20 - 118: VASCULAR TUMORS

Question 1

most significant risk factor for development of infantile hemangioma (IH)

Answer 1

LBW <2500g

Question 2

Characteristic of infantile hemangiomas, except

a. presence at birth
b. presence of a nascent IH
c. presence of area of pallor
d. presence of telangiectasias

Answer 2

a. presence at birth – absence at birth

Question 3

S1 IH corresponds to anomalies in the

a. cerebral, cerebrovascular, ocular
b. less associated with extracutaneous features
c. airway involvement
d. cardiovascular abnormalities

Answer 3

a. cerebral, cerebrovascular, ocular

Question 4

most common extracutaneous features of PHACE

Answer 4

cardiac anomalies

Question 5

Areas most highly associated with PHACE

Answer 5

S3 – and S1

Question 6

area that carries 60% of symptomatic airway disease

Answer 6

S3

Question 7

Multifocal IH are considered if the patient presents with ___ or more IH

Answer 7

c. 6 p 2045

Question 8

MC complication of IH

a. GI bleeding
b. early onset vascular stroke
c. Ulceration
d. DVT

Answer 8

c. Ulceration

Question 9

rare complication in infants with diffuse infantile hemangiomas

a. Hypothyroidism
b. Hyperthyroidism
c. Hypercalcemia
d. Hypocalcemia

Answer 9

a. Hypothyroidism

Question 10

Alternative drug to propranolol that lacks bronchial reactivity

Answer 10

Atenolol

Question 11

most common tumor of infancy

Answer 11

Infantile hemangiomas

Question 12

most significant risk factor for IH development

Answer 12

Low birth weight

Question 13

characteristic features of IH

Answer 13

• ABSENT at birth or presence of a nascent IH often an area of pallor, telangiectasias or duskiness

Question 13

There is _____% risk increase of infantile hemangioma for every _______ g decrease in birth weight

Answer 13

40%
500g

Question 14

peak Infantile hemangioma growth period

Answer 14

5.5 and 7.5 weeks of age

Question 15

Most common location of IH

Answer 15

Head and neck
but can be found on any cutaneous or mucosal site

Question 16

Fill in the blanks.

Almost all IHs with a superficial component become apparent in the _______ month of life, and most will at least double in size within the first _______months of life.

Answer 16

• become apparent in the First month of life
• double in size within the first 2 months of life

Question 17

While 80% of growth has occurred by _____months of age, 80% of IHs have completed all growth by ______ months of age

Answer 17

• 80% of growth has occurred by 3 months of age
• 80% of IHs have completed all growth by 5 months of age

Question 18

The late proliferative stage of ongoing slower growth that occurs after peak rapid growth typically ends by _______ months of age

Answer 18

9 months

Question 19

Question 20

Evidence of involution, often referred to as ________

Answer 20

graying

• involves change to a dull red, then gray or milky-white color, followed by flattening and softening

Question 21

Evidence of involution, often referred to as graying, is usually apparent by what age?

Answer 21

usually apparent by 1 year of age

Question 22

More than 90% of IHs have completed involution by what age?

Answer 22

3.5 to 4 years of age

Question 23

True or False:

DEEP IHs are more likely to develop residual skin changes following involution compared with SUPERFICIAL IHs

Answer 23

FALSE

Superficial IHs are more likely to develop residual skin changes following involution compared with deep IHs

Question 24

A less-common subtype of IH is distinguished by its minimal-to-absent proliferation and telangiectatic surface

Answer 24

IH with minimal or abortive growth

Question 25

the presence of facial segmental IH is associated with risk of what?

Answer 25

PHACE (posterior fossa brain malformations, hemangiomas of the face, arterial anomalies, cardiac anomalies, and eye abnormalities)

Question 26

Segmental IHs on the lower body confer risk of what syndrome?

Answer 26

myelopathy and genitourinary anomalies in LUMBAR (lower body hemangioma and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies) syndrome

Question 27

term used to those that are not clearly identifiable as localized or segmental IH; some authors consider them to be “subsegmental”

Answer 27

Indeterminate IHs

Question 28

Multifocal (multiple localized) IHs are less common, with fewer than 3% of infants presenting with _________ or more IHs.

Answer 28

6

Question 29

This subtype of IHs are 11 times more likely to experience complications and 8 times more likely to receive treatment

Answer 29

segmental hemangiomas

Question 30

possible complications of infantile hemangioma

Answer 30

ulceration, severe bleeding, scarring, pain and infection

Question 31

High-risk Infantile Hemangioma Features

Answer 31

Question 32

Question 33

Enumerate the 4 segments of facial segmental IH

Answer 33

S1: frontotemporal
S2: maxillary
S3: mandibular
S4: frontonasal

Question 34

this segment of facial segmental IH is associated with cerebral, cerebrovascular, and ocular anomalies

Answer 34

S1: frontotemporal segment

Question 35

this segment of facial segmental IH is less-frequently associated with extracutaneous involvement

Answer 35

maxillary segment (S2)

Question 36

this segment of facial segmental IH is correlated with airway IH, ventral developmental defects, and cardiovascular abnormalities

Answer 36

mandibular segment (S3)

Question 37

this segment of facial segmental IH encompasses the high-risk territory of the nose, including the nasal tip, 30 and confers risk of cerebral and cerebrovascular involvement

Answer 37

frontonasal segment (S4)

Question 38

facial segments most highly associated with PHACE

Answer 38

frontotemporal (S1) and mandibular (S3) segments

Question 39

Question 40

most common extracutaneous features in PHACE

Answer 40

Congenital vascular anomalies

Question 41

segmental IHs on the lower body involving the perineum or lumbosacral area are risk factors for associated spinal, bony, and genitourinary anomalies

Answer 41

LUMBAR syndrome describes the constellation of lower body hemangioma and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies

Question 42

most commonly associated extracutaneous abnormality in LUMBAR syndrome presenting as tethered cord or lipomyelo(meningo)cele

Answer 42

Myelopathy

Question 43

most common ocular complication, occurring in 40% to 60% of infants with untreated periocular IH

Answer 43

Amblyopia

Question 44

IHs larger than ______in diameter confer greater risk of amblyopia, significantly lowering the threshold to initiate treatment.

Answer 44

1 cm

Question 45

The most favorable prognostic sign to herald normal vision following involution

Answer 45

absence of asymmetrical refractive error

Question 46

most common extracutaneous site for IH

Answer 46

Liver

Question 47

T/F.

Focal hepatic hemangiomas are not true IHs.

Multifocal and diffuse hepatic hemangiomas are true IHs.

Answer 47

True

Question 48

most common complication of IH

Answer 48

ulceration

• occurring in greater than 20% of infants in a referral setting, typically during the proliferative phase, with a median age of onset of 4 months

Question 49

sensitive marker of impending ulceration of IH

Answer 49

Early gray-white discoloration in patients younger than 3 months

Question 50

most reliable histologic marker of IH, which is expressed in all stages of IH

Answer 50

GLUT1, a red blood cell glucose transporter protein

Question 51

Question 52

only medication approved by the United States Food and Drug Administration (FDA) for the treatment of complicated IH

Answer 52

Propranolol