Vascular Path Robbins Part 4 Flashcards
benign neoplasms
- hemangioma
- lymphangioma
- glomus tumor
- vascular ectasias
- reactive vascular proliferations (bacillary angiomatosis)
intermediate neoplasms
- kaposi sarcoma
- hemangioendothelioma
malignant neoplasms
- angiosarcoma
- hemangiopericytoma
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
vascular ectasias- what are they?
- congenital or acquired
- not true neoplasms!!!
- ectasia- local dilation of a structure
vascular ectasias
- nevus flammeus
- port wine stain
- spider telangiectasias
- hereditary hemorrhagic telangiectasia
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
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
Sturge-Weber syndrome
- uncommon congenital disorder
- facial port wine nevi, ipsil venous angiomas in cortical leptomeninges, mental retardation, seizures, hemiplegia, skull radio-opacities
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)
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
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
Hemangiomas- types
- capillary
- cavernous
- pyogenic granuloma (lobular capillary hemangioma)
- juvenile (“strawbery type”)
capillary hemangioma
- most common
- thin-walled capillaries, tightly packed together (distinct border)
cavernous hemangioma
- irregular, dilated vascular channels- indistinct border
- more likely to involve deep tissue and to bleed
- component of VHL disease
pyogenic granuloma
(lobular capillary hemangioma)
- type of capillary hemangioma
- rapidly growing! oral mucosa! (may ulcerate)
- pregnancy tumor- occurs in gingiva of pregnant women (uncommon)
lymphangioma- types
- simple lymphangioma
- cavernous lymphangioma (cystic hygroma)
simple lymphangioma
- appear similar to capillary hemangiomas, but w/o RBCs
- subcutis of head/neck and axillae
cavernous lymphangioma
(cystic hygroma)
- neck or axilla of children
- often seen in Turner syndrome!! (in neck)
Glomus tumor
benign tumor- arises from glomus bodies
- distal fingers!
- smooth m origin (not endothelial)
- painful!!!
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!
epithelioid hemangioendothelioma
- intermediate!
- neoplastic endo cells are plump and cuboidal, resembling epit
- vascular channels difficult to recognize
- variable clinical behavior- metastasis in 20-30%
Kaposi sarcoma- caused by?
- intermediate
- caused by HHV8 (human herpesvirus 8)
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
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
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
hepatic angiosarcoma
-assoc with carcinogenic exposures- arsenic, thorotrast (contrast dye), polyvinyl chloride
hemangiopericytoma
-tumors that arise from pericytes, the myofibroblast-like cells assoc with capillaries and venules
