Vascular Tumors Flashcards
What are vascular ectasias/telangiectasias?
Ectasia:
- abnormal dilations of small blood vessels (generic term)
- benign/non-neoplastic
Telangiectasia:
-permanent dilation of pre-existing small blood vessels
What is the most common vascular ectasia?
Nevus flammeus nuchae (nevus simplex)
What is nevus flammeus nuchae?
Nevus simplex:
- common (40-70% of newborns) vascular ectasia -> birth mark
- lesions typcially regress
- common on forehead, eyelid, nose ,and upper lip
What is nevus flammeus?
Port-wine stain:
- rare, progressive vascular ectasia
- persists into adulthood
What is Sturge-Weber syndrome?
Non-inherited, somatic mutation of GNAQ gene during development
Skin:
-**port-wine stain** commonly along disribution of trigeminal nerve
CNS:
- underlying, ipsilateral leptomeningeal angioma w/ calcification-> skull radiopacities
- **mental retardation**
- seizures
Eye:
-glaucoma
What is a spider telangiectasia?
(causes)
telangiectasia consisting of central red papule with radiating red lines
Causes (increased estrogen):
- pregnancy
- liver disease/cirrhosis (decreased estrogen metabolism)
What is hereditary hemorrhagic telangiectasia?
(cause and presentation)
Osler-Weber-Rendu disease:
- autosomal dominant defect inTGF-β singaling
- telangiectasia and AV malformations
-in the skin, mucous membranes, and organs
Presentation:
- telangiectasias (skin and oral cavity are visible locations)
- recurrent epistaxis
-hematuria
-GI bleeding
What is the most common benign tumor of infancy and childhood?
-hemangiomas
What is a hemangioma?
benign, vascular neoplasm:
-increased number of normal or abnormal blood vessels
- blood filled
- typically external on head or neck
- occasionally internal, most commonly affecting the liver
- some types may regress
- malignant transformation is rare
What are the main types of hemangiomas?
- capillary hemangioma
- congenital/juvenile hemanioma (strawberry hemangioma)
- cavernous hemangioma
- pyogenic granuloma
What is the most common type of hemangioma?
-capillary hemangioma
What is a capillary hemangioma?
red spot on the skin, mucous membranes, or viscera
-consists of tightly packed, thin-walled capillaries
What is a congenital/juvenile hemangioma?
subtype of capillary hemangioma
- present at birth
- typically regresses
What is a cavernous hemangioma?
large, dilated vascular channel -> cavernous, blood-filled space
- indistinct, unencapsulated mass
- more likely to affect deep structures
- do not regress
- frequently with dystrophic calcification or thrombosis
Complication:
-risk of bleeding
What disease presents with cavernous hemangiomas?
-von Hippel-Lindau disease
What is a pyogenic granuloma?
rapidly growing capillary hemangioma
- most often in oral mucosa, occasionally on finger
- can occur following trauma
What condition is associated with pyogenic granulomas?
Granulosa gravidum (pregnancy tumor):
- pyogenic granuloma on the gingiva during pregnancy
- can regress or become fibrotic
- 1% of pregnancies
What is a glomus tumor?
mesenchymal (smooth muscle) tumor of glomus body (shunts superficial blood during thermoregulation)
- benign
- can occur anywhere, but most common under the fingernail (subungal)
- painful, especially with temperature changes
- bluish color
What is a lymphangioma?
benign lymphatic neoplasm:
-NOT blood filled
What is the main distinguishing histologic feature of hemangiomas and lymphangiomas?
-pressence or absence of blood in the lumen
What are the main types of lymphangioma?
- simple (capillary) lymphangioma
- cavernous lymphangioma (cystic hygroma)
What is a simple lymphangioma?
- tightly packed lymphatic channels (similar appearance to capillary hemangioma, distinguished only by absence of blood cells)
- typically located on the head, neck, or axilla
- up to 1-2 cm
What is a cavernous lymphangioma?
large (potentially up to 15 cm) dilated lymphatic space
- indistinct, unencapsulated mass (difficult to remove)
- lymphoid aggregates
- typically on neck or axilla of children
What conditions present with cavernous lymphangiomas?
Common in Turner syndrome (XO)
Occasionally in Down syndrome (tri 21) and Klinefelter syndrome (XXY)
What is bacillary angiomatosis?
(appearance)
vascular proliferation due to Bartonella (cat scratch)
- occurs in immunocomprimised pts
- benign
- treated with antibiotics
Appearance:
- red papule/nodule
- capillary proliferation w/ Bartonella bacilli (Warthin-Starry stain)
What is Kaposi sarcoma?
- bordeline-malignant vascular tumor
- caused by HHV8
- red-purple macules -> plaques -> nodules (progression)
- lesions spread and become larger
- typically limited to skin and lower limbs (classic form)
What are the main forms of Kaposi sarcome?
based of affected demographic
Classic:
- older men
- Mediterranean, Middle Eastern, and Jewish
- generally restricted to skin of lower extremities
Endemic (African):
- African children (most common tumor in Africa)
- frequently involves LNs
Transplant-associated:
- T-cell immunosuppressed pts
- can be diffuse
AIDS associated:
- most common type in US
- diffuse and aggressive
What is angiosarcoma?
Malignant vascular tumor
What are the main types/locations of angiosarcoma?
- hepatic angiosarcoma
- lymphangiosarcoma (most common in upper extremities)
What are risk factors for developing angiosarcoma?
Hepatic angiosarcoma:
- aresnic (pesticides)
- Thorostat (radioactive contrast, no longer used)
- PVC (plastics)
Lymphangiosarcoma:
- chronic lymphedema (especially following radical mastectomy)
- radiation for carcinoma
What is the prognosis of angiosarcoma?
poor, they are very invasive and easily metastasize
What are gross/histologic features of angiosarcomas?
If cutaneous:
-multiple red papules/nodules -> fleshy masses with blurred margins
Histologic:
- highly variable; atypical endothelium with scattered vascular channels -> minimal differentiation with minimal blood channels (appearing similar to carinoma/melanoma)
- CD31 staining
