Chapter 188 - Pediatric vascular tumors

Question 1

Types of pediatric vascular tumor

Answer 1

1) infantile hemangioma 2) congenital hemangioma 3) kaposiform hemangioendothelioma 4) pyogenic granuloma

Question 2

Pediatric vascular tumor that can also be seen at any age

Answer 2

Pyogenic granuloma

Question 3

Pediatric vascular tumour that’s considered intermediate and not fully benign

Answer 3

KHE

Question 4

Differentiating vascular tumour from malformation

Answer 4

Tumours have proliferative endothelium

Question 5

Infantile hemangioma cell type

Answer 5

Progenitor cells that are supposed to develop into blood vessels Multipotent hemangioma-derived stem cells

Question 6

Likely triggers of infantile hemangioma

Answer 6

hypoxia –> endothelial proliferation

Question 7

Natural course of infantile hemangioma

Answer 7

Involute before age 4 tumour replaced by fat cells also derived from same stem cells

Question 8

Factors associated with infantile hemangioma

Answer 8

1) caucasian 2) premature low birth weight 3) female

Question 9

Most common location of lesions in infantile hemangioma

Answer 9

1) head & neck 2) trunk 3) extremity

Question 10

Infantile hemangioma that are solitary lesions (rate)

Answer 10

80%

Question 11

Appearance of infantile hemangioma tumour

Answer 11

if superficial dermis = bright red if deeper = bluish or normal

Question 12

Chance of visceral hemangioma if > 5 cutaneous

Answer 12

16%

Question 13

Most common visceral hemangioma location

Answer 13

liver

Question 14

Infantile hemangioma course

Answer 14

Growth - birth to 9 month; first 8 weeks rapid

Age 5 - 80% volume growth

Involution 1-4 years

Question 15

Disease associated with diffuse infantile hemangioma

Answer 15

1) replace liver parenchyma –> massive hepatomegaly
2) IVC compression
3) hypothyroidism (from deiodinase inactivation of thyroid hormone)

Question 16

Hypothyroidism complication in children

Answer 16

Mental retardation

Question 17

Segmental midline lumbosacral region infantile hemangioma associated with

Answer 17

33% spinal anomaly

1) tethered cord
2) lipoma
3) intraspinal hemangioma

Question 18

Lower body infantile hemangioma associated with

Answer 18

LUMBAR

1) urogenital anomalies or ulceration
2) myelopathy
3) bone deformities
4) anorectal malformation
5) arterial and renal anomalies

mostly girls

Question 19

Segmental plaquelike infantile hemangioma of the face associated with

Answer 19

PHACES syndrome

1) posterior fossa brain malformation (72%)
2) hemangioma
3) arterial anomalies
4) coarctation of aorta and cardiac defects
5) eye abnormalities
6) sternal clefting or supraumbilical raphe

Mostly female

Question 21

Risk of PHACE syndrome in all infantile hemangioma

Answer 21

3%

Question 22

Congenital hemangioma differentiate from infantile hemangioma

Answer 22

1) no post-natal growth
2) solitary usually 2-20 cm

Question 23

Classic lesion of congenital hemangioma

Answer 23

1) red violaceous color
2) course telangiectasia
3) central pallor
4) peripheral pale halo

Question 24

Congenital hemangioma distribution in body

Answer 24

mostly in extremity

Question 25

Gender distribution of congenital hemangioma

Answer 25

equal sex distribution

Question 26

Classification of congenital hemangioma based on involution

Answer 26

1) rapidly involuting
2) partially involuting
3) non-involuting

Question 27

Rapid involuting congenital hemangioma time frame for involution

Answer 27

Birth and most gone by 7-14 months

Question 28

Skin appearance after involution of congenital hemangioma

Answer 28

1) atrophic skin
2) lack underlying subcutaneous adipose tissue (unlike IH)

Question 29

Staining marker for infantile hemangioma

Answer 29

GLUT 1

Question 30

Noninvoluting congenital hemangioma typical location

Answer 30

Head/neck 43%

Extremity 38%

torso 19%

Question 31

Large rapidly involuting congenital hemangioma can cause

Answer 31

thrombocytopenia

Question 32

Characteristic of a kaposiform hemangioendothelioma

Answer 32

1) > 5cm in diameter +/- hair
2) 60% in neonatal and 90% by infancy
3) locally invasive but not metastatic
4) profound thrombocytopenia (Kasabach-Merritt)
5) reddish purple with ill-defined border

Question 33

Epidemiology of kaposiform hemangioendothelioma

Answer 33

Equal gender distribution

1/100000

Question 34

Distribution of kaposiform hemangioendothelioma

Answer 34

Head/neck 40%

Truck/extremity 30%

Question 35

Pyogenic granuloma characteristic

Answer 35

1) lobular capillary hemangioma
2) solitary red papule 6.5 mm
3) rapid growth with stalk
4) bleeds and ulcerates

Question 36

Epidemiology of pyogenic granuloma

Answer 36

Male:female 2:1

mean age onset 7 years

less likely at older ages

Question 37

Location of pyogenic granuloma in the skin

Answer 37

90% cutaneous

12% mucous membrane

Question 38

Causes of pyogenic granuloma

Answer 38

1) trauma
2) insect bite
3) viral infection
4) dermatologic disorder

Question 39

Distribution of pyogenic granuloma in body

Answer 39

Head/neck 62%

trunk 19%

UE 13%

LE 5%

Question 40

Ultrasound of infantile hemangioma

Answer 40

1) well-circumscribed hypervascular mass
2) low resistance arterial waveform
3) venous drainage

Question 41

MRI of infantile hemangioma in proliferative phase

Answer 41

1) parenchymal mass with dilated vessels and signal void
2) isointense on T1
3) hyperintense on T2
4) enhances homogeneously after admin of contrast

Question 42

MRI of involuting infantile hemangioma

Answer 42

1) lobular with adipose tissue and less vessels
2) signal void or enhancement

Question 43

Microscopic characteristics of infantile hemangioma in proliferative phase

Answer 43

1) tightly packed capillaries
2) plump endothelial cells
3) minimal intervascular stroma

Question 44

Microscopic characteristics of infantile hemangioma in involution stage

Answer 44

1) reduced capillaries
2) increased stroma
3) increased fibrofatty tissue

Question 45

MRI findings on KHE

Answer 45

1) infiltrative lesions
2) hypertense on T2
3) enhances with contrast
4) subcutaneous stranding
5) ill-defined boarders
6) prominent vessels with hemosiderin (signal voids)

Question 46

Histopathology of KHE

Answer 46

1) lobules of spindled lymphatic endothelial cells
2) glomeruloid appearance
3) vessels with thrombi and hemosiderin
4) tumor immuno stain for lymphatic markers D240 and PROX1

Question 47

Microscopic characteristics of pyogenic granuloma

Answer 47

1) exophytic mass with narrow stalk
2) normal number of mast cells (not like IH)
3) immature capillaries interspersed with fibroblastic tissue superficially
4) proliferating capillaries in lobular pattern deep

Question 48

Rate of skin ulceration in IH

Answer 48

16%

Question 49

Treatment of IH

Answer 49

1) conservative
2) topical antibiotic and vaseline
3) topical lidocaine
4) topical corticosteroid (clobestasol
5) timolol application
6) Propranolol if not improving (stops 90% of tumor growth)

Question 50

Contraindication to systemic beta blocker for treating IH

Answer 50

1) reactive airway disease
2) congenital heart disease
3) glucose abnormalities

Question 51

Systemic drugs for IH

Answer 51

1) beta blocker
2) steroid
3) interferon or vincristine (high side effect like spastic diplegia)

Question 52

Surgical treatment for IH

Answer 52

1) embolization
2) pulsed dye laser therapy
3) excision of lesion

Question 53

Managing of congenital hemangioma

Answer 53

1) self limiting if rapidly involuting
2) surgical resection if non-involuting

Question 54

Kasabach-Merritt phenomenon

Answer 54

Profound thrombocytopenia < 25

consumptive coagulopathy

life threatening in 12-24% of the time

Question 55

Goals of treating Kaposiform Hemangioendothelioma

Answer 55

1) prevent complication of Kasabach-Merritt
2) minimize fibrosis
3) reduce chronic pain
4) reduce stiffness and contractures

Question 56

Treatment for Kasabach-Merritt

Answer 56

1) corticosteroid (10% effective)
2) vincristine (90%)
3) interferon (50%)