Chapter 17 - Vascular Tumors Flashcards
Most common h/n paraganglioma, and presentation of it
Carotid body tumor
Percentage of paragangliomas that are familial
10-28%
Percentage of paragangliomas that are multicentric
30-40% familial
10% sporadic
How paragangliomas are passed along
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
Color of nasal polyps vs JNA
Polyps: Pale
JNA: Bluish
What are paraganglia
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
Where are paragangliomas most commonly found?
Adrenal medulla 90%
Abdomen 8.5%
Thorax 1.2%
h/n 0.3%
Head and neck locations other than carotid body where paragangliomas can be found
Carotid body Jugular Promontory middle ear Vagal Larynx, thyroid, sinus
Presentation of paragangliomas
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
Growth rate of paragangliomas
0.5cm per year
Where jugular paragangliomas originate
Bulb, places IX, X, XI at risk
Workup of functional paraganglioma
Test urine metanephrines, VMA, serum catecholamines
Only 1-3% h/n paragangliomas are functional
CN exam
Imaging for paraganglioma
U/S to diff between LN
CT con or MRI gad (flow voids)
Angiography for preop embolization (see splaying ICA/ECA)
Genes involved with paraganglioma
PGL1 - succinate dehydrogenase
SDHB
Treatment options for paraganglioma
Surgery, observation, radiation
Rate of malignancy of h/n paragangliomas
Low, only way to tell is if LN/distant mets
Vagal 16%, carotid body 6%, jug/tymp 4%
Jugular paraganglioma treatment considerations
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
Radiation success and dose for paragangliomas
90% control rate
45 Gy
JNA
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
Female to male ratio for infantile hemangiomas
3:1
Kasabach Merritt
Large facial hemangioma, pt devvelops coagulopathy
Platelet trapping in tumor, DIC-like syndrome
Tx cryo, fibrinogen, FFP, platelets, etc
How many kids with subglottic hemangiomas also have cutaneous lesions
50%
Sturge Weber
Port wine stain V1-2
Cerebral leptomeninges angioma
Need neuro, ophtho
How to treat cutaneous vascular lesions with laser
Pulsed dye laser great for port wine stain
Also argon, KTP which target hemoglobin
