0:01

I previously showed an example of bilateral carotid

0:05

body tumors and that entity of multiple

0:10

paragangliomas may suggest that there is a

0:13

hereditary predisposition for paragangliomas.

0:16

I've listed here some of the entities that are

0:19

associated with hereditary multiple paragangliomas

0:23

and they include this MEN type two syndrome,

0:27

this pheochromocytoma genotype that's autosomal dominant,

0:32

these succinate dehydrogenase subunit D,

0:37

SDHD related hereditary paragangliomas

0:41

and pheochromocytomas.

0:43

And all these can lead to multiple and multifocal

0:47

head and neck paragangliomas,

0:50

sympathetic paragangliomas pheochromocytomas.

0:54

These are usually benign tumors.

0:56

They have a low potential for metastases.

0:58

This begs the question how do we evaluate for these

1:01

small lesions that may be scattered throughout

1:04

the body? And as I mentioned,

1:06

sometimes you may use this octreotide scan.

1:09

But the other thing that has gained favor is this

1:12

DOTATATE agent which can be used for the treatment

1:17

of multiple paragangliomas or large paragangliomas

1:21

rather than going with surgical treatment.

1:24

So when we have one of these Shamblin type three

1:28

tumors where the risk is of taking

1:30

the internal carotid artery,

1:32

you may instead make the choice of going with the

1:37

nuclear medicine DOTATATE therapeutic agent.

1:40

And this is where we're getting into theranostics

1:44

with nuclear medicine. Unfortunately,

1:46

there is some risk associated with these agents and

1:49

that is that you can have a catecholamine

1:51

crisis or tumor lysis syndrome.

1:54

I should mention that paragangliomas may have

1:58

hormonal agent vasoactive hormones

2:02

that they can secrete,

2:04

which could potentially lead to elevations in the

2:07

blood pressure during the time that

2:09

they are being operated on.

2:12

The rate at which these tumors secrete

2:15

vasoactive chemicals is around 5%.

2:19

So it's a little bit of an overstated risk,

2:21

but it is there and the surgeons and the

2:24

anesthesiologists have to be aware of that.

2:27

When they are going after the paraganglioma,

2:31

sometimes they will do the metanephrine compound

2:35

screening with blood serum analysis in preparation

2:40

for the surgery, particularly if it's a large tumor.