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Paraganglioma Summary

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We talked about the retrotympanic white mass in

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referring to the cholesteatoma. However,

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there is a differential diagnosis that encompasses

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different things when we have a retrotympanic red or

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vascular mass. Here are the things to consider.

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For a retrotympanic red mass,

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we worry about the vascular anomalies,

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such as an aberrant internal carotid

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artery or a persistent artery.

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We worry about another vascular abnormality,

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which is the dehiscent jugular bulb with or without

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a diverticulum. But we also consider the tumors,

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including the glomus tympanicum and the glomus jugulotympanicum,

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and we'll differentiate between the two.

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There is another entity known as the hemangioma,

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and although some people would consider

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that to be a neoplasm,

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most people feel that it really represents a venous

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vascular malformation. We'll take a look at all of these.

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Here we have a patient who had a

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retrotympanic vascular mass.

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And what one sees is a soft tissue mass which is present

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over the cochlear promontory in the middle ear cavity.

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This is the axial scan,

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and this is the coronal scan with a marker placed,

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as you can see, right along the mass.

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And you can also see the pars tensa

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of the tympanic membrane.

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So this is a red mass which is seen at otoscopy

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through the tympanic membrane.

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And this is a typical location along the cochlear

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promontory of a glomus tympanicum.

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This is the round window niche.

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This is the sinus tympani.

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This is the pyramidal eminence,

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and this is the facial nerve recess

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to orient you to the anatomy.

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The glomus tympanicum is said to arise from the tympanic

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plexus, or Jacobson's nerve of cranial nerve nine.

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This is another patient who had a

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retrotympanic vascular mass.

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And we're seeing a post gadolinium enhanced

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MRI scan in the center here.

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What you see in this case is a much larger mass,

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which is at the skull base.

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We're actually down at the jugular foramen this mass has

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grown from the jugular foramen upward

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into the middle ear cavity here's.

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The ear and the tympanic membrane would be here,

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and there was a retro tympanic vascular mass.

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This is a glomus jugulotympanicum.

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By jugulotympanicum,

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we suggest that it has components which

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are both at the jugular foramen,

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where you would normally have a glomus jugulare,

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which in general grows more inferiorly into the skull base.

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Base region and you have that component which

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is extending into the middle ear,

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which is the tympanic portion of

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the glomus jugulotympanicum.

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So this is a nice diagram that was provided to demonstrate

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the skull base portion with the larger mulberries

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representing the glomus jugulare portion and the portion

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that's ending into the tympanic membrane into the

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middle ear cavity. Again, retrotympanic red mass.

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This would be the tympanic portion of

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a glomus jugulotympanicum.

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This is another diagram showing the involvement of the

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jugular foramen by the glomus jugulare and then the

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extension that goes into the middle ear

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for the jugulotympanicum portion.

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I want to just remind you that these are paragangliomas

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and the paragangliomas can occur in four places,

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typically in the head and neck.

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They include the middle ear cavity with the glomus

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tympanicum, the jugular foramen with the glomus jugulare,

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and the upper skull base in the carotid sheath for the

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glomus vagale and at the carotid bifurcation

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for the carotid body tumor.

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These are the classic four locations of paragangliomas

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in the neck. Interestingly enough,

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there is a high incidence of around ten to 15% of multiple

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paragangliomas and for that reason high resolution imaging

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in the neck and looking bilaterally and at these multiple

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sites is important when you have a patient

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who has one paraganglioma.

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So they are hereditary in 7% of cases, multiple, as I said,

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in around 10% of the sporadic,

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but 30% to 40% of the hereditary cases.

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And there is a small instance of recurrence

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or residual tumor after surgery.

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Remember, these are highly vascular lesions and therefore we usually

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don't do fine needle aspirations or biopsies,

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but instead the lesion is removed

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in its entirety at surgery.

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There is values for somatostatin receptor scintigraphy

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which allows you to identify multiple paragangliomas.

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Remember that they can occur elsewhere in the body,

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including in the abdominal and pelvic cavities as well as

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in the chest and rarely we see them

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elsewhere in the head and neck.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Temporal bone

Neuroradiology

Neoplastic

MRI

Head and Neck

Brain

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