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Lacrimal gland tumors : An Overview

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Lacrimal Gland
Tumors: An Overview
Maya Hada MD, Rachna Meel MS, Neelam Pushker MD,
Seema Kashyap MD, Mandeep Bajaj MS
Dr. Rajendra Prasad Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences, New Delhi
L acrimal gland tumors represent 5% to 25% of orbital
tumors1. It has been described that about 50% of lacrimal
masses are epithelial and 50% are nonepithelial2 (Table1).
But the recent literature shows that the inflammatory lesions
and lymphoid tumors of the lacrimal gland are two to three
times more common than epithelial tumors3.
Nonepithelial Lacrimal Gland Lesions
Most of the nonepithelial lesions of the lacrimal gland
represent lymphoid proliferations or inflammations.
Preoperative characterization of the particular lesion is
key in the management, which is based on the duration of
symptoms, the presence of pain, and radiologic findings.
Table 1: Classification of Lacrimal Gland Tumors
*50% of the Orbital Lymphoproliferative lesions occur in the
Lacrimal Gland.
OcularOcular Malignancies
Malignancies
Maya Hada
MD
Inflammatory
Acute onset of swelling associated with periorbital pain,
chemosis and indurated lid indicate an inflammatory
process of either idiopathic (pseudotumor) or of infective
etiology (dacryoadenitis). A computed tomography
scan usually reveals a diffuse lacrimal enlargement with
irregular margins, frequently demonstrating contrast
enhancement and no bony change. Idiopathic orbital
inflammatory disease (pseudotumor) is treated with a short
course of corticosteroids. In case of failure to resolve over
a few weeks, an incisional biopsy should be taken should,
since acute inflammatory episodes may be related to an
underlying carcinoma (Figure 1).
Lymphoproliferative lesions
These are characterized by insidious onset, painless
proptosis in a slightly older population and is often bilateral.
CT scans show that all lymphoid tumors mold themselves
around the existing orbital structures, such as the globe and
the bony orbit, without eroding bone or enlarging the orbit
(Figure 2).
(a) (b)
Figure 1(a): 18 year old female presented with an inflammatory
swelling in the right upper eyelid which failed to resolve with oral
steroids. (b): Histopathologically, the tumor proved to be an adenoid
cystic carcinoma with a typical cribriform (Swiss cheese) pattern.
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Ocular Malignancies: Lacrimal Gland Tumors: An Overview

Table 2: Classification of Epithelial Lacrimal Gland

(a) (b) Tumors4
Classification Epithelial Lacrimal Gland Tumor
Benign Pleomorphic adenoma,
Pleomorphic adenoma with atypia
Myoepithelioma,
Oncocytoma,
Cystadenoma
Figure 2(a): CT scan axial cut shows enlargement of right lacrimal Malignant Carcinoma ex pleomorphic adenoma/
gland, with slight enhancement. Note the molding of the tumor around Low grade malignant mixed tumor (where the
the globe without bony destruction. (b): Photomicrograph showing
low-grade, marginal zone B-cell lymphoma.
carcinoma is noninvsive or minimally
invasive [<1.5mm] as defined by the
WHO classification)
Epithelial tumors of the lacrimal gland Polymorphous low-grade carcinoma
A pathologic classification of the epithelial tumors of the Mucoepidermoid, grades 1 and 2
lacrimal gland (Table 2) is recently proposed, based on the Epithelial-myoepithelial carcinoma,
current WHO classification of salivary gland tumors4. Acinic cell carcinoma,
Basal cell carcinoma
Pleomorphic Adenoma Mucinous adenocarcinoma
It is the most common benign neoplasm of the lacrimal High grade Carcinoma ex plomorphic adenoma/
gland. The term “benign mixed tumor” denotes that malignant mixed tumor, which includes
these tumors are derived from a mixture of epithelial and adenocarcinoma and adenoid cystic
mesodermal elements. carcinoma arising in a pleomorphic
adenoma (where the carcinoma is
Clinical Features
invasive (>1.5 mm] as defined by the
Pleomorphic adenoma usually occurs in the fourth and fifth WHO classification)
decade of life, and incidence is equal for both genders. It Adenoid cystic carcinoma not
presents with symptoms of painless, progressive proptosis otherwise specified
and downward and inward displacement of the globe. A Adenocarcinoma not otherwise
non tender palpable mass in the superotemporal orbital specified
quadrant is present in most patients (Figure 3a). Although Mucoepidermoid, grade 3
pleomorphic adenoma usually involves the orbital lobe Ductal acenocarcinoma
of the lacrimal gland, palpebral lobe involvement is seen Squamous cell carcinoma
in 10% of cases5. These palpebral lobe tumors are freely Sebaceous adernocarcinoma
movable and do not produce proptosis or bony changes6. Myoepithelial carcinoma
Lymphoepithelial carcinoma
Radiologic Features Other rare and unclassifiable
CT images (Figure 3b) show well-defined, round to oval carcinomas
lesions that are smooth in outline and are associated Dedifferentiation in any of the above
with prominent lacrimal fossa formation due to pressure carcinomas
erosion. Long-standing large tumors may show lobulations Histologic Grade cannot be assessed
and radiolucent areas of cystic degeneration. On MRI, grade Well differentiated
the tumor is hypointense on the T1-weighted image and Moderately differentiated, includes
hyperintense on the T2-weighted image. adenoid cystic carcinoma without
Pathology basaloid (solid) pattern
Poorly differentiated, includes adenoid
Grossly (Figure 4), the tumor is greyish white, solitary, cystic carcinoma with basaloid (solid
bosselated and well circumscribed by a thin pseudocapsule pattern
formed by compressed adjacent tissue. Undifferentiated.
Histopathologic examination (Figure 3c) shows the mixture
of epithelial and mesenchymal elements. Focal squamous Management and Prognosis
metaplasia may also be present. The management is complete excision of the tumor. Lateral
orbitotomy is described for the complete surgical removal

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 Ocular Malignancies

of these tumors, however anterior orbitotomy can be

performed especially if the mass is primarily located in the
anterior orbit. Stallard-Wright incision or lid-crease incision
can be used.
To minimize any tumor seeding, adjacent periorbita
should be removed. Removal of the palpebral lobe of the
lacrimal gland is also advocated to reduce the recurrence
rate. However, sacrificing the palpebral lobe may lead to
postoperative dry eye and the need for topical lubricants.
These tumors have high recurrence rate following
incomplete excision or incisional biopsy. The 5-year
recurrence rate for completely excised lesions is 3% and
for incompletely excised tumors, it is 32%. Recurrent
pleomorphic adenoma can also undergo malignant change.
As biopsy of pleomorphic adenomas can have disastrous
Figure 3: Pleomorphic adenoma. (a): A 68-yr female presented
consequences, these tumors should be diagnosed before with right upper eyelid mass with inferior globe dystopia and
surgery, so that biopsy can be avoided. In patients with mechanical ptosis. (b): CT scan, coronal cut shows well-defined
pleomorphic adenoma who have undergone biopsy, the mass in the lacrimal gland fossa. (c): Histopathologic appearance
biopsy track and skin scar are excised in continuity with a of the tumor shows the myxomatous matrix and cords of
epithelial cells. (d): Post operative picture showing resolution of
total removal of lacrimal gland. proptosis and improvement in ptosis.
Adenoid Cystic Carcinoma
Adenoid cystic carcinoma is the most common malignant
epithelial tumor of the lacrimal gland. A bimodal peak in
the fourth and sixth decades is described8. As this tumor (a) (b)
may present at an earlier age, a high index of suspicion
is required for any unilateral mass in the upper temporal
quadrant, in young adults, where it might be mistaken for
pseudotumor9 (Figure 1) or dermoids.
Clinical Features
It usually presents with a shorter history of proptosis,
globe displacement and pain; which may be associated Figure 4(a): Modified Stallard-Wright incision given for anterior
orbitotomy. (b): Tumor is excised with intact capsule.
with motility disturbance, diplopia, ptosis, lacrimation,
numbness and decreased vision. Pain is a characteristic
feature of this malignancy due to perineural invasion. The described: (i) cribriform (“Swiss cheese”) (Figure 1b), (ii)
reported incidence of pain ranges from 37.5 to 79%10. solid (basaloid), (iii) sclerosing, (iv) comedocarcinomatous,
and (v) tubular (ductal), in order of frequency. The “Swiss
Radiologic Features cheese” pattern is associated with longer survival and
CT shows more elongated, irregular mass extending basaloid pattern has poorer prognosis. Perineural invasion
along the lateral orbital wall, with bone invasion11,12. In is frequently observed, accounting for the symptoms of
a series, CT abnormalities in adenoid cystic carcinoma pain and numbness.
consisted of bone erosion (75%), bone destruction (34%),
and calcification (22%). High-resolution CT with bone Management and Prognosis
windows is recommended. Contrast enhancement helps to There are no clear guidelines regarding the optimal
reveal involvement of the dura and intracranial extension. treatment for adenoid cystic carcinoma. Tumor removal
Intracranial extension of the tumor in cavernous sinus and and postoperative radiotherapy comprise the most common
brain can be best assessed by contrast enhanced MRI. treatment. The surgical techniques of tumor removal
The tumor is hypointense on the T1-weighted image and include local resection, en bloc removal, exenteration,
hyperintense on the T2-weighted image. and radical exenteration. Even with the radical approach,
the survival rate is reported to be 20% at 10 years, with
Pathology the median survival of 5 years13. Mortality in this tumor is
Grossly, this tumor is firm, nodular, with ill-defined primarily due to intracranial spread as a result of perineural
borders. Microscopically, five histologic patterns have been invasion and pulmonary metastasis.

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Ocular Malignancies: Lacrimal Gland Tumors: An Overview

Table 3: Clinico-radiological features, management and prognosis of lacrimal gland tumors

Clinical features Imaging features Management Prognosis
Idiopathic Acute onset swelling Diffuse mass with Short course of oral Prompt response to
Inflammatory with periorbital pain, ill-defined margins, corticosteroids steroids is diagnostic
(Pseudotumor) chemosis and lid Moderately enhancing and
edema No bony changes.
Lympho- Insidious onset, Characteristic molds Radiotherapy >95% Achieve
proliferative painless older age around the globe and the (moderate-low dose local control Overall
Mass often bilateral bone without eroding RT) ± Chemotherapy prognosis depends on
histological type.
Benign Painless, without Round to oval, well Complete excision Excellent prognosis
(Pleomorphic inflammation >1 year circumscribed, with periorbita if removed with
adenoma) duration Enlargement of the (Incisional Biopsy is pseudocapsule (5 year
lacrimal fossa without Contraindicated) recurrence 3%)5
invasion of overlying bone
Malignant Painful swelling More elongated mass Biopsy followed by Even with radical
(Adenoid cystic Shorter duration extending along the lateral En bloc resection/ management 10 year
carcinoma) Steroid unresponsive orbital wall with bone Exenteration survival is 20%
invasion. Calcifications are ± Radiotherapy
more common

Post operative radiotherapy in the dose of 50 to 60 Gy
following local resection of the tumor significantly delays
the onset of tumor recurrence and prolongs the survival.
Use of neoadjuvant chemotherapy is also described for
patients with inoperable tumors to shrink them to a more
surgically amenable size. Intra-arterial chemotherapy
with intracarotid cisplatin has recently been advocated
for supplemental management14. Role of adjuvant
chemotherapy is not clear. We have reported survival of
6 and 4 years respectively in 2 patients of adenoid cystic
carcinoma after they received adjuvant chemotherapy
following local resection of tumor15.
Malignant Mixed Tumor
A malignant mixed tumor represents malignant
degeneration of pleomorphic adenoma. It accounts for 4 to
15 % of lacrimal gland epithelial tumors. Font and Gamel
described that adenocarcinoma arising from pleomorphic
adenoma is more common in males, whereas adenoid
cystic carcinoma arising from pleomorphic adenoma is
more often seen in females.
Clinical Features
Malignant mixed tumors usually present in three clinical
ways16,17.
(1) Incompletely excised benign mixed tumor may
develop a sudden recurrence several years later.
(2) Long-standing lacrimal tumor presents with sudden
expansion of the mass, as well as pain and swelling of
the upper eyelid.
(3) De novo presentation, where patient has rapidly
develops symptoms of pain and bone destruction.
Radiologic Features
On CT, lacrimal fossa mass with bone destruction suggests
malignant tumors, which is best seen in bone window. It is
indistinguishable from the other carcinomas of the lacrimal
gland on CT.
Pathology
Malignant mixed tumors have the microscopic features
of a benign mixed tumor with areas of malignant change.
Mostly, the malignant elements are poorly differentiated
adenocarcinoma. Less commonly there are adenoid cystic
carcinoma, squamous cell carcinoma, and, rarely, spindle
cell sarcoma.
Management and Prognosis
A transseptal biopsy should be taken to confirm the
diagnosis if malignancy is suspected in preoperative
evaluation, which should be followed by en bloc resection
of the neoplasm with periorbita. Postoperative radiotherapy
is also recommended. Even with extensive surgery, patient
mortality remains high.
Adenocarcinoma
Adenocarcinoma tends to occur in an older population5
and is associated with a shorter patient survival time than
adenoid cystic carcinoma. It often manifests as a rapidly
growing mass, exceeding the limits of adequate surgical
excision at the time of presentation and metastasize earlier.

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Histopathologically, the tumor consists of pleomorphic
cells with many mitotic figures and lumen formation
with mucin production, which can be demonstrated with
mucicarmine and alcian blue stains.
The shorter survival of the patients with this malignancy
probably is related to early lymphatic dissemination to
regional lymph nodes and pulmonary metastasis. Radical
management with monobloc craniofacial orbitectomy and
regional lymph node dissection is recommended for longer
survival.
Mucoepidermoid carcinoma
It is the most common primary carcinoma of the salivary
glands, however rare to involve the lacrimal gland. It
usually presents as a painless, slowly growing mass of the
lacrimal gland that is sometimes mistaken preoperatively
for benign mixed tumor.
Histologically, these tumors comprise a mixture of
mucus-secreting cells (signet ring cells) interspersed with
epidermoid and basal cells. It is classified as either low or
high grade depending on the number of mucin-producing
cells and degree of differentiation.
Low grade tumors (grade 1) have large, well-differentiated
cells with abundant cytoplasm, a relative paucity of the
epidermoid cells, and no mitotic figures.
High grade tumors (grade 3) have smaller cells with
hyperchromatism and frequent mitotic figures predominated
by epidermoid elements.
The clinical behavior and prognosis parallel the histologic
grading.
Other tumors
The following are the few rare lacrimal gland tumors,
which are infrequently encountered even in busy ocular
oncology practices.
Acinic Cell Carcinoma
Acinic cell carcinoma is a rare tumor of salivary gland
origin and even fewer cases are reported in the lacrimal
gland18. The peak incidence is in the sixth decade with
female preponderance. It usually presents as a painless,
slow-growing mass but can invade intracranially in a more
aggressive manner. Microscopically, there are four growth
patterns described: solid, microcystic, papillary cystic, and
follicular. The solid and microcystic patterns are considered
to be the most common.
Spindle Cell Myoepithelioma
Myoepithelioma is a monomorphic adenoma with
proliferation of only myoepithelial cells, in contrast to
the pleomorphic adenoma which has proliferation of
both epithelial and myoepithelial elements in various
combinations. It is defined as a tumor composed of
Ocular Malignancies
myoepithelial cells with ≤10% ductal elements. These are
benign tumors and biologically behaves like pleomorphic
adenoma.
Oncocytoma (Oxyphil Cell Adenoma)
Oncocytes are also called oxyphil cells because of their
eosinophilic cytoplasm. These cells are found in mucous
membranes such as the caruncle, conjunctiva, lacrimal
sac, and lacrimal glands and increases in number with age.
So the tumor tends to occur in older patients. Benign and
malignant oncocytomas have been reported in the lacrimal
gland. Malignant oncocytoma can be associated with
intracranial extension.
Solitary Fibrous Tumor
Solitary fibrous tumor is a rare spindle cell neoplasm that
has a clinical presentation is similar to that of pleomorphic
adenoma. Careful follow-up is required for this tumor
because there may be recurrence after several years.
References
1. Shields CL, Shields JA, Eagle RC, et al. Clinicopathologic review of
142 cases of lacrimal gland lesions. Ophthalmology 1989;96:431–
35.
2. Reese AB. Expanding lesions of the orbit (Bowman lecture). Trans
Ophthalmol Soc U K 1971;91:85–104.
3. Font RL, Smith SL, Bryan RG.. Malignant epithelial tumors of
the lacrimal gland. A clinicopathologic study of 21 cases. Arch
Ophthalmol 1998;116:613–16.
4. Weis E, Rootman J, et al. Epithelial lacrimal gland tumors: pathologic
classification and current understanding. Arch Ophthalmol. 2009.
5. Rose GE, Wright JE. Pleomorphic adenoma of the lacrimal gland. Br
J Ophthalmol 1992;76:395–400.
6. Vangveeravong S, Katz SE, Rootman J, et al. Tumors arising
in the palpebral lobe of the lacrimal gland. Ophthalmology
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7. Wright JE, Rose GE, Garner A. Primary malignant neoplasms of the
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8. Wright JE, Stewart WB, Krohel GB. Clinical presentation and
management of lacrimal gland tumours. Br J Ophthalmol
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9. Lee DA, Campbell RJ, Waller RR, et al. A clinicopathologic
study of primary adenoid cystic carcinoma of the lacrimal gland.
Ophthalmology 1985;92:128–34.
10. Mafee MF, Edward D, Koeller KK, et al. Lacrimal gland tumors and
simulating lesions. Clinicopathologic and MR imaging features.
Radiol Clin North Am 1999;37:219–39.
11. Lloyd GAS. Lacrimal gland tumours: the role of CT and conventional
radiology. Br J Radiol 1981;54:1034–38.
12. Gamel JW, Font RL. Adenoid cystic carcinoma of the lacrimal gland:
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13. Meldrum ML, Tse DT, Benedetto P. Neoadjuvant intracarotid
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 Ocular Malignancies: Lacrimal Gland Tumors: An Overview

14. Meel R, Pushker N, Bakhshi S. Adjuvant chemotherapy in lacrimal
gland adenoid cystic carcinoma. Pediatric Blood & Cancer 2009; 53
(6): 1163–64.
15. Perzin KH, Jakobiec FA, Lovolsi VA, et al. Lacrimal gland malignant
mixed tumors (carcinomas arising in benign mixed tumors): a
clinicopathologic study. Cance 1980;45:2593–2606.
16. Henderson JW, Farrow GM. Primary malignant mixed tumors of the
lacrimal gland. Report of 10 cases. Ophthalmology 1980;87:466–
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