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(J2) CPR II Test 1 (Cardiovascular) > Vascular Tumors > Flashcards

Vascular Tumors Flashcards

1
Q

What are vascular ectasias/telangiectasias?

A

Ectasia:

  • abnormal dilations of small blood vessels (generic term)
  • benign/non-neoplastic

Telangiectasia:

-permanent dilation of pre-existing small blood vessels

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2
Q

What is the most common vascular ectasia?

A

Nevus flammeus nuchae (nevus simplex)

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3
Q

What is nevus flammeus nuchae?

A

Nevus simplex:

  • common (40-70% of newborns) vascular ectasia -> birth mark
  • lesions typcially regress
  • common on forehead, eyelid, nose ,and upper lip
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4
Q

What is nevus flammeus?

A

Port-wine stain:

  • rare, progressive vascular ectasia
  • persists into adulthood
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5
Q

What is Sturge-Weber syndrome?

A

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

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6
Q

What is a spider telangiectasia?

(causes)

A

telangiectasia consisting of central red papule with radiating red lines

Causes (increased estrogen):

  • pregnancy
  • liver disease/cirrhosis (decreased estrogen metabolism)
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7
Q

What is hereditary hemorrhagic telangiectasia?

(cause and presentation)

A

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

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8
Q

What is the most common benign tumor of infancy and childhood?

A

-hemangiomas

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9
Q

What is a hemangioma?

A

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
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10
Q

What are the main types of hemangiomas?

A
  • capillary hemangioma
  • congenital/juvenile hemanioma (strawberry hemangioma)
  • cavernous hemangioma
  • pyogenic granuloma
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11
Q

What is the most common type of hemangioma?

A

-capillary hemangioma

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12
Q

What is a capillary hemangioma?

A

red spot on the skin, mucous membranes, or viscera

-consists of tightly packed, thin-walled capillaries

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13
Q

What is a congenital/juvenile hemangioma?

A

subtype of capillary hemangioma

  • present at birth
  • typically regresses
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14
Q

What is a cavernous hemangioma?

A

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

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15
Q

What disease presents with cavernous hemangiomas?

A

-von Hippel-Lindau disease

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16
Q

What is a pyogenic granuloma?

A

rapidly growing capillary hemangioma

  • most often in oral mucosa, occasionally on finger
  • can occur following trauma
17
Q

What condition is associated with pyogenic granulomas?

A

Granulosa gravidum (pregnancy tumor):

  • pyogenic granuloma on the gingiva during pregnancy
  • can regress or become fibrotic
  • 1% of pregnancies
18
Q

What is a glomus tumor?

A

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
19
Q

What is a lymphangioma?

A

benign lymphatic neoplasm:

-NOT blood filled

20
Q

What is the main distinguishing histologic feature of hemangiomas and lymphangiomas?

A

-pressence or absence of blood in the lumen

21
Q

What are the main types of lymphangioma?

A
  • simple (capillary) lymphangioma
  • cavernous lymphangioma (cystic hygroma)
22
Q

What is a simple lymphangioma?

A
  • 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
23
Q

What is a cavernous lymphangioma?

A

large (potentially up to 15 cm) dilated lymphatic space

  • indistinct, unencapsulated mass (difficult to remove)
  • lymphoid aggregates
  • typically on neck or axilla of children
24
Q

What conditions present with cavernous lymphangiomas?

A

Common in Turner syndrome (XO)

Occasionally in Down syndrome (tri 21) and Klinefelter syndrome (XXY)

25
Q

What is bacillary angiomatosis?

(appearance)

A

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)
26
Q

What is Kaposi sarcoma?

A
  • 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)
27
Q

What are the main forms of Kaposi sarcome?

A

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
28
Q

What is angiosarcoma?

A

Malignant vascular tumor

29
Q

What are the main types/locations of angiosarcoma?

A
  • hepatic angiosarcoma
  • lymphangiosarcoma (most common in upper extremities)
30
Q

What are risk factors for developing angiosarcoma?

A

Hepatic angiosarcoma:

  • aresnic (pesticides)
  • Thorostat (radioactive contrast, no longer used)
  • PVC (plastics)

Lymphangiosarcoma:

  • chronic lymphedema (especially following radical mastectomy)
  • radiation for carcinoma
31
Q

What is the prognosis of angiosarcoma?

A

poor, they are very invasive and easily metastasize

32
Q

What are gross/histologic features of angiosarcomas?

A

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
33
Q
A

(J2) CPR II Test 1 (Cardiovascular) (18 decks)

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