28- Benign/Malignant Vascular Neoplasms Flashcards

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1
Q

Most common benign tumor of childhood

A

Infantile (strawberry) hemangioma

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2
Q

Dome shaped lesion, dull to bright red, sharp borders, soft and easily compressed
60% on head and neck

Tend to grow and usually involutes by 7 years old

A

Infantile hemangioma

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3
Q

Preliminary screening for infantile hemangioma that may include visceral lesions

A

Stool guaiac test

Liver imaging

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4
Q

CD133 stem cells within the hemangioma differentiate into mature blood vessels that express this type of glucose transporter

A

GLUT1

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5
Q

Indications for pharma intervention in infantile hemangioma

A
Hemorrhage
Thrombocytopenia 
Cardio compromise
Obstruction
Skin ulceration
Threatened vital function- respi, feeding, limbs, vision, passage of urine/stool
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6
Q

Pharma Treatment for infantile hemangioma

A

Beta blockers
Oral prednisone 2-4mg/kg/day

Others: topical imiquimod, lasers

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7
Q

Round slightly elevated ruby red papules most common in trunk

Seen in adults
Early lesions may mimic petechiae

A

Cherry angiomas (senile angioma, de Morgan spots)

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8
Q

Central brown/violaceous papule surrounded by ecchymotic halo

Represent trauma to preexisting hemangioma

A

Targetoid hemosideritic hemangioma

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9
Q

Glumeruloid hemangioma has been associated with these diseases:

-small firm violaceous papules in trunk and proximal extremities

A

POEMS syndrome
Castleman disease
ITP
Sjögren

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10
Q

Poorly defined dull red macules with mottled appearance and associated hypertrichosis

Histo: cannonball pattern (angiomatous lobules scattered in dermis)

Occasional spontaneous regression
Tx: low dose aspirin, laser, steroids, radiation

A

Tufted angioma (angioblastoma)

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11
Q

Frequently occurs in retroperitoneum
Cutaneous variant may be associated with lymphangiomatosis
Locally aggressive
Prognosis based on depth and location
Combined features of cellular infantile hemangioma and Kaposi sarcoma

A

Kaposiform hemangioendothelioma

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12
Q

Skin colored or slightly dusky blue, firm nodule
Extremely tender
Usually subungual in location, digital lesions more common in women

A

Glomus tumor

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13
Q

Glomus tumor is usually associated with this disease

A

Neurofibromatosis

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14
Q

Glomus cells in histo resemble

A

Strings of black pearls

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15
Q

Treatment of solitary glomus tumors

A

Excision

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16
Q

Vascular tumor that presents in child/young adult who develops blue nodules of firm consistency on a distal extremity

Represent benign vascular proliferation in response to traumatoca larger vessel

A

Spindle cell hemangioma

Dermal nodule with dilated vascuar spaces with fascicles of spindle cells in between

17
Q

Solitary slow growing papule or nodule on distal extremity
Behaves as low grade malignancy

Occurs in males less than 25 years old

Wide excision

A

Epitheloid hemangioendothelioma

18
Q

Low grade malignancy that presents as slow growing exophytic mass, dermal plaque, or subcutaneous nodule on upper or lower extremity of young adults

A

Retiform hemangioendothelioma

19
Q

This virus has been found in retiform hemangioendothelioma

A

HHV 8

20
Q

Treatment for retiform hemangioendothelioma

A

Wide excision

21
Q

5 Types of Kaposi sarcoma

A
Classic
African cutaneous 
African lymphadenopathic
AIDS associated 
Lymphoma/immunosuppressive therapy
22
Q

What type of Kaposi sarcoma?

Reddish, violaceous or bluish black macules and patches that coalesce
Rubbery consistency, toes and soles
Slowly progressive
Enlargement of extremity due to lymphedema
Involute- atrophic/hyperpigmented scar

A

Classic

23
Q

What type of Kaposi sarcoma?

Nodular infiltrating vascular masses on extremities
Men 20-50 years old
Locally aggressive

A

African cutaneous

24
Q

What type of Kaposi sarcoma?

Lymph node involvement with or without skin lesions

Children under 10 years old
Aggressive course fatal withjn 2 years

A

African lymphadenopathic

25
Q

What type of Kaposi sarcoma?

One or several red to purple macules progressing rapidly to papules nodules and plaques

Predilection for head neck trunk and mucous membranes
Fulminant progressive course with nodal and systemic involvement

A

AIDS- associated

26
Q

Most frequent site of internal involvement in classic Kaposi sarcoma

A

GIT (small intestine)

27
Q

Orher characteristic internal involvement in classic KS

A

Skeletal changes

Bone involvement- widespread disease

28
Q

Most common lymph node involved in african lymphadenopathic KS

A

Cervical

29
Q

Most often internal organs involved in AIDS associated KS

A

GIT
Lymph nodes
Lungs

30
Q

Immunosuppression associated KS is usually due to

A

Oral prednisone
Chronic immunosuppression
Transplant patients

31
Q

KS is usually with increased risk of developing second malignancies such as

A

Lymphoma
Leukemia
Myeloma

32
Q

Virus associated with KS

A

HHV8

33
Q

Treatment for KS

A

Radiation therapy
Localized lesions- excision, cryotherapy, alitretinoin gel, IFN, laser

Vincristine 0.1mg/ml- involutes tumore x8mos

34
Q

Systemic therapy for KS indicated when

A

More than 10 new lesions in 1 month

Symptomatic lymphedema, pulmo dse, visceral involvement

35
Q

Treatment for HIV KS

A

Protease inhibitors

Overall improvement of immune function

36
Q

1st and 2nd line monotherapy antiretrovirals for advanced KS

A

Liposomal anthracycline

Paclitaxel

37
Q

4 clinical settings of angiosarcoma

A
  1. Head and neck of elderly
  2. Chronic lymphedematous area ( after mastectomy-upper arm)
  3. Previously irradiated sites
  4. Small subset- others