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Adenoid Cystic Carcinoma of the Lacrimal Gland

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This next case is a patient who had a palpable abnormality,

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affecting the right lacrimal gland.

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It was painless.

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Looking at the thin section images through

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the orbits on this CT post-contrast,

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one sees a mass that is infiltrating the lacrimal fossa along the

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lateral aspect of the orbit, and one can see that it represents the

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lacrimal gland which is enlarged compared

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to the normal left lacrimal gland.

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In looking at this lesion,

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I would again be concerned if I were to find any

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abnormality that suggested perineural spread.

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Here we demonstrate the pterygopalatine

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fossa on the right side with the fat.

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Let me move that just a little bit further anterior for you.

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Here we have the pterygopalatine fossa which is normal in

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appearance, with fat and the ganglion within it, as well as,

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as you can see, some small vessels.

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So there is no perineural spread in this case.

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So what is our differential diagnosis

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for lacrimal gland enlargement?

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In addition to the inflammatory conditions, which we mentioned

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previously, which include sarcoidosis, orbital pseudotumor,

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also known as orbital inflammatory syndrome,

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or idiopathic orbital inflammation,

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we might consider IgG4 related ophthalmic disease.

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Since IgG four rod

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can affect the lacrimal gland as its most common manifestation,

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this is usually found as enlargement of the lacrimal

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gland with dacrioadenitis.

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However, IgG4 disease is bilateral in 50% of cases.

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Remember that IgG4 disease is usually a systemic disease that

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can be associated with retroperitoneal fibrosis

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and other abdominal abnormalities,

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as well as sclerosing, cholangitis, and pancreatic sclerosis.

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After considering the inflammatory and granulomas disease,

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one should consider neoplasms that affect the lacrimal gland.

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As mentioned previously,

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the lacrimal gland has pathology that simulates salivary glands.

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The most common benign tumor of the lacrimal gland, and the most

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common benign tumor of the salivary

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gland is a pleomorphic adenoma.

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Pleomorphic adenomas may subsequently degenerate

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into adenocarcinomas if they are not treated.

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The most common malignancy affecting the lacrimal gland, as

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well as the salivary glands, is adenoid cystic carcinoma.

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This tumor looked relatively well-defined.

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And I was scrolling through this thinking, well,

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this might just be a pleomorphic adenoma.

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However, there was one area that concerned me,

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and that was at this border with the top of the orbit where it

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looked like there may be some element of bony erosion here.

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Unfortunately, on the contralateral side,

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there also looked to be an area where there was a dehiscence.

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So, let's go and check out the coronal imaging.

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As we scroll through the coronal images,

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we see that this is a well-defined mass that is affecting the

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lacrimal gland and may be slightly displacing the globe medially.

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If you look at the distance

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from the edge of the orbit from side to side,

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you notice that the globe does look like

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it's slightly medially displaced.

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This area, superiorly, in the orbit where there appears to be

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enhancing tissue, does look like, even on the coronal image,

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it's symmetric with the contralateral side.

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However,

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this infiltration of the periorbita, superiorly, bothered me

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sufficiently that I suggested this could represent a malignancy.

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And of the malignancies, adenoid cystic carcinoma is most common.

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There was no evidence of perineural spread of tumor.

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This tumor was taken out and did represent adenoid

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cystic carcinoma of the lacrimal gland.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Orbit

Neuroradiology

Neuro

Neoplastic

Head and Neck

CT

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