28- Benign/Malignant Vascular Neoplasms Flashcards
Most common benign tumor of childhood
Infantile (strawberry) hemangioma
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
Infantile hemangioma
Preliminary screening for infantile hemangioma that may include visceral lesions
Stool guaiac test
Liver imaging
CD133 stem cells within the hemangioma differentiate into mature blood vessels that express this type of glucose transporter
GLUT1
Indications for pharma intervention in infantile hemangioma
Hemorrhage Thrombocytopenia Cardio compromise Obstruction Skin ulceration Threatened vital function- respi, feeding, limbs, vision, passage of urine/stool
Pharma Treatment for infantile hemangioma
Beta blockers
Oral prednisone 2-4mg/kg/day
Others: topical imiquimod, lasers
Round slightly elevated ruby red papules most common in trunk
Seen in adults
Early lesions may mimic petechiae
Cherry angiomas (senile angioma, de Morgan spots)
Central brown/violaceous papule surrounded by ecchymotic halo
Represent trauma to preexisting hemangioma
Targetoid hemosideritic hemangioma
Glumeruloid hemangioma has been associated with these diseases:
-small firm violaceous papules in trunk and proximal extremities
POEMS syndrome
Castleman disease
ITP
Sjögren
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
Tufted angioma (angioblastoma)
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
Kaposiform hemangioendothelioma
Skin colored or slightly dusky blue, firm nodule
Extremely tender
Usually subungual in location, digital lesions more common in women
Glomus tumor
Glomus tumor is usually associated with this disease
Neurofibromatosis
Glomus cells in histo resemble
Strings of black pearls
Treatment of solitary glomus tumors
Excision
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
Spindle cell hemangioma
Dermal nodule with dilated vascuar spaces with fascicles of spindle cells in between
Solitary slow growing papule or nodule on distal extremity
Behaves as low grade malignancy
Occurs in males less than 25 years old
Wide excision
Epitheloid hemangioendothelioma
Low grade malignancy that presents as slow growing exophytic mass, dermal plaque, or subcutaneous nodule on upper or lower extremity of young adults
Retiform hemangioendothelioma
This virus has been found in retiform hemangioendothelioma
HHV 8
Treatment for retiform hemangioendothelioma
Wide excision
5 Types of Kaposi sarcoma
Classic African cutaneous African lymphadenopathic AIDS associated Lymphoma/immunosuppressive therapy
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
Classic
What type of Kaposi sarcoma?
Nodular infiltrating vascular masses on extremities
Men 20-50 years old
Locally aggressive
African cutaneous
What type of Kaposi sarcoma?
Lymph node involvement with or without skin lesions
Children under 10 years old
Aggressive course fatal withjn 2 years
African lymphadenopathic
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
AIDS- associated
Most frequent site of internal involvement in classic Kaposi sarcoma
GIT (small intestine)
Orher characteristic internal involvement in classic KS
Skeletal changes
Bone involvement- widespread disease
Most common lymph node involved in african lymphadenopathic KS
Cervical
Most often internal organs involved in AIDS associated KS
GIT
Lymph nodes
Lungs
Immunosuppression associated KS is usually due to
Oral prednisone
Chronic immunosuppression
Transplant patients
KS is usually with increased risk of developing second malignancies such as
Lymphoma
Leukemia
Myeloma
Virus associated with KS
HHV8
Treatment for KS
Radiation therapy
Localized lesions- excision, cryotherapy, alitretinoin gel, IFN, laser
Vincristine 0.1mg/ml- involutes tumore x8mos
Systemic therapy for KS indicated when
More than 10 new lesions in 1 month
Symptomatic lymphedema, pulmo dse, visceral involvement
Treatment for HIV KS
Protease inhibitors
Overall improvement of immune function
1st and 2nd line monotherapy antiretrovirals for advanced KS
Liposomal anthracycline
Paclitaxel
4 clinical settings of angiosarcoma
- Head and neck of elderly
- Chronic lymphedematous area ( after mastectomy-upper arm)
- Previously irradiated sites
- Small subset- others
