Vascular anomaly

Question 1

4 big vascular tumors

Answer 1

infantile hemagioma
congenital hemagioma
Kaposiform hemangioendothelioma (KHE)
Angiosarcoma

Question 2

compare infantile and congenital hemangioma

Answer 2

infantile: not present at birth, involutes by 3-5y, no coagulopathy, GLUT-1 positive, propranolol

Congenital: present at birth, involutes by 18 mo, transient coagulopathy, GLU-1 neg, no therapy

Question 3

angiosarcoma features

Answer 3

aggressive
20% GLUT-1 positive
directed therapy

Question 4

Kaposiform Hemangioendothelioma

Answer 4

newborn
rapid size increase
Kasabach Merritt phenomenon
Tx: sirolimus, VCR, CS

Question 5

malignant vascular tumors

Answer 5

Epithelioid hemangioendothelioma
angiosarcoma

Question 6

locally aggressive vascular tumors

Answer 6

kaposiform hemangioendothelioma
retiform hemangioentothelioma
papilary intralymphatic angioendothelioma
Kaposi sarcoma

Question 7

Benign vascular tumors

Answer 7

infantile/congenital hemangioma
tufted angioma
spindle cell hemangioma
pyogenic granuloma

Question 8

PHACES syndrome acronym and sites

Answer 8

posterior fossa
hemangioma
arterial (CNS)
cardiac anom (coarc)
eye
sternal

multiple cutaneous hemangiomas: >5 are associated with internal hemangiomas

liver hemangiomas
- focal usually congenital
- multifocal usually infantile

Question 9

Infantile hemangioma timeline

Answer 9

not present at birth
proliferate 4-6mo
stability
involute by 3-5y

Question 10

infantile hemangioma US findings

Answer 10

well defined mass
high flow vessel during proliferative phase
low resistive arterial flow

Question 11

infantile hemangioma tx and s/e

Answer 11

propranolol
- sleep change, irritability, GI, hypotension
- contraind: neonate <4wo, reactive airway disease, AV block, hypoglycemia

Question 12

Epithelioid hemangioendothelioma features, path, tx

Answer 12

liver, bone, soft tissue, skin

WWTR1-CAMTA1 gene fusion

observe, resect, sirolimus, MEK inhibitor

Question 13

Kasabach Merritt Syndrome features, what tumors, tx

Answer 13

consumptive coagulopathy
severe thrombocytopenia
+- MAHA

seen in kaposiform hemangioendothelioma, tufted angioma

sirolimus
CS
VCR

Question 14

what is localized intravascular coagulopathy

Answer 14

venous malformation
high D dimer, low fibrinogen, low/N plt

Question 15

lymphatic anomalies somatic mutations

Answer 15

PIK3CA
BRAF

Question 16

PIK3CA related overgrowth syndrome (PROS)

Answer 16

• CLOVES: congenital lipomatous overgrowth with vascular anomalies, epidermal nevi, spinal issues
• Klippel-Tranaunay: large malformation affecting one lower quadrant

Tx: Alpelisib