Chapter 17 - Vascular Tumors

Question 1

Most common h/n paraganglioma, and presentation of it

Answer 1

Carotid body tumor

Question 2

Percentage of paragangliomas that are familial

Answer 2

10-28%

Question 3

Percentage of paragangliomas that are multicentric

Answer 3

30-40% familial

10% sporadic

Question 4

How paragangliomas are passed along

Answer 4

AD, imprinting
Gene that mother passes down is shut down
Females can inherit the trait, but cannot pass it on
50% of heterogenous male’s offspring will develop paragangliomas

Question 5

Color of nasal polyps vs JNA

Answer 5

Polyps: Pale
JNA: Bluish

Question 6

What are paraganglia

Answer 6

Paraganglia are in vascular adventitia and neurons, paraganglioma can form where they are NCC origin
Paraganglia release catecholamines, neurotransmitters
Most paraganglia degenerate after birth, many along ANS persist

Question 7

Where are paragangliomas most commonly found?

Answer 7

Adrenal medulla 90%
Abdomen 8.5%
Thorax 1.2%
h/n 0.3%

Question 8

Head and neck locations other than carotid body where paragangliomas can be found

Answer 8

Carotid body
Jugular
Promontory middle ear
Vagal
Larynx, thyroid, sinus

Question 9

Presentation of paragangliomas

Answer 9

Incidental on imaging (splay ICA/ECA)
Carotid: pulsatile mass, mobile horizontally
Tymp: bluish mass behind eardrum, pulsatile tinnitus, CHL
Usually non-functional, but may have flush/heat intol/sweat/palp

Question 10

Growth rate of paragangliomas

Answer 10

0.5cm per year

Question 11

Where jugular paragangliomas originate

Answer 11

Bulb, places IX, X, XI at risk

Question 12

Workup of functional paraganglioma

Answer 12

Test urine metanephrines, VMA, serum catecholamines
Only 1-3% h/n paragangliomas are functional
CN exam

Question 13

Imaging for paraganglioma

Answer 13

U/S to diff between LN
CT con or MRI gad (flow voids)
Angiography for preop embolization (see splaying ICA/ECA)

Question 14

Genes involved with paraganglioma

Answer 14

PGL1 - succinate dehydrogenase

SDHB

Question 15

Treatment options for paraganglioma

Answer 15

Surgery, observation, radiation

Question 16

Rate of malignancy of h/n paragangliomas

Answer 16

Low, only way to tell is if LN/distant mets

Vagal 16%, carotid body 6%, jug/tymp 4%

Question 17

Jugular paraganglioma treatment considerations

Answer 17

If pt younger, CN already damaged, compensation has likely occurred, do better after surgery
Consider radiation for all pt, especially those with NL CNs, since surgery can render them non-functional

Question 18

Radiation success and dose for paragangliomas

Answer 18

90% control rate

45 Gy

Question 19

JNA

Answer 19

Male adolescent (hormone responsive)
locally invasive, can go intracranial
CT w/ contrast to evaluate
Only tx with radiation if unresectable
Regress once reach adulthood if incompletely excised, but recur during adolescence

Question 20

Female to male ratio for infantile hemangiomas

Answer 20

3:1

Question 21

Kasabach Merritt

Answer 21

Large facial hemangioma, pt devvelops coagulopathy
Platelet trapping in tumor, DIC-like syndrome
Tx cryo, fibrinogen, FFP, platelets, etc

Question 22

How many kids with subglottic hemangiomas also have cutaneous lesions

Answer 22

50%

Question 23

Sturge Weber

Answer 23

Port wine stain V1-2
Cerebral leptomeninges angioma
Need neuro, ophtho

Question 24

How to treat cutaneous vascular lesions with laser

Answer 24

Pulsed dye laser great for port wine stain

Also argon, KTP which target hemoglobin