REVIEW

CURRENT
OPINION Overview of benign and malignant lacrimal
gland tumors
Ahmet Kaan G€ uz a, Yağmur Seda Yeşiltaşa, and Carol L. Shields b
und€

Purpose of review
The goal of this article is to review the key clinical and radiological features, treatment strategies, and
prognosis of lacrimal gland tumors.
Recent findings
Debulking of the orbital lobe of the lacrimal gland may offer improved control rates in dacryoadenitis
without compromise of tear film function. Contrary to previous belief, careful biopsy of the lacrimal gland
prior to excision does not appear to increase the risk of recurrence in cases with suspected pleomorphic
Downloaded from http://journals.lww.com/co-ophthalmology by BhDMf5ePHKbH4TTImqenVCyN9N7ltPfEAbvREDUsdrCRSeQA/KvjsB0w+tRi/mYUPclCHyx3XWw= on 09/09/2018

adenoma. Low-dose radiation (4 Gy) in two 2-Gy fractions appears to be effective and well tolerated in
indolent non-Hodgkin lymphoma of the ocular adnexa with high local control rate. Eye-sparing surgery for
adenoid cystic carcinoma (ACC) leaving minimal or no tumor residual in the orbit followed by adjuvant
radiation therapy or chemoradiotherapy may provide good local control and long-term survival outcomes.
Intra-arterial chemotherapy has been found to decrease recurrence and improve survival in ACC and can
also be used as part of an eye-sparing treatment strategy. The development of targeted drugs may offer
palliation for patients with unresectable or metastatic disease in lacrimal gland carcinoma.
Summary
This article offers an update on diagnosis, management, and prognosis of the major lacrimal gland lesions.
Keywords
Adenoid cystic carcinoma, exenteration, lacrimal gland, orbitotomy, pleomorphic adenoma, radiotherapy, tumor

INTRODUCTION (malignant mixed tumor or carcinoma ex-pleomor-

Lacrimal gland lesions have an estimated incidence
of 1 per 1 million people per year [1]. Lacrimal gland
lesions are fairly common in the orbit accounting
for 10% of all orbital tumors, ranging from 3–18% in
different series (Table 1) [2–5].
Lesions of the lacrimal gland can be divided into
epithelial and nonepithelial lesions. Nonepithelial
tumors account for 55–80% of lacrimal gland
masses [2–8]. Nonepithelial masses include dacryoa-
denitis (nongranulomatous and granulomatous
inflammation) (Fig. 1), lymphoid tumors (Fig. 2),
and other mesenchymal tumors such as cavernous
hemangioma, dermoid cyst, and others. Epithelial
tumors account for about 20–45% of lacrimal gland
tumors [2–8]. Benign epithelial tumors constitute
about half of epithelial tumors. Malignant epithelial
tumors comprise the other half of epithelial tumors.
Benign epithelial tumors include lacrimal ductal
cysts (dacryops) (Fig. 3) and pleomorphic adenoma
(benign mixed tumor) (Fig. 4). Malignant epithelial
tumors include adenoid cystic carcinoma (ACC)
(Figs. 5 and 6), pleomorphic adenocarcinoma
phic adenoma), de novo adenocarcinoma (Fig. 7),
and other rare entities [6–8]. Of the malignant
epithelial tumors, ACC is the most common
accounting for about 60% of the cases followed by
pleomorphic adenocarcinoma (20%), and de novo
adenocarcinoma (10%) [6–8].
CLINICAL FEATURES
All lacrimal gland lesions can present at any age.
Epithelial tumors are commonly seen in middle-
aged adults. Pleomorphic adenoma and ACC usually
a
Department of Ophthalmology, Ankara University Faculty of Medicine,
Ankara, Turkey and bOcular Oncology Service, Wills Eye Hospital,
Thomas Jefferson University, Philadelphia, Pennsylvania, USA
Correspondence to Ahmet Kaan G€ und€
uz, MD, Professor of Ophthal-
mology, Farilya Is Merkezi 8/50, Ufuk Universitesi Cad, Cukurambar
Sogutozu, Ankara, Turkey. E-mail: drkaangunduz@gmail.com
Curr Opin Ophthalmol 2018, 29:458–468
DOI:10.1097/ICU.0000000000000515

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 Lacrimal gland tumors G€
und€
uz et al.

age of 50 years [10]. Lymphomas on the other hand

KEY POINTS
 Debulking of the orbital lobe of the lacrimal gland may
offer improved control rates in dacryoadenitis without
any compromise of tear film function.
 Careful biopsy of the lacrimal gland prior to excision
does not appear to increase the risk of recurrence in
cases with suspected pleomorphic adenoma when the
diagnosis is not certain.
 Low-dose radiation with 4 Gy in two 2-Gy fractions
appears to be effective and well tolerated in the
treatment of indolent non-Hodgkin lymphoma of the
ocular adnexa with high local control rate.
 Eye-sparing surgery for adenoid cystic carcinoma
leaving minimal or no tumor residual in the orbit
followed by adjuvant radiation therapy or
chemoradiotherapy may provide good local control
and long-term survival outcomes.
 The development of targeted drugs based on the
molecular biology of lacrimal gland carcinoma may
offer palliation for patients with unresectable or
metastatic disease.
present in patients with an average age of 40 years
[1]. ACC has a bimodal age distribution and has a
second peak in 10–20 years of age [9]. Adenocarci-
noma of the lacrimal gland usually occurs in an
older population compared to ACC, with a mean
present in older patients aged 60–70 years [11].
Pain is uncommon in patients with benign lac-
rimal gland lesions with the exception of dacryoa-
denitis. Dacryoadenitis typically develops acutely
and presents with pain, eyelid swelling, and hyper-
emia although there are also chronic dacryoadenitis
cases that have an indolent disease course (Fig. 1a)
[12]. Pain is often suggestive of malignant lacrimal
gland lesions including ACC and adenocarcinoma
and may be secondary to bone or orbital nerve
involvement.
Dacryoadenitis and lymphoma of the lacrimal
gland can be bilateral [13] (Fig. 2a). On the other
hand, epithelial lacrimal gland tumors are usually
unilateral and lead to inferior and nasal displacement
of the globe (Fig. 4a). Benign tumors typically have a
history of more than 6 months while malignant
tumors have a history of less than 6 months [10].
In dacryops, the palpebral lobe is affected far
more commonly than the orbital lobe. The charac-
teristic finding is a cystic swelling visible through
the upper temporal conjunctiva (Fig. 3a) [14].
Lacrimal gland ACC and adenocarcinoma
(Fig. 7a) are aggressive tumors and have a propensity
to metastasize to regional lymph nodes including
preauricular, submandibular, and cervical lymph
nodes and distant sites including lung, liver, and
brain. Metastatic adenocarcinoma is associated with
a shorter patient survival time than ACC [10].

Table 1. The frequency of lacrimal gland tumors among all orbital tumors in the series of Shields et al. [2] (n ¼ 1264)
Seregard and Sahlin [4] (n ¼ 300), G€
unalp and G€
und€
uz [3] (n ¼ 1092), and Johansen et al. [5] (n ¼ 841)
Seregard and
Sahlinb G€unalp Johansen
Shields et al.a Sweden and G€ uzb Turkey
und€ et al.b Denmark
USA (1264 cases) (300 cases) (1092 cases) (841 cases)
Number of Number of Number of Number of
Lacrimal gland tumors patients (%) patients (%) patients (%) patients (%)

Nonepithelial tumors 63 (5) 26 (9) 9 (1) 59 (7)

Dacryoadenitis 37 (3) 17 (6) 9 (1) 31 (4)
Lymphoma 16 (1) 4 (1) – 9 (1)
Others 10 (1) 5 (2) – 19 (2)
Epithelial tumors 51 (4) 27 (9) 22 (2) 61 (7)
Dacryops 19 (2) 8 (3) – 8 (<1)
Pleomorphic adenoma 11 (<1) 13 (4) 14 (1) 23 (2)
Pleomorphic adenocarcinoma 4 (<1) – 2 (<1) 6 (1)
Adenoid cystic carcinoma 14 (1) 2 (1) 6 (<1) 15 (2)
Adenocarcinoma – 1 (<1) – 3 (<1)
Others 3 (<1) 3 (1) – 6 (1)
Total 114 (9) 53 (18) 31 (3) 120 (14)

Percents are rounded and show the percentage of lacrimal gland lesions within all orbital lesions.
a
Combined clinical and histopathologic diagnosis.
b
Histopathologic diagnosis only.

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Oculoplastic and orbital surgery

FIGURE 1. (a) Facial photograph of a 52 year-old male with right dacryoadenitis presenting with upper eyelid edema and
pain. (b) T1-weighted axial MRI shows enlarged right lacrimal gland that is isointense with respect to extraocular muscles and
cerebral gray matter. The tumor has an ill-defined appearance and appears molded to bone and globe. (c) The tumor
demonstrates marked enhancement after contrast injection. (d) T2-weighted axial MRI shows that the right lesion in the lacrimal
gland area is hypointense to isointense. (e) Gross photograph of the totally excised lacrimal gland after dacryoadenectomy
via anterior orbitotomy using upper lid skin crease approach. (f) Cut surface of the lacrimal gland that proved to be
dacryoadenitis (idiopathic orbital inflammation) on histopathological examination.

Pleomorphic adenocarcinoma can occur from transformation can span a period of years, even dec-
spontaneous malignant transformation of a pleomor- ades [15]. The clinical symptoms and signs are similar
phic adenoma or from an incompletely excised to those described for ACC of the lacrimal gland. Local
pleomorphic adenoma. The period of malignant recurrence and distant metastasis to bone can occur.

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 Lacrimal gland tumors G€
und€
uz et al.

FIGURE 2. (a) Bilateral lacrimal gland lymphoma in a 60 year-old female with bilateral periocular edema. (b) T1-weighted
axial MRI shows that the lacrimal gland masses have a molded appearance and are isointense to the extraocular muscles. (c)
T2-weighted axial MRI shows that the lacrimal gland lymphomas are isointense to the extraocular muscles.

IMAGING FEATURES In ACC, CT and MRI may show a well defined

Dacryoadenitis and lymphomas are typically visual-
ized on computed tomography(CT)/MRI as an ill-
defined mass with molding to the surrounding
structures such as the globe, bone, and extraocular
muscles (Figs. 1b–d, 2b and c). The lesion is usually
isointense on T1-weighted images, hypointense to
isointense on T2-weighted images and demonstrates
contrast enhancement (Figs. 1b–d, 2b and c). In
dacryops, there is a cystic lesion that has a signal
intensity similar to vitreous on T1-weighted and T2-
weighted image on MRI (Fig. 3b). In pleomorphic
adenomas, CT and MRI show a round-to-ovoid well
circumscribed superotemporal orbital mass (Figs. 4b
and c) [16]. The tumor may cause thinning (erosion)
of the overlying bone, but usually there is no bone
invasion [17]. The tumor is usually isointense on T1-
weighted images with respect to muscle, and hyper-
intense on T2-weighted images with a heterogenous
internal structure and demonstrates moderate con-
trast enhancement (Figs. 4b and c) [16].
round-to-oval mass similar to pleomorphic ade-
noma initially (Fig. 6a), but the mass later becomes
ill-defined as it grows. Bone destruction (Figs. 5a and
b) and intralesional calcium are sometimes present.
Although lacrimal gland fossa calcification is sug-
gestive of malignancy, there may be other causes
including pleomorphic adenoma, primary orbital
amyloidosis, idiopathic sclerosing orbital inflamma-
tion, calcified orbital cysts, and schwannomas as
well [18]. Although ACC has similar MRI features
to pleomorphic adenoma (Fig. 6a), one study found
that diffusion-weighted MRI can distinguish malig-
nant from benign epithelial tumors with an accu-
racy of 93% [19]. Recently, Lorenzo and Rose
described the ‘wedge sign’ as a sign of aggressive
lacrimal gland tissue. Wedge sign denotes a distinct
triangle of lacrimal gland tissue extending to the
orbital apex in a space bounded by the lateral and
superior recti and lateral orbital bone, without any
involvement of the intraconal space. The authors

FIGURE 3. (a) Dacryops (lacrimal gland ductal cyst) presenting as a bluish cystic mass in the upper temporal fornix. (b) T1-
weighted axial MRI shows a well circumscribed cystic lesion isointense to vitreous.

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Oculoplastic and orbital surgery

FIGURE 4. (a) A 62 year-old male presenting with right proptosis characterized by medial and inferior displacement of the
globe. The tumor proved to be lacrimal gland pleomorphic adenoma. (b) T1-weighted coronal MRI shows a well
circumscribed tumor located superiorly in the orbit. The tumor shows marked contrast enhancement and does not mold to the
globe or orbital bones. (c) T2-weighted coronal MRI demonstrates that the tumor is hyperintense with respect to extraocular
muscles and cerebral gray matter. (d) Gross photograph demonstrates completely excised pleomorphic adenoma.

reported that the wedge sign may be associated with
lacrimal gland carcinoma, fast-growing lymphomas,
&
and severe dacryoadenitis (Fig. 7b) [20 ].
MANAGEMENT
Dacryoadenitis
A major issue of debate in the management of
dacryoadenitis is the need for orbital biopsy. Given
the low morbidity of the procedure and the high
incidence of systemic disease involving the lacrimal
gland, biopsy is recommended for isolated inflam-
mation of the lacrimal gland. Typical cases can be
initially treated with oral corticosteroids without
biopsy. However, an orbital biopsy is usually war-
ranted in recurrent cases.
High dose of systemic corticosteroids (usually in
the order of 1 mg/kg/day prednisone) with a slow
taper is the generally accepted standard treatment
for dacryoadenitis. However, response rate to
corticosteroids range from 55% to 79%. Further,
several studies showed that 42–78% of patients
demonstrated recurrence after tapering of steroids
&&
and 26% became steroid dependent [12,21 ,22].
Recently, debulking of the lacrimal gland instead
of an incisional biopsy was proposed as the primary
&&
treatment for dacryoadenitis (Figs. 1e and f) [21 ].
In this surgery, the orbital portion of the lacrimal
gland is usually removed in total or a generous
biopsy is taken from the gland so that only a minor
&&
unresectable portion is remaining [21 ]. In a review
of 46 patients from two different institutions treated
with surgical debulking and one-time intraorbital
corticosteroids, a full clinical recovery was seen in
80% of patients. For those that demonstrated insuf-
ficient improvement or recurrence, various combi-
nations of repeat intraorbital corticosteroids,
external beam radiotherapy (EBRT), rituximab,
&&
and methotrexate were used [21 ]. Debulking sur-
gery for idiopathic dacryoadenitis did not lead to
additional dry eye that was not present before

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 Lacrimal gland tumors G€
und€
uz et al.

FIGURE 5. A 47 year-old female with left adenoid cystic carcinoma of the lacrimal gland. (a) T1-weighted axial image shows
a tumor with oval configuration, irregular margins (arrow), bone destruction, and temporal fossa invasion (arrowhead). (b) T2-
weighted axial image shows adenoid cystic carcinoma that is heterogenously hyperintense to the extraocular muscle and
cerebral gray matter.

surgery provided that the palpebral lobe is preserved
since the excretory ducts pass through the palpebral
&&
lobe and then empty into the superior fornix [21 ].
Lymphoid tumors
Surgery
Complete excision of lacrimal gland lymphoma is
rarely possible. Usually, the tumor presents with ill-
definedborders,makingcompleteexcisionimpossible.
External Beam Radiotherapy
EBRT has been the most frequently used treatment
for lacrimal gland lymphoma in the absence of any
systemic involvement. A wide variation of radiation
doses has been recommended, ranging from 20 to 25
Gy for benign reactive hyperplasia up to 35–40 Gy
for malignant lymphoma [23]. A recent study found
that much lower radiation doses in the order of 2 Gy
 2 was effective with a high overall response rate of
100% and minimal side effects in the management
of ocular adnexal non-Hodgkin lymphoma. The
authors of this study pointed out that with the
low dose irradiation given initially there is the
option of reirradiation in case local recurrence
&&
occurs [24 ]. Use of partial orbit irradiation in treat-
ing low-grade, localized orbital lymphoma achieved
5-year survival of 100% with a 5.3% failure and
21.4% progression rate, providing comparable out-
&
comes to whole-orbit irradiation [25 ].

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Oculoplastic and orbital surgery

FIGURE 6. A 32-year-old female with adenoid cystic carcinoma of the lacrimal gland on the left side. (a) T2-weighted coronal
MRI demonstrates a relatively well circumscribed tumor in the left lacrimal fossa that is heterogenously hyperintense to the
extraocular muscles and cerebral gray matter. (b) Gross photograph of the completely excised ACC shows a well
circumscribed mass with irregular margins. (c) Histopathologic examination shows adenoid cystic carcinoma consisting of
solid and cribriform growth patterns (Hematoxylin and eosin, 40). (d) Postoperative T1-weighted axial MRI shows no
evidence of recurrence 2 years after orbitotomy and external beam irradiation.

Chemotherapy necessary in symptomatic cases with motility

With solitary lacrimal gland lymphoma, systemic
chemotherapy is not indicated, except for diffuse
large B-cell lymphoma. When systemic involve-
ment occurs, chemotherapy should be given [26].
Immunotherapy
The local use of interferon-a for lacrimal gland
lymphoma and ocular adnexal lymphoma is not
yet established. Monoclonal antilymphocyte anti-
bodies represent the newest form of lymphoma
treatment, especially for low-grade B-cell follicular
lymphoma or mucosa-associated lymphoid tissue
lymphomas. The most commonly used is the mono-
clonal antiCD20 antibody (Rituximab) which leads
to the destruction of B cells using mechanisms of
complement- and antibody-mediated destruction,
as well as induction of apoptosis [27].
Dacryops (Lacrimal gland ductule cyst)
restriction of the globe and cases with dacryops
abscess [14].
Pleomorphic adenoma
Pleomorphic adenoma should be excised intact with
a cuff of normal surrounding orbital fat tissue
(Fig. 4d). The orbitotomy can usually be done with-
out the need for an osteotomy (Kronlein approach).
During excision of pleomorphic adenoma, an ade-
quate margin of the surrounding lacrimal gland and
the adjacent periorbita should be removed to mini-
mize any tumor seeding from microscopic exten-
sions through the pseudocapsule. Incisional biopsy
is usually not done. One paper quoted a 5-year
recurrence rate of 32% when biopsy is done before
excision [28]. On the other hand, Lai et al. found
that prior incisional biopsy and subsequent excision
of the lacrimal gland did not increase the risk of
recurrence in pleomorphic adenoma [29 ]. Recur-
&&

The treatment for dacryops consists usually of obser- rence of pleomorphic adenoma typically occurs in
vation. Complete removal of the intact cyst may be an infiltrative fashion and may necessitate extensive

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 Lacrimal gland tumors G€
und€
uz et al.

FIGURE 7. (a) A 69-year-old male with adenocarcinoma of the left lacrimal gland. The patient had undergone incisional
biopsy of the lesion elsewhere for a rapidly growing eyelid mass. He presented with a ptotic eyelid and limited eye motility in
all directions of gaze. (b) T2-weighted axial MRI shows the lacrimal gland adenocarcinoma demonstrating an ill-defined tumor
extending to the orbital apex. The tumor blends imperceptively with the lateral rectus muscle and is restricted to the space
between the lateral rectus muscle and the lateral orbital bone (the wedge sign). (c) Atypical epithelial cells form solid nests. A
few abortive adenoid structures are also seen (Hematoxylin and eosin, x100). (d) Mucin is detected in the lumen of abortive
adenoid structures (Mucicarmine, 100).

resection or even exenteration. About 10% of recur-
rent pleomorphic adenomas undergo malignant
transformation by 20 years and 20% undergo malig-
nant change by 30 years [28].
Many pleomorphic adenomas of the lacrimal
gland express transcription factor genes PLAG1 and
HMGA2 [30]. Additionally, alterations including
amplification, gene fusion, and translocations
in 12q genes, such as HMGIC, HMGA2, and
MDM2, are thought to play a role in malignant
transformation [15].
Adenoid cystic carcinoma
Earlier studies on ACC seemed to favor aggressive
surgical intervention with EBRT [31,32]. In ACC,
complete tumor excision and postoperative EBRT
usually in the order of 50–60 Gy are usually recom-
mended [33] (Figs. 6a–d). Proton beam radiotherapy
(60 GyE) has also recently been used in the treat-
ment of ACC with good results and less side effects
compared to conventional EBRT techniques [34]. If
complete excision of ACC is not possible, exentera-
tion surgery with or without bone removal should
be considered. The prognosis for ACC was once
considered to be very poor. However, early diagnosis
and better treatment have improved the prognosis
considerably [35].
Ahmad et al. reported on a total of 53 patients
with orbital ACC managed at eight different insti-
tutions. The authors found poor outcomes in
patients who had basaloid variant and perineural

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Oculoplastic and orbital surgery
&
invasion [36 ]. Ahmad et al. concluded that for ACC
classified as less than T3 (AJCC), eye-sparing surgery
versus orbital exenteration did not make a differ-
ence in the local recurrence rate. Of the 15 patients
classified as less than T3, 12 had received EBRT. On
the other hand, among 38 patients with at least T3
tumors, the risk of local recurrence (in the orbit or
skull base) was higher in patients treated with con-
servative surgery as opposed to orbital exenteration
and EBRT. Overall, 17 (45%) of the 38 patients with
more than T3 tumors and only 1 (7%) of the 15
patients with less than T3 tumors died of disease
&
during the study period [36 ]. Esmaeli et al. reported
on 11 cases of lacrimal gland carcinoma including 7
ACCs with AJCC stages ranging from T1 to T4c
managed with eye-sparing surgery, postoperative
&&
EBRT, and chemotherapy [37 ]. The authors found
that gross resection of the orbital mass leaving tumor
residual in areas like the superior orbital fissure and
orbital apex, where further attempted excision may
cause functional loss, did not adversely affect the
outcome. All 11 patients were alive at a median of
33 months follow-up after surgery. Therefore, the
goal should be to remove as much tumor as possible
&&
in an eye-sparing treatment approach [37 ]. Noh
et al. in a review of 19 cases of ACC concluded that
gross residual disease after eye-sparing surgery was
associated with poorer recurrence and survival rates
despite adjuvant EBRT [38].
Recently Tse et al. advocated neoadjuvant intra-
arterial cytoreductive chemotherapy (IACC) for
&&
ACC [39 ]. The protocol consisted of 2 to 3 cycles
of intra-arterial cisplatin and intravenous doxorubi-
cin, orbital exenteration, chemoradiation with an
EBRT dose of 55–60 Gy, and 3–4 cycles of intrave-
nous cisplatin and doxorubicin. Tse et al. found that
this technique improves overall survival and
&&
decreases disease recurrence [39 ]. An intact lacri-
mal artery, no disruption of bone barrier or tumor
manipulation other than incisional biopsy, tumor
confined in the orbit, and protocol compliance were
factors responsible for favorable outcomes. The che-
&&
motoxicity complications were manageable [39 ].
The authors pointed out that exenteration should be
done after cytoreductive chemotherapy is com-
pleted but not before. Furthermore, there may also
be potential efficacy of IACC in patients lacking an
intact lacrimal artery such as those after removal of
lacrimal gland or even exenteration.
There is also recent unpublished information
from the same authors that exenteration may be
avoided after neoadjuvant intra-arterial chemother-
apy, similar to the eye-sparing treatment approach
discussed above. After neoadjuvant chemotherapy,
the tumor is smaller and better delineated from the
surrounding tissues making surgical excision easier.
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Therefore, there has been a paradigm shift in the
management of ACC. The use of neoadjuvant intra-
arterial and/or intravenous chemotherapy may offer
good local control rates and improve survival [40].
However, prospective randomized controlled clini-
cal trials on IACC and other eye-sparing treatments
are missing.
In cases where minimal residual ACC remains
after surgical intervention, brachytherapy with
iodine-125 (I-125) plaques has also been used. In a
small study by Shields et al. of 4 patients with
microscopic residual tumor after excision, brachy-
therapy with I-125 plaques with a target dose of 50
Gy was applied. Of the 4 patients, 3 had tumor
control while one patient required exenteration
for recurrence separate from the area of treatment
[41].
One study showed oncogenic mutations in
more than half of cases of lacrimal gland ACC, with
KRAS mutations in 10 of 24 patients, suggesting
potential benefit of treatments targeting the
&
EGFR-RAS-RAF cascade [42 ]. The most promising
alteration is perhaps the fusion oncogene MYB-NFIB
found in the majority of ACCs [43]. Lacrimal gland
ACCs are frequently positive for MYB although this
finding has not been found to correlate with clinical
&
outcome [44 ]. Recent data suggests that mutations
in the Notch pathway may cause hyperactivation of
Notch signaling in ACC [45].
Pleomorphic adenocarcinoma (Carcinoma ex-
pleomorphic adenoma)
The management is the same as for ACC. If the
lesion is small and circumscribed, it can be removed
completely. In more advanced cases that cannot be
completely removed, orbital exenteration with pos-
sible bone removal may be necessary [15].
Adenocarcinoma
The recommended treatment is exenteration and
EBRT as soon as the diagnosis has been established
histopathologically [10]. Overexpression of Her-2
protein has been observed in lacrimal gland adeno-
carcinomas. Pharmacological agents such as lapati-
nib with CNS penetration and directed at Her-2
overexpression may be a therapeutic option in
patients with metastatic lacrimal gland adenocarci-
&
noma to the CNS [46 ].
CONCLUSION
Lacrimal gland tumors encompass a broad spectrum
of disease entities. Dacryoadenitis and lacrimal gland
carcinomas are two broad categories presenting with
Volume 29  Number 5  September 2018

pain. A triangular shaped extension of tissue from the
lacrimal gland fossa to orbital apex (the wedge sign)
on CT and MRI may be a sign of extensive lacrimal
gland carcinoma, inflammation, and lymphoma.
Low-dose radiation (4 Gy) in two 2-Gy fractions
may be effective in the treatment of indolent non-
Hodgkin lymphoma with a high local control rate. If
there is doubt about the diagnosis of pleomorphic
adenoma, careful biopsy of the lacrimal gland can be
done without an increased risk of recurrence. More
recently, eye-sparing surgery and adjuvant radiation
therapy and chemotherapy have gained popularity in
the treatment of lacrimal gland carcinoma. Neoadju-
vant intra-arterial and intravenous chemotherapy
seem to be promising in reducing the size of the
lacrimal gland carcinoma, decreasing recurrence,
and improving survival. Intra-arterial chemotherapy
would be of particular interest if combined with eye-
sparing surgery.
Acknowledgements
None.
Financial support and sponsorship
None.
Conflicts of interest
The authors declare no conflicts of interest.
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Volume 29  Number 5  September 2018