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Residents’ Section • Pat tern of the Month

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Gao et al.
Lacrimal Gland Masses

Residents’ Section
Pattern of the Month

Lacrimal Gland Masses


Yiming Gao1

L
acrimal gland lesions generally tory processes tend to have a diffuse pattern,
Gul Moonis1 present as palpable masses in the typically involving both the orbital and palpe-
Mary E. Cunnane2 superolateral aspects of the or- bral lobes of the gland. The palpebral lobe is
Ronald L. Eisenberg1 bits. Approximately 50% of lac- the portion of the lacrimal gland that is usu-
rimal gland masses are inflammatory le- ally visible when the upper eyelid is everted
Gao Y, Moonis G, Cunnane ME, Eisenberg RL sions, 25% are lymphoid lesions or lymphoma, on physical examination. The lacrimal gland
and the other 25% are salivary gland type tu- normally measures approximately 20 × 12 × 5
mors. Although there are overlaps and ex- mm. Although the size of a normal lacrimal
ceptions, features such as laterality, portion gland may vary from person to person, the
of gland involvement, presence or absence of glands are usually symmetric, and asymme-
bony findings, enhancement pattern, and try is an important indicator of abnormality.
clinical presentation are valuable in differen- On CT, the lacrimal gland is isodense to the
tiating among lacrimal gland lesions (Table muscle. The medial border is outlined by or-
1). In general, epithelial neoplasms predomi- bital fat and the lateral border by orbital bone
nantly involve the orbital lobe and are unilat- (Fig. 1C). Calcifications and bony changes are
eral. Lymphoproliferative and inflammatory well seen on CT (Fig. 2), and normal glands
lesions tend to involve both the orbital and show symmetric contrast enhancement. The
palpebral lobes. Bony scalloping may be superior resolution of MRI permits better as-
seen with lymphoproliferative lesions, but sessment of the extent of glandular and peri-
most benign epithelial neoplasms or inflam- glandular involvement. Normal lacrimal
matory lesions do not cause significant bone glands have intermediate (sometimes hetero-
change. Malignant neoplasms may result in geneous) signal on both T1-weighted and T2-
bone destruction or erosion although this is weighted imaging and enhance symmetrical-
not a common finding in our experience. As ly after gadolinium administration (Fig. 2).
a rule, if clinical presentation is atypical, im-
Keywords: lacrimal gland, lacrimal gland masses aging findings must be correlated with tissue Epithelial Neoplasms
diagnosis to definitely exclude malignancy. Primary lacrimal gland tumors arising
DOI:10.2214/AJR.12.9553
from epithelial cells are relatively com-
Received June 26, 2012; accepted after revision Normal Lacrimal Gland mon, accounting for 20% of all solid lac-
December 16, 2012. The lacrimal gland is an almond-shaped, rimal gland masses. They typically present
eccrine secretory gland for tear production. as unilateral lacrimal gland enlargement
1
Department of Radiology, Beth Israel Deaconess It is located in the superolateral aspect of the with inferomedial displacement of the eye.
Medical Center, Harvard Medical School, 330 Brookline
Ave, Boston, MA 02215. Address correspondence to
orbit, abutting the superior rectus and lateral This causes proptosis and, if enlargement of
R. L. Eisenberg (rleisenb@bidmc.harvard.edu). rectus muscles (Fig. 1C). The lacrimal gland the gland is rapid, may even produce diplo-
consists of an orbital lobe (Figs. 1A and 2A) pia. Epithelial tumors typically arise from
2
Massachusetts Eye and Ear Infirmary, Harvard Medical and a palpebral lobe (Figs. 1B and 2B), which and involve the orbital lobe of the lacri-
School, Boston, MA.
are separated anatomically by the lateral horn mal gland, although nearly 20% of epithe-
WEB of the aponeurosis of the levator palpebrae lial tumors may involve the palpebral lobe.
This is a web exclusive article. muscle (Fig. 2C). The orbital lobe is located About half of epithelial neoplasms are be-
posterior and superior to the levator palpebrae nign and half malignant. Benign neoplasms
AJR 2013; 201:W371–W381 aponeurosis, and the palpebral lobe is situated include pleomorphic adenoma and oncocy-
0361–803X/13/2013–W371
anterior and inferior to it. The orbital lobe is toma. The most common malignant epithe-
larger and the site of most lacrimal gland epi- lial neoplasms are adenoid cystic carcinoma
© American Roentgen Ray Society thelial neoplasms. Infiltrative and inflamma- and mucoepidermoid carcinoma.

AJR:201, September 2013 W371


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Gao et al.

TABLE 1:  Common Patterns of Lacrimal Gland Masses


Cell Type Lesion Laterality Lobe Involved Bone Pain
Epithelial Pleomorphic adenoma Unilateral Orbital Scalloping No
Adenoid cystic carcinoma Unilateral Orbital Destruction Yes, with or without paresthesia
Mucoepidermoid carcinoma Unilateral Orbital Scalloping, destruction Yes
Lymphoid Lymphoid hyperplasia Bilateral Orbital and palpebral None, with or without No
occasional remodeling
Lymphoma Variable Orbital and palpebral None, with or without No
occasional remodeling
Leukemic Chloroma Variable Orbital and palpebral Variable Yes
Metastasis Metastases Variable Variable Variable Variable
Inflammatory Infectious dacryoadenitis Unilateral Orbital and palpebral None Yes
Sarcoidosis Bilateral Orbital and palpebral None Yes
Pseudotumor (inflammatory Variable, unilateral Orbital and palpebral None Variable
dacryoadenitis) more than bilateral
Sjögren syndrome Bilateral Orbital and palpebral None Yes (dryness)
Others Sickle cell disease Variable Orbital and palpebral None Yes

Pleomorphic Adenoma perineural spread results in severe pain, which with painful proptosis due to rapid growth of
Pleomorphic adenoma is the most common is sometimes associated with paresthesia. this infiltrative lesion with bony scalloping or
benign epithelial tumor of the lacrimal gland. On CT, adenoid cystic carcinoma appears destruction and occasional invasion into adja-
Patients usually present between 20 and 40 as a well-defined, homogeneous, and unilat- cent structures (Fig. 5). Distant metastases to
years old with unilateral and painless proptosis. eral solid mass involving the orbital lobe of the brain have been reported. The imaging fea-
On CT, the tumor typically appears as a well- the lacrimal gland. There is typically associ- tures may be indistinguishable from adenoid
circumscribed unilateral mass that involves ated bone destruction (Fig. 4), and there may cystic carcinoma, and treatment includes re-
the orbital lobe of the lacrimal gland (Figs. 3A be calcifications within the lesion. The tumor section, radiation, and chemotherapy.
and 3C), although up to 10% of these lesions may extend along perineural pathways (cra-
may occur solely in the palpebral lobe. Slow nial nerve branches of V1 and V2) through Lymphoproliferative Lesions
growth of a pleomorphic adenoma often results the superior orbital fissure into the cavernous Reactive Lymphoid Hyperplasia
in smooth bony scalloping (Fig. 3B). Moder- sinus. After contrast administration, the tu- Reactive lymphoid hyperplasia is benign
ate contrast enhancement is common, but cal- mor usually shows diffuse enhancement. and represents part of the spectrum of lym-
cification within the tumor is rare. On MRI, a On MRI, an adenoid cystic carcinoma is phoid diseases of the orbit. Reactive lymphoid
pleomorphic adenoma appears as a unilateral hypointense to muscle on T1-weighted imag- hyperplasia has a tendency to involve bilat-
orbital lobe mass that has low-to-intermediate ing and intermediate-to-high in signal com- eral lacrimal glands. The disease is diffuse
signal compared with muscle on T1-weight- pared with brain cortex on T2-weighted im- and involves both orbital and palpebral lobes
ed imaging and intermediate signal compared aging. There is diffuse enhancement after arising from intrinsic lacrimal lymphoid tis-
with brain cortex on T2-weighted imaging. The gadolinium administration. MRI has supe- sues. Similar to lymphoma, lymphoid hyper-
lesion shows moderate contrast enhancement rior resolution in showing perineural spread plasia usually has a concave inner margin and
after gadolinium administration. Although be- and involvement of subjacent soft tissues. molds to the globe. Although lymphoid hy-
nign, up to 20% of pleomorphic adenomas un- Adenoid cystic carcinoma has a poor perplasia is, in general, not readily differenti-
dergo malignant degeneration, especially after prognosis. Perineural extension of the le- ated from lymphoma on CT, it is more hetero-
incomplete excisional biopsy. Therefore, treat- sion is so common that complete surgical geneous compared with lymphoma, which is
ment consists of complete surgical removal removal is difficult. Therefore, brachyther- typically homogeneous in density. On MRI,
without rupture of the tumor capsule. If preop- apy with 125I is sometimes used as adjuvant it appears T1 hypointense to the brain cortex
erative incisional biopsy is performed, subse- treatment. Local recurrence is common, (Fig. 6C), and shows slightly heterogeneous
quent complete surgical removal plus excision and distant metastasis most often involves contrast enhancement (Fig. 6D).
of adjacent periorbital tissues almost complete- the lung. Current treatment regimens pri-
ly eliminates the chance of recurrence. marily focus on local disease control. Lymphoma
The lacrimal gland contains intrinsic lym-
Adenoid Cystic Carcinoma Mucoepidermoid Carcinoma phoid as well as epithelial tissue. Primary
Adenoid cystic carcinoma is the most com- Mucoepidermoid carcinoma of the lacrimal lymphoproliferative disease of the orbit, how-
mon malignant epithelial tumor of the lacrimal gland is rare, representing only 1% of all lac- ever, including that of the lacrimal gland, is
gland. Patients typically present in the fourth rimal gland neoplasms. Like most malignant relatively rare. Most lymphoid disease arises
decade of life with a painful unilateral lacrimal neoplasms of the lacrimal glands, patients with elsewhere and involves the orbit secondarily.
gland mass. The propensity of this tumor for mucoepidermoid carcinoma typically present The most common lymphoma affecting the

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Lacrimal Gland Masses

lacrimal gland is of the mucosa-associated MRI, the lesion is hypointense to isointense Ocular involvement, most commonly uveitis,
lymphoid tissue type, which has a much bet- to muscle on T1-weighted imaging (Figs. occurs in 80% of patients with sarcoidosis. Sar-
ter prognosis than the less common secondary 9A and 9B) and isointense to brain cor- coidosis of the lacrimal gland is typically bi-
follicular lymphoma of the orbit. Lymphoma tex on T2-weighted imaging (Figs. 9C and lateral and diffuse, and it may cause painless
tends to occur in older patients who typically 9D). Marrow involvement is common, and enlargement of the orbital and palpebral lobes.
present with painless lacrimal gland masses. the degree of contrast enhancement is vari- On CT, sarcoidosis produces diffuse bilater-
CT shows bilateral or, less commonly, uni- able. The treatment of chloroma includes lo- al lacrimal gland enlargement with hyperen-
lateral diffuse involvement of the lacrimal cal disease control, chemotherapy, and bone hancement on contrast-enhanced images (Figs.
glands, which involves both the palpebral (Fig. marrow transplantation. 12A and 12C) and no bone involvement (Figs.
7A) and orbital (Fig. 7B) lobes. Lacrimal gland 12B and 12D). Concurrent imaging of the chest
lymphoma is well defined and homogeneous Metastasis usually shows prominent mediastinal and hi-
and conforms to the globe and subjacent osse- Rare metastatic involvement of the lacri- lar lymphadenopathy. On MRI, there is also
ous structures. Lymphoma is isodense to mus- mal gland cannot be distinguished from a pri- diffuse bilateral enlargement of the lacrimal
cle and shows mild enhancement after contrast mary lacrimal gland tumor on the basis of any glands, which appear hypointense to isointense
administration (Fig. 7A). There is typically no specific imaging feature. Patients are general- on T1-weighted imaging and heterogeneously
infiltrative bone destruction, although bone re- ly older and present with a unilateral lacrimal hyperintense on T2-weighted imaging, with
modeling and/or sclerosis may be present. mass or bilateral lacrimal masses without any avid enhancement after gadolinium adminis-
On MRI, lacrimal gland lymphoma is homo- specific pattern of lobe involvement, and there tration. The mainstay of treatment of sarcoid-
geneous and isointense to muscle on T1-weight- may be associated bone involvement. Primary osis is corticosteroids. More aggressive forms
ed imaging (Fig. 8A) and isointense-to-slightly neoplasms that most commonly metastasize of disease may require immunosuppressants,
hyperintense to brain cortex on T2-weighted to the lacrimal gland include carcinomas of such as methotrexate and cyclophosphamide.
imaging (Fig. 8C). There is moderate enhance- the breast, prostate, kidney, thyroid, and mela-
ment after gadolinium administration (Fig. noma. In the pediatric population, Ewing sar- Pseudotumor
8B). Lymphoma is hypercellular and shows coma or neuroblastoma may extend from ad- Pseudotumor (also known as inflamma-
slowed diffusion on diffusion-weighted imag- jacent bony structures to secondarily involve tory dacryoadenitis and idiopathic orbital
ing (Fig. 8D). The various subtypes of lym- the lacrimal gland. Treatment focuses on local inflammation) is an idiopathic fibroinflam-
phoma have similar appearances and cannot be control and eradication of the primary malig- matory process that can involve the lacrimal
differentiated by imaging criteria. nant disease. glands and is thought to be related to autoim-
The treatment of lacrimal gland lympho- mune derangement. Patients typically pres-
ma depends on the histologic classification. Inflammatory Lesions ent with long-standing occasionally painful
Local radiotherapy is used to treat lesions Infectious Dacryoadenitis unilateral more often than bilateral enlarge-
confined to the orbit, whereas chemotherapy Dacryoadenitis is inflammation of the ment of the lacrimal glands, which may have
is used for more widespread disease. lacrimal gland, which may be acute or a relapsing remitting course. Mass effect in
chronic, infectious, or inflammatory. Acute severe disease may lead to compression of
Leukemic Lesions infectious dacryoadenitis is more common the globe or extraocular muscles, but visu-
Leukemia-Chloroma or Granulocytic Sarcoma in children and young adults, who typical- al acuity usually remains intact. The diagno-
Orbital granulocytic sarcoma, also known ly present with unilateral painful propto- sis is made only when other diseases causing
as chloroma because of its green color related sis. On CT, there is unilateral involvement lacrimal gland enlargement have been ex-
to high myeloperoxidase content, can rarely with diffuse enlargement of the lacrimal cluded. Biopsy shows nongranulomatous in-
secondarily involve the lacrimal gland. This gland that affects both the orbital and palpe- flammation, excluding neoplasm. Lacrimal
leukemia-related tumor of myeloid cell ori- bral lobes (Fig. 10A). There is avid contrast gland pseudotumors have been recognized
gin represents a collection of these cells out- enhancement in the acute phase of disease as part of an emerging rare multiorgan sys-
side the bone marrow. Children and young representing hyperemia, often with peri- tem disorder known as IgG4-related disease,
adults are most likely to be affected. Patients glandular inflammatory changes and occa- which presents with pseudotumors and fibro-
typically present with progressive painful sionally complicated by abscess formation sclerosing disease involving multiple organs.
proptosis, which may develop either before (Figs. 11A and 11B). Inflammation may ex- On CT, pseudotumor appears as diffuse
or after the onset of systemic leukemia (most tend to subjacent muscles, causing superior unilateral or bilateral enlargement of the lac-
commonly in acute myelogenous leukemia). and lateral rectus myositis, but there is no rimal glands involving both the orbital and
The uvea, choroids, retina, optic nerve, and bone involvement. MRI shows similar uni- palpebral lobes. There is variable contrast
orbital fat are more commonly primarily in- lateral diffuse enlargement and hyperen- enhancement and no bone involvement. On
volved, with secondary disease extension to hancement of the lacrimal gland, with sur- MRI, these lacrimal gland masses are usu-
one or both of the lacrimal glands. rounding inflammatory changes but no bone ally isointense to hypointense to muscle on
On CT, a chloroma of the lacrimal gland involvement. Acute infectious dacryoadeni- T1-weighted imaging (Fig. 13A) and isoin-
is homogeneous, well-defined, isodense or tis responds well to antibiotic therapy. tense to hypointense to brain cortex on T2-
hypodense to muscle, and can be associated weighted imaging (Figs. 13D and 13E) and
with bone involvement. Contrast-enhanced Sarcoidosis show variable contrast enhancement. When
images show varying degrees of homoge- Sarcoidosis is the most common inflam- compared with most lacrimal gland tumors,
neous or heterogeneous enhancement. On matory disease involving the lacrimal gland. pseudotumors typically are relatively more

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Gao et al.

T2 hypointense (Figs. 13D and 13E). Treat- of lacrimal glands, with patchy areas of rela- appearing enlarged and homogeneously en-
ment response of pseudotumor to corticoste- tive T1 hyperintensity (Fig. 14C), reflecting hancing or hyperenhancing on CT and MRI.
roid therapy is usually excellent, although components of fatty replacement, with subtle Miscellaneous neoplastic causes of lac-
more resistant and recurrent disease may re- heterogeneous appearance after contrast ad- rimal gland masses include rhabdomyosar-
quire treatment with immunosuppressants. ministration (Fig. 14D). Treatment includes coma and solitary fibrous tumor. Lacrimal
artificial tears and avoidance of low-humid- gland involvement has also been described in
Sjögren Syndrome ity environments. the setting of post transplantation lymphopro-
Sjögren syndrome is an autoimmune dis- liferative disease, which is exceedingly rare.
ease that targets the lacrimal glands and sali- Others
vary glands, producing the sicca syndrome of Chronic inflammatory dacryoadenitis may Suggested Readings
dry eyes and dry mouth. Diagnosis relies on develop in a variety of other entities, includ- 1. Jung WS, Ahn KJ, Park MR, et al. The radiologi-
lacrimal gland biopsy, lacrimal flow rate mea- ing tuberculosis, amyloidosis, thyroid oph- cal spectrum of orbital pathologies that involve
surement, and rose Bengal staining. Involve- thalmopathy, and anti-neutrophil cytoplasmic the lacrimal gland and the lacrimal fossa. Korean
ment tends to be bilateral and diffuse. Early antibody–associated granulomatous vasculi- J Radiol 2007; 8:336–342
in the disease process, there is enlargement tis (formerly known as Wegener granuloma- 2. Som PM, Curtin HD. Head and neck imaging, 5th
of the glands, then progressive fatty deposi- tosis). Involvement in these conditions may ed. St. Louis, MO: Mosby Elsevier 2011
tion, and subsequent atrophy. On CT, there is be unilateral or bilateral, and treatment is 3. Kostick DA, Linberg JV. Lacrimal gland tumors.
bilateral lacrimal gland enlargement or atro- targeted to the underlying disease. Lacrimal In: Tasman W, Jaeger EA, eds. Duane’s ophthal-
phy without bone involvement (Figs. 14A and gland involvement has also been reported in mology, 2006 ed., vol. 2. Philadelphia, PA. Lip-
14B). On MRI, unenhanced T1-weighted im- patients with sickle cell disease, which pres- pincott Williams & Wilkins, 2006:chapter 40 ed.
aging shows bilateral enlargement or atrophy ents as lacrimal gland edema, with glands Vol. 2, Chapter 40

A B

Fig. 1—Normal CT of lacrimal glands.


A and B, Unenhanced axial CT images show orbital
(solid arrows, A) and palpebral (dashed arrow, B)
lobes of lacrimal glands.
C and D, Coronal (C) and sagittal (D) CT images show
normal lacrimal glands (arrows), which are isodense
to muscle, in superolateral aspects of orbits.
C D

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Lacrimal Gland Masses

Fig. 2—Normal MRI of lacrimal glands.


A and B, Gadolinium-enhanced T1-weighted fat-
saturated axial MR images show orbital (A) and
palpebral (B) lobes of normal lacrimal glands (arrows),
which have homogeneous enhancement and are
isointense to adjacent extraocular muscles.
C and D, Gadolinium-enhanced T1-weighted fat-
saturated and T2-weighted coronal images show
location of levator palpebrae muscle aponeurosis
(arrows, C), which divides lacrimal gland into
posterior-superior orbital lobe (solid arrow, D) and
anterior-inferior palpebral lobe (dashed arrow, D).

A B

C D

A B

Fig. 3—Pleomorphic adenoma.


A–D, Axial (A and B) and coronal (C and D) contrast-
enhanced CT images show rounded heterogeneously
enhancing mass in orbital portion of lacrimal gland
(arrows, A and C). Note subtle associated bone
remodeling (arrows, B and D).
C D

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Gao et al.

Fig. 4—Adenoid cystic carcinoma.


A–D, Axial (A and B) and coronal (C and D)
unenhanced CT images show right orbital lobe
lacrimal gland mass (black arrows, A and D) with
permeative destruction of right sphenoid bone (white
arrows, B and C).

A B

C D

A B

Fig. 5—Mucoepidermoid carcinoma.


A–D, Contrast-enhanced axial (A), coronal (B),
and sagittal (D) CT images show homogeneously
enhancing mass in orbital lobe of right lacrimal gland
(arrows), with associated subtle bone remodeling
(arrowhead, B).
C D

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Lacrimal Gland Masses

Fig. 6—Lymphoid hyperplasia.


A–D, Axial unenhanced T1-weighted (A) and
T2-weighted (B) and axial unenhanced (C) and
gadolinium-enhanced (D) images show right greater
than left bilateral lacrimal gland masses (arrows)
that mold to globe and show slightly heterogeneous
contrast enhancement.

A B

C D

A B

Fig. 7—Lymphoma.
A and B, Contrast-enhanced axial CT images show
diffuse infiltration and enlargement of right lacrimal
gland. There is involvement of both orbital (black
arrow, A) and palpebral lobes (white arrow, A)
without bony change.
C and D, Contrast-enhanced coronal CT images show
lymphoma to be isodense to extraocular muscles
with mild enhancement (arrow, C) and no bone
changes (D).
C D

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Gao et al.

A B C
Fig. 8—Lymphoma.
A–C, Unenhanced (A) and gadolinium-enhanced (B)
axial T1-weighted and T2-weighted (C) MR images
show right lacrimal mass involving both orbital and
palpebral lobes of gland to be isointense to slightly
hypointense to muscle on T1-weighted image,
isointense to brain cortex on T2-weighted image,
and with low level homogeneous enhancement on
contrast-enhanced image (arrows).
D and E, Diffusion-weighted axial image of same
lesion (D) and apparent diffusion coefficient map (E)
show restricted diffusion (arrows), consistent with
lymphomatous hypercellularity.

D E

A B
Fig. 9—Chloroma.
A and B, Coronal (A) and axial (B) gadolinium-enhanced T1-weighted MR images show homogeneous mildly enhancing mass hypointense to isointense to muscle in
orbital lobe of right lacrimal gland (arrows).
(Fig. 9 continues on next page)

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Lacrimal Gland Masses

C D
Fig. 9 (continued)—Chloroma.
C and D, Coronal (C) and axial (D) T2-weighted images show mass to be isointense to brain cortex (arrows).

Fig. 10—Infectious dacryoadenitis.


A and B, Contrast-enhanced axial (A) and coronal (B)
CT images show diffusely enlarged and enhancing
left lacrimal gland (arrows). Patient presented with
significant left eye pain and was diagnosed with
bacterial dacryoadenitis. There are no bony changes
as expected in setting of acute inflammation.

A B

Fig. 11—Abscess.
A and B, Contrast-enhanced axial (A) and coronal
(B) CT images show rim-enhancing lesion within
edematous and hyperenhancing left lacrimal gland
(arrows).
A B

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Gao et al.

Fig. 12—Sarcoidosis.
A–D, Unenhanced axial (A) and coronal (C) CT images
show diffuse bilateral lacrimal gland enlargement
involving both palpebral and orbital lobes (arrows).
Axial (B) and coronal (D) bone window images show no
bone changes.

A B

C D

A B C

Fig. 13—Pseudotumor.
A–C, Axial unenhanced (A) and gadolinium-enhanced
(B) and coronal gadolinium-enhanced (C) MR images
show large bilateral masses involving both palpebral
and orbital portions of lacrimal gland (arrows).
D and E, Axial T2-weighted images show relative
hypointensity of lesions (arrows) compared with
brain cortex.
D E

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Lacrimal Gland Masses

Fig. 14—Sjögren syndrome.


A and B, Axial unenhanced CT images show bilateral
lacrimal gland enlargement without bony involvement
(arrow, A).
C and D, Axial unenhanced (C) and gadolinium-
enhanced (D) T1-weighted MR images show
bilateral enlarged glands with faint patchy intrinsic
T1 hyperintensity reflecting fatty deposition and
mildly heterogeneous enhancement after contrast
administration (arrows).

A B

C D

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