vascular neoplasia

Question 1

Nevus flammeus

Answer 1

“birthmark”

most common form of vascular ectasia

light pink to deep purple flat lesion on the head or neck composed of dilated vessels. Most ultimately regress spontaneously.

Question 2

port wine stain

Answer 2

special form of nevus flammeus.

tend to grow during childhood,

thicken the skin surface

do not fade with time.

Question 4

autosomal dominant disorder caused by mutations in genes that encode components of the TGF-β signaling pathway.

lesions can spontaneously rupture, causing seri­ous epistaxis (nosebleed), gastrointestinal bleeding, or hematuria.

Answer 4

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)

autosomal dominant disorder caused by mutations in genes that encode components of the TGF-β signaling pathway.

telangiectasias are malformations composed of dilated capillaries and veins that are present at birth.

widely distributed over the skin and oral mucous membranes, as well as in the respiratory, gastrointestinal, and urinary tracts. The lesions can spontaneously rupture, causing seri­ous epistaxis (nosebleed), gastrointestinal bleeding, or hematuria.

Question 5

nonneoplastic vascular lesions that blanch with pressure…these are most frequently associated with..

Answer 5

1. neoplasm described called “Spider telangiectasias”
   
   1. nonneoplastic vascular lesions
   2. grossly resembling spider
   3. manifest as radial, often pulsatile arrays of dilated subcutaneous arteries or arterioles (resembling spider legs) about a central core (resembling a spider’s body)
   4. blanch with pressure
   5. These commonly occur on the face, neck, or upper chest
   6. most frequently associated with hyperestrogenic states
      
      1. pregnancy or liver cirrhosis.

Question 6

lesions in the distribution of the trigeminal nerve

Answer 6

1. port wine stain (special form of nevus flammeus) associated with Sturge-Weber syndrome
   
   1. also called encephalotrigeminal angiomatosis
   2. “large facial telangiectasia in a child with mental deficiency “
2. uncommon congenital disorder
3. associated with
   
   1. facial port wine nevi
   2. ipsilateral venous angiomas in the cortical leptomeninges
   3. mental retardation
   4. seizures
   5. hemiplegia
   6. skull radio-opacities

Question 7

occur in the skin, subcutaneous tissues, and mucous membranes of the oral cavities and lips, as well as in the liver, spleen, and kidneys

Histologically, they are composed of thin-walled capillaries with scant stroma

Answer 7

Capillary hemangiomas

the most common type of hemangioma

Question 8

arise in the skin and grow rapidly for a few months, but then fade by 1 to 3 years of age and completely regress by age 7 in the vast majority of cases.

Answer 8

juvenile hemangioma

Question 10

composed of large, dilated vascular channels

mass is unencapsulated, has infiltrative borders, and is composed of large vascular spaces separated by connective tissue stroma

Answer 10

Cavernous hemangiomas

Question 11

one component of von Hippel-Lindau disease. what clinical complications are common with these neoplasia?

Answer 11

cavernous hemangiomas

Intravascular thrombosis and associated dystrophic calcification are common

Question 12

capillary hemangiomas that present as rapidly growing red pedunculated lesions on the skin, gingival, or oral mucosa. They bleed easily and are often ulcerated.

Answer 12

Pyogenic granulomas, of which Pregnancy tumor (granuloma gravidarum) is one form

Question 13

Answer 13

cavernous hemangioma

Question 14

Answer 14

either capillary hemangioma or a pyogenic granuloma (also a hemangioma)

Question 15

Answer 15

Pyogenic granuloma of the lip.

Question 16

1. slightly elevated
2. sometimes pedunculated lesions
3. up to 1 to 2 cm in diameter
4. occur predominantly in the head, neck, and axillary subcutaneous tissues.
5. Histologically: exhibit networks of endothelium-lined spaces that can be distinguished from capillary channels only by the absence of red cells.

Answer 16

Simple (capillary) lymphangiomas

Question 17

cystic hygromas

Answer 17

1. Cavernous lymphangiomas (cystic hygromas)
2. typically found in the neck or axilla of children, and more rarely in the retroperitoneum
3. can occasionally be enormous (up to 15 cm in diameter) and may fill the axilla or produce gross deformities about the neck. Of note, cavernous lymphangiomas of the neck are common in Turner syndrome

Question 18

lesions are composed of massively dilated lymphatic spaces lined by endothelial cells and separated by intervening connective tissue stroma containing lymphoid aggregates

Answer 18

Cavernous lymphangiomas (cystic hygromas)

Question 19

benign but exquisitely painful tumors arising from modified smooth muscle cells in structures involved in thermoregulation, rather than endothelial cells.

Answer 19

Glomus Tumor (Glomangioma).

Question 20

most commonly found in the distal portion of the digits, especially under the fingernails. Excision is curative.

Answer 20

Glomus Tumor (Glomangioma).

Question 21

a vascular proliferation in immunocompromised hosts (e.g., patients with AIDS) caused by opportunistic gram-negative bacilli

Answer 21

1. Bacillary angiomatosis
   
   1. vascular proliferation in immunocompromised hosts (e.g., patients with AIDS) caused by opportunistic gram-negative bacilli of the Bartonella family. Lesions can involve the skin, bone, brain, and other organs. Two species are implicated:
   2. Bartonella henselae
      
      1. principal reservoir is the domestic cat; this organism causes cat-scratch disease(a necrotizing granulomatous disorder of lymph nodes) in immunocompetent hosts.
   3. Bartonella quintana
      
      1. transmitted by human body lice; this microbe was the cause of “trench fever” in World War I.

Question 22

red papules and nodules, or rounded subcutaneous masses; histologically, there is capillary proliferation with prominent epithelioid endothelial cells exhibiting nuclear atypia and mitoses.

Lesions contain stromal neutrophils, nuclear dust, and the causal bacteria.

Answer 22

Bacillary Angiomatosis

Question 23

Answer 23

1. Bacillary Angiomatosis.
2. Bacillary angiomatosis is a vascular proliferation in immunocompromised hosts (e.g., patients with AIDS) caused by opportunistic gram-negative bacilli of the Bartonella family. Lesions can involve the skin, bone, brain, and other organs. Two species are implicated
   
   1. Bartonella henselae , whose principal reservoir is the domestic cat; this organism causes cat-scratch disease(a necrotizing granulomatous disorder of lymph nodes) in immunocompetent hosts
   2. Bartonella quintana , which is transmitted by human body lice; this microbe was the cause of “trench fever” in World War I.

Question 24

vascular proliferation results from induction of host hypoxia-inducible factor-1 (HIF-1), in turn drives vascular endothelial growth factor (VEGF) production

Answer 24

Bacillary Angiomatosis

Question 25

1. manifests as multiple red-purple skin plaques or nodules, usually in the distal lower extremities; these progressively increase in size and number and spread proximally.
2. typically asymptomatic and remain localized to the skin and subcutaneous tissue.

Answer 25

Classic KS is a disorder of older men of Mediterranean, Middle Eastern, or Eastern European descent (especially Ashkenazic Jews); it is uncommon in the United States. It can be associated with malignancy or altered immunity, but is not associated with HIV infection

Question 26

occurs in HIV-seronegative individuals younger than age 40

can follow an indolent or aggressive course

involves lymph nodes frequently tIn combination with AIDS-associated KS , now the most common tumor in central Africa

Answer 26

Endemic African KS

Question 27

all KS lesions are infected by

Answer 27

human herpesvirus 8 (HHV8) , also known as Kaposi sar­coma herpesvirus

γ-herpesvirus

Question 28

classic KC morphology

Answer 28

1. patches: red-purple macules typically confined to the distal lower extremities
   
   1. show only dilated irregular endothelial cell–lined vascular spaces: interspersed by lymphocytes, plasma cells, and macrophages (sometimes containing hemosiderin).
2. raised plaques: dermal accumulations of dilated, jagged vascular channels lined and surrounded by plump spindle cells
3. nodules
   
   1. ​ composed of sheets of plump, proliferating spindle cells, mostly in dermis or subcutaneous tissues
   2. encompass small vessels and slitlike spaces containing red cells.
   3. Marked
      
      1. hemorrhage
      2. hemosiderin pigment
      3. mononuclear inflammation are present
      4. mitotic figures are common
      5. round, pink, cytoplasmic globules representing degenerating red cells within phagolysosomes are common

Question 29

arise in the setting of lymphedema, classically in the ipsilateral upper extremity several years after radical mastectomy (i.e., with lymph node resection) for breast cancer; the tumor presumably arises from lymphatic vessels

Answer 29

Angiosarcoma

a malignant endothelial neoplasm that primarily affects older adults. There is equal gender predilection, and the tumor may occur at any site, but most often involves skin, soft tissue, breast, and liver.

can also arise in the setting of lymphedema, classically in the ipsilateral upper extremity several years after radical mastectomy (i.e., with lymph node resection) for breast cancer; the tumor presumably arises from lymphatic vessels (lymphangiosarcoma). Angiosarcoma has also been induced by radiation and are rarely associated with foreign material introduced into the body either iatrogenically or accidentally.