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Adenoid Cystic Carcinoma – Summary

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Adenoid cystic carcinoma is another of the

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tumors that sort of fulfills that adage

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I said previously that malignancies are not

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as malignant as typical malignant tumors.

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Adenoid cystic carcinoma's prognosis

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is generally termed in decades.

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So the long-term survival is high,

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but the tumor will persist in the body for

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a long period of time.

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In other words,

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it's a tumor that you can live with for a long

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period of time but hard to get rid of.

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And that is because of the high

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rate of perineural spread.

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We say that adenoid cystic carcinoma has

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a 50% to 60% rate of perineural spread.

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And in the parotid gland,

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that means we got to be careful about the 7th

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cranial nerve and the third division

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of the fifth cranial nerve,

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the mandibular nerve in the region of the floor of

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the mouth with the submandibular gland

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and the sublingual gland. We

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worry about the 7th cranial nerve,

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the 12th cranial nerve, the 9th cranial nerve.

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And in the minor salivary glands,

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it usually is affecting the second division of

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the fifth cranial nerve, the maxillary nerve.

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It accounts for about 4% of all salivary gland tumors,

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but a higher rate of the malignant salivary gland

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tumors, particularly in the minor salivary gland tissue.

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As you can tell,

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there is a higher rate of distant metastases and

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nodal metastases with adenoid cystic

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carcinoma than, for example,

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our low-grade mucoepidermoid carcinoma.

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Here is a...

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the two cases I showed previously where a

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patient has a mass in the parotid gland and has

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perineural spread up the third division of the

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fifth cranial nerve through the foramen ovale

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to come to the Meckel's Cave region,

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where the Gasserian ganglion,

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the trigeminal ganglion, resides.

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And here you see another patient with perineural

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spread up the 7th cranial nerve through the

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stylomastoid foramen from adenoid cystic carcinoma.

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This is an example of a patient who presented 20

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years after the initial diagnosis of adenoid

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cystic carcinoma of the hard palate.

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You notice on this coronal scan that the hard

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palate on the left side has

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been surgically removed.

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However, when we look at the coronal image,

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we see this large mass

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which extends through

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the base of the skull at the orbit,

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the base of the orbit, and is infiltrating the orbit.

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Here's the normal optic nerve,

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and extraocular muscles, and retrobulbar fat.

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Here we have an orbit that is grossly

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infiltrated by tumor.

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And you can see that it goes through the orbital

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roof and is abutting the frontal lobe of the left

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anterior cranial fossa. How did this happen?

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This tumor, although it was resected 20 years earlier,

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had gotten into the nerves.

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The second division of the fifth cranial nerve.

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Here is our demonstration of the pterygopalatine

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fossa and the pterygopalatine ganglion.

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You notice that you have the branches of the

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greater and lesser palatine foramina that are

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innervating the teeth as well as the hard palate.

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This tumor went up those nerves and then proceeded

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superiorly to extend into the inferior orbital

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fissure to get into the orbit.

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So again,

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this is along the greater and lesser palatine

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foramina, up the pterygopalatine fossa,

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to the pterygopalatine palatine ganglion.

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And right here is where it enters through the

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inferior orbital fissure to get into

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the orbit and infiltrate it.

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This is perineural spread of adenoid cystic

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carcinoma, 20 years after the initial diagnosis.

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Fascinating.

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Here is a patient who had a sublingual gland mass.

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And although it looks for all the world like a

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well-defined tumor and bright on

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T2-weighted scan, surprised us,

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this ended up being an adenoid cystic

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carcinoma of the sublingual gland.

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And remember that in the sublingual

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gland and submandibular gland,

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the rate of malignancy is about 50:50.

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And of those malignancies, in those glands,

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they are dominated by adenoid cystic carcinoma.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Salivary Glands

Orbit

Oral Cavity/Oropharynx

Neuroradiology

Neoplastic

MRI

Head and Neck

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