 8

• special form of nevus flammeus
• grow during childhood, thicken the skin surface, don’t fade with time
• Sturge-Weber syndrome- lesions in trigeminal n distribution

Question 9

Sturge-Weber syndrome

Answer 9

• uncommon congenital disorder
• facial port wine nevi, ipsil venous angiomas in cortical leptomeninges, mental retardation, seizures, hemiplegia, skull radio-opacities

Question 10

spider telangiectasias

Answer 10

• nonneoplastic vascular lesions, resemble a spider
• radial, often pulsatile arrays of dilated subcutaneous a’s or arterioles about a central core that blanch with P
• face, neck, upper chest
• assoc with hyperestrogenic states (pregnancy, liver cirrhosis)

Question 11

hereditary hemorrhagic telangiectasia

Answer 11

(Oslder-Weber-Rendu disease)

• autosomal dominant disorder
• mutations in genes that encode parts of TGF-B signaling pathway
• dilated capillaries and v’s that are present at birth
• skin and oral mucous membranes, resp, GI, urinary tracts
• can spontaneously rupture- epistaxis, GI bleeding, hematuria

Question 12

Hemangiomas- what is it? common sites?

Answer 12

• common benign tumors, most present from birth
• localized increase in neoplastic BVs
• skin, mucous membranes of head and neck, liver
• congenital (junvenile, “strawberry”)- often regress

Question 13

Hemangiomas- types

Answer 13

• capillary
• cavernous
• pyogenic granuloma (lobular capillary hemangioma)
• juvenile (“strawbery type”)

Question 14

capillary hemangioma

Answer 14

• most common

- thin-walled capillaries, tightly packed together (distinct border)

Question 15

cavernous hemangioma

Answer 15

• irregular, dilated vascular channels- indistinct border
• more likely to involve deep tissue and to bleed
• component of VHL disease

Question 16

pyogenic granuloma

Answer 16

(lobular capillary hemangioma)

• type of capillary hemangioma
• rapidly growing! oral mucosa! (may ulcerate)
• pregnancy tumor- occurs in gingiva of pregnant women (uncommon)

Question 17

lymphangioma- types

Answer 17

• simple lymphangioma

- cavernous lymphangioma (cystic hygroma)

Question 18

simple lymphangioma

Answer 18

• appear similar to capillary hemangiomas, but w/o RBCs

- subcutis of head/neck and axillae

Question 19

cavernous lymphangioma

Answer 19

(cystic hygroma)

• neck or axilla of children
• often seen in Turner syndrome!! (in neck)

Question 20

Glomus tumor

Answer 20

benign tumor- arises from glomus bodies

• distal fingers!
• smooth m origin (not endothelial)
• painful!!!

Question 21

Bacillary angiomatosis

Answer 21

• vascular proliferation- in response to Bartonella henselae (cat-scratch disease) and quintana (trench fever- lice)
• on skin of immunocompromised pts!
• localized, red papules
• prolif of capillaries with plump endo cells
• PCR or Warthin-Starry stain- to identify bacteria
• macrolide antibiotics!

Question 22

epithelioid hemangioendothelioma

Answer 22

• intermediate!
• neoplastic endo cells are plump and cuboidal, resembling epit
• vascular channels difficult to recognize
• variable clinical behavior- metastasis in 20-30%

Question 23

Kaposi sarcoma- caused by?

Answer 23

• intermediate

- caused by HHV8 (human herpesvirus 8)

Question 24

Kaposi sarcoma- 4 clinical forms

Answer 24

• AIDS-assoc- most common form in US; most common AIDS-related malignant tumor; may spread to LNs, viscera
• Classic- older men from middle eastern, mediterranean, E europe descent; skin!
• Endemic African- pts <40; may involve LNs
• Transplant-assoc- T-cell immunosuppression; may involve LNs and viscera

Question 25

classic KS- cutaneous lesions- 3 stages

Answer 25

• patches- red/purple macules in LE’s
• raised plaques- spread and become larger
• nodular- become neoplastic- proliferating spindle cells- nodal and visceral involvement in African and AIDs-assoc variants

Question 26

Angiosarcoma- affects? what is it? most common sites? marker?

Answer 26

• malignant endothelial tumor
• age- older, male
• skin, soft tissue, breast, liver
• locally invasive, may metastasize
• 5 yr survival- 30%
• endo marker- CD31

Question 27

hepatic angiosarcoma

Answer 27

-assoc with carcinogenic exposures- arsenic, thorotrast (contrast dye), polyvinyl chloride

Question 28

hemangiopericytoma

Answer 28

-tumors that arise from pericytes, the myofibroblast-like cells assoc with capillaries and venules