Vascular Tumors

Question 1

What are vascular ectasias/telangiectasias?

Answer 1

Ectasia:

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

Telangiectasia:

-permanent dilation of pre-existing small blood vessels

Question 2

What is the most common vascular ectasia?

Answer 2

Nevus flammeus nuchae (nevus simplex)

Question 3

What is nevus flammeus nuchae?

Answer 3

Nevus simplex:

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

Question 4

What is nevus flammeus?

Answer 4

Port-wine stain:

• rare, progressive vascular ectasia
• persists into adulthood

Question 5

What is Sturge-Weber syndrome?

Answer 5

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

Question 6

What is a spider telangiectasia?

(causes)

Answer 6

telangiectasia consisting of central red papule with radiating red lines

Causes (increased estrogen):

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

Question 7

What is hereditary hemorrhagic telangiectasia?

(cause and presentation)

Answer 7

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

Question 8

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

Answer 8

-hemangiomas

Question 9

What is a hemangioma?

Answer 9

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

Question 10

What are the main types of hemangiomas?

Answer 10

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

Question 11

What is the most common type of hemangioma?

Answer 11

-capillary hemangioma

Question 12

What is a capillary hemangioma?

Answer 12

red spot on the skin, mucous membranes, or viscera

-consists of tightly packed, thin-walled capillaries

Question 13

What is a congenital/juvenile hemangioma?

Answer 13

subtype of capillary hemangioma

• present at birth
• typically regresses

Question 14

What is a cavernous hemangioma?

Answer 14

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

Question 15

What disease presents with cavernous hemangiomas?

Answer 15

-von Hippel-Lindau disease

Question 16

What is a pyogenic granuloma?

Answer 16

rapidly growing capillary hemangioma

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

Question 17

What condition is associated with pyogenic granulomas?

Answer 17

Granulosa gravidum (pregnancy tumor):

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

Question 18

What is a glomus tumor?

Answer 18

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

Question 19

What is a lymphangioma?

Answer 19

benign lymphatic neoplasm:

-NOT blood filled

Question 20

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

Answer 20

-pressence or absence of blood in the lumen

Question 21

What are the main types of lymphangioma?

Answer 21

• simple (capillary) lymphangioma
• cavernous lymphangioma (cystic hygroma)

Question 22

What is a simple lymphangioma?

Answer 22

• 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

Question 23

What is a cavernous lymphangioma?

Answer 23

large (potentially up to 15 cm) dilated lymphatic space

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

Question 24

What conditions present with cavernous lymphangiomas?

Answer 24

Common in Turner syndrome (XO)

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

Question 25

What is bacillary angiomatosis? | (appearance)

Answer 25

**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**)

Question 26

What is **Kaposi sarcoma**?

Answer 26

- **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)

Question 27

What are the main forms of Kaposi sarcome?

Answer 27

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**

Question 28

What is angiosarcoma?

Answer 28

**Malignant** vascular tumor

Question 29

What are the main types/locations of angiosarcoma?

Answer 29

- **hepatic angiosarcoma** - **lymphangiosarcoma** (most common in upper extremities)

Question 30

What are risk factors for developing angiosarcoma?

Answer 30

_Hepatic angiosarcoma_: - **aresnic** (pesticides) - **Thorostat** (radioactive contrast, no longer used) - **PVC** (plastics) _Lymphangiosarcoma_: - **chronic lymphedema** (especially _following radical mastectomy_) - **radiation** for carcinoma

Question 31

What is the prognosis of angiosarcoma?

Answer 31

**poor**, they are _very invasive and easily metastasize_

Question 32

What are gross/histologic features of angiosarcomas?

Answer 32

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**