Vascular Path Robbins Part 4 Flashcards Preview

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Flashcards in Vascular Path Robbins Part 4 Deck (28)
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1

benign neoplasms

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

2

intermediate neoplasms

-kaposi sarcoma
-hemangioendothelioma

3

malignant neoplasms

-angiosarcoma
-hemangiopericytoma

4

benign vs malignant tumors

-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

5

vascular ectasias- what are they?

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

6

vascular ectasias

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

7

nevus flammeus

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

8

port wine stain

-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

9

Sturge-Weber syndrome

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

10

spider telangiectasias

-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)

11

hereditary hemorrhagic telangiectasia

(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

12

Hemangiomas- what is it? common sites?

-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

13

Hemangiomas- types

-capillary
-cavernous
-pyogenic granuloma (lobular capillary hemangioma)
-juvenile ("strawbery type")

14

capillary hemangioma

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

15

cavernous hemangioma

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

16

pyogenic granuloma

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

17

lymphangioma- types

-simple lymphangioma
-cavernous lymphangioma (cystic hygroma)

18

simple lymphangioma

-appear similar to capillary hemangiomas, but w/o RBCs
-subcutis of head/neck and axillae

19

cavernous lymphangioma

(cystic hygroma)
-neck or axilla of children
-often seen in Turner syndrome!! (in neck)

20

Glomus tumor

benign tumor- arises from glomus bodies
-distal fingers!
-smooth m origin (not endothelial)
-painful!!!

21

Bacillary angiomatosis

-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!

22

epithelioid hemangioendothelioma

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

23

Kaposi sarcoma- caused by?

-intermediate
-caused by HHV8 (human herpesvirus 8)

24

Kaposi sarcoma- 4 clinical forms

-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

25

classic KS- cutaneous lesions- 3 stages

-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

26

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

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

27

hepatic angiosarcoma

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

28

hemangiopericytoma

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