British Journial of Ophthalmology, 1979, 63, 600-606

Clinical presentation and management oflacrimal gland tumoursJOHN E. WRIGHT, WILLIAM B. STEWART,' AND GREGORY B. KROHEL2From the Orbital Clinic, Moorfields Eye Hospital, City Road, London

SUMMARY This paper presents the clinical and pathological findings in 40 consecutive patientsseen with primary tumours arising from the lacrimal gland. Twenty patients had a benign mixed-cell tumour. They presented in a recognisable clinical manner with a painless mass in the region ofthe lacrimal gland which slowly enlarged over a period of at least 1 year before consultation.Twenty patients had a carcinoma of the lacrimal gland. They had a short history and experiencedpain. On clinical grounds they could not be distinguished from inflammatory lesions in the regionof the lacrimal gland. The method of treating these 2 groups of patients is described and methodsof dealing logically with their problems are suggested.

This communication presents an analysis of themode of clinical presentation of a large series oflacrimal gland tumours. The correct managementof lacrimal fossa lesions depends on recognisingthe characteristic clinical presentation of benignmixed cell tumours so that a biopsy is avoided andthe capsule of the tumour remains intact. Excisionof the whole of the lacrimal gland together with thetumour avoids any risk of seeding tumour cells intothe adjacent tissues with the possibility of a recur-rence with malignant potential (Rubin, 1947;Zimmerman et al., 1962; Waller et al., 1973).

All other lesions in this region, apart from acutedacryoadenitis and dermoid cysts, require incisionalbiopsy through an anterior approach so that adefinitive histological diagnosis can be made.After such a biopsy histological as well as clinicalcriteria can be used to decide which type of treat-ment is most appropriate.

Material and methods

Clinical records and histopathological findings of108 patients with lesions of the fossa of the lacrimalgland seen in the Orbital Clinic at Moorfields EyeHospital, London, from 1968 to 1979 were reviewed

"Department of Ophthalmology, Pacific Medical Center,San Francisco, California, USA.2Neuro-Ophthalmology Service, Department of Ophthal-mology, Albany Medical College, New York, USA.

Correspondence to John E. Wright, FRCS, MoorfieldsEye Hospital, City Road, London EC1V 2PD.

in an attempt to identify clinical characteristics thatwould allow recognition of benign mixed lacrimalgland tumours from other lesions of the supero-temporal orbit. There were 40 cases of benign ormalignant epithelial lacrimal gland tumours. Allhad plain radiographs and a histological diagnosis.The other cases were nonepithelial lesions such asacute and chronic nongranulomatous inflammation,granulomatous inflammation, ideopathic inflamma-tory pseudotumour, lymphoma, and dermoidcysts. Orbital involvement in some of these caseswas not confined to the lacrimal fossa.

Evaluation of patients presenting with lacrimalgland fossa symptoms and signs included a generalmedical examination, blood studies, and plain andtomographic radiographs of the orbits. Orbitalultrasonography and computerised tomographywere performed in selected cases.

Results

Twenty patients in the series had benign mixedlacrimal gland tumours. They comprised 11 menand 9 women, with an age range of 21-68 years(Table 1). The duration of symptoms was greaterthan 1 year in 19 patients, only 1 patient with abenign mixed tumour had pain. Radiologicalabnormalities were seen in 18 patients; all hadenlargement of the lacrimal gland fossa due topressure erosion of the overlying bone. Occasionallythis erosion was sufficient to breach the orbitalwall, with exposure of the dura of the anteriorcranial fossa. In addition sclerosis of bone was

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Clinical presentation and management of lacrimal gland tumours

Benignmixed celllacrimalglandtumours(20)

3565

Malignant epithelial tumours (20)

Malignantmixed celltumours(2)

Adenocysticcarcinomas(9)

2

2

2

Othercarcinomas(9)

24

11 - 4 59 2 5 4

1 3

_ 2t 2

1 1

316

3

2

3

1 1 8 6

19 1 1 3

nerve. Radiological changes were encountered in17 patients. Thirteen showed a pressure erosion ofoverlying bone; 4 patients had demonstrabledestructive changes in adjacent bone on plain ortomographic views. Of the 13 patients with pressurechanges, 3 had calcification within the lacrimalgland, and 2 had sclerotic changes in the bone adja-cent to the expanding lacrimal fossa.Nine of the malignant neoplasms were considered

inoperable at the time of presentation at MoorfieldsEye Hospital, either because of extensive involve-ment of bone or evidence of spread beyond thelimits of even the most extensive surgical approach.All the cases in which tumour had spread beyondpotential surgical margins had an interval fromtheir initial presentation to an outside ophthalmicservice until referral and/or definitive tissue diag-nosis of greater than 3 months. The average intervalwas 4 months. For those patients who had resec-table tumours the interval was only 2 months.The nonepithelial lesions in the review included

acute dacryoadenitis, idiopathic inflammatorypseudotumours, and lymphomatous lesions. Theselesions represent a broad and varied spectrum ofclinical presentation. Ages ranged from the first tothe eighth decades. The duration of symptoms wasgenerally less than 6 months. Inflammatory signsand discomfort were relatively common. X-rayabnormalities were most unusual.

Discussion

X-ray findings(plain andtomographic)Normal

PressureDestruction

2

18 22

6

1

5

3

present in 1 patient in association with pressurechanges.

Malignant neoplasms accounted for 20 of the40 patients with primary epithelial tumours origi-nating in the lacrimal gland. There were 9 adenoidcystic carcinomas, 2 malignant mixed cell tumours,and 9 carcinomas of other types. There was a wideage distribution. It was noteworthy that 7 patientswere under 30 years of age, and 4 of the 7 had beentreated elsewhere for suspected orbital cellulitis.Fifteen patients had had symptoms for less than 1

year. Only 1 of the patients with a history of longerthan 12 months had an operable tumour. This wasa case of carcinomatous change in a pre-existingbenign mixed tumour. Fifteen patients had noticedpain, and 3 of these patients had associated lossof sensation in the distribution of the frontal

It is essential when dealing with lesions of thelacrimal gland that a neoplasm arising from theepithelium of the gland is recognised as soon aspossible. The survival of the patient depends on thecorrect choice of treatment for the lesion. Analysisof cases presented in this communication has shownthat, while carcinomas of the lacrimal gland cannotbe distinguished from other rapidly expandinglesions in this region other than by histologicalexamination, benign mixed-cell tumours can bereadily recognised on clinical grounds.The pattern of presentation of benign mixed

lacrimal gland tumours is characteristic. Theypresent in patients in their late 20s to early 60swith a slowly progressive painless swelling in theupper lid without inflammatory symptoms or signs.A palpable mass is often in the upper temporalquadrant of the orbit (Fig. 1). A careful detailedhistory will reveal that the duration of symptomsis more than 12 months. Radiographs usually showenlargement of the lacrimal fossa without invasionof overlying bone (Fig. 2) (Jones and Pfeiffer, 1954;Newton, 1962; Lloyd, 1975). Careful tomographicstudy may disclose this characteristic change when

Table 1 Clinical details of 40 patients withlacrimal gland tumours

Age(years)

0-

20-30-40-50-60-70

SexM

F

Duration ofsymptoms(months)0-

3-6-9-12-

18-

PainPresentNot present

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plain x-rays are negative (this occurred in 1 patientin our series). Coronal computerised tomography(CT) scans will confirm the presence and extentof the tumour (Fig. 3). Although the clinicalpresentation of cases of benign mixed lacrimal glandtumours has been described in a number of excellentreviews (Forrest, 1954; Davies, 1954; Reese, 1956;Zimmerman et al., 1962; Henderson, 1973), thecharacteristic clinical presentation of these tumourshas not been fully recognised.

Occasionally a benign mixed lacrimal glandtumour may present in a different way. The patientgives a short history, there is a readily palpablemass, and radiographs are normal. This tumourarises from the palpebral lobe of the lacrimal glandand produces a slowly enlarging mass in the outerthird of the upper eyelid. In this situation the mass

Fig. 3 Coronal CT scan demonstrating benign mixedcell tumnour of the right lacrimal gland causinge-~~~~~~~~~.'downward displacement of the globe

is noticed at an early stage, and because it liesanterior to the lacrimal fossa there is no boneerosion. Inevitably the mass is either biopsied orremoved and the true nature of the lesion discovered

_Vz?-rfBl- _ by frozen section or routine histology. One such5_ case is included in this communication.Malignant tumours arising from the lacrimal

gland in our series had 2 characteristic features-ashort history with a rapidly worsening course andpain (Fig. 4). Both these features have been noted

Fig. 1 Patient with benign mixed cell tumour in before. Two-thirds of the patients in Zimmerman'sright orbit. Fullness of the right upper eyelid and series with carcinoma unrelated to mixed tumoursproptosis noticed for 18 months had symptoms of less than 9 months when they first

Fig. 2 Plain radiograph oforbits showing expansion ofright lacrimalfossa (arrowed)produced by the slow growth ofthe benign mixed tumour

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Clinical presentation and management of lacrimal gland tumours

Fig. 4 Patient aged 31 with an adenoid cysticcarcinoma. Painful mass for 6 months

presented with orbital complaints. The tendency forpatients with malignant tumours to experience painin a high percentage of patients was also noted byZimmerman and Forrest. Radiographic findingsmay also help in distinguishing between themalignant and benign lacrimal gland tumour.Carcinomatous lesions often enlarge so rapidly thatradiographs in the early stages are normal. Later,enlargement of the lacrimal fossa, with or withoutdemonstrable invasion of bone, may be seen. In3 patients calcification within the malignant tumourwas demonstrated (Fig. 5). In all such patients it isimportant that both axial and coronal tomogramsare obtained so that the structure of the bones inrelation to the tumour can be examined in some

detail. This type of assessment has now beensupplemented by coronal and axial CT scans.

Fig. 5 Oblique radiograph of right orbit showingexpansion of the lacrimal fossa by a partially calcifiedadenoid cystic carcinoma

Thus an overall picture of the extent of the lesioncan be made.

Other lesions affecting the lacrimal fossa must beconsidered in the differential diagnosis. Acutedacryoadenitis, although described by Duke-Elderand MacFaul (1974) as a rare entity, occurs fairlyfrequently. Patients present with a short historymeasured in days rather than months. The upperlid is red, swollen, and extremely tender, and thereis usually some conjunctival chemosis with anassociated purulent discharge. The preauricularand cervical nodes are often enlarged and thepatient pyrexial. Radiographs are invariably nor-mal, and blood studies frequently reveal a raisedwhite cell count. This type of dacryoadenitis shouldsettle rapidly with appropriate systemic antibiotictherapy. Secondary dacryoadenitis related to mumps,infectious mononucleosis, or herpes zoster does notrespond to antibiotic therapy and is usually seen inassociation with characteristic systemic abnormali-ties. Chronic dacryoadenitis may follow acuteinflammation or be due to sarcoidosis or some ofthe odd inflammatory reactions which merge intothe pseudotumour group. It is this group of patientswho are difficult to distinguish from those withearly malignant tumours arising from the lacrimalgland.

Inflammatory pseudotumours in the region ofthe lacrimal gland are relatively common. Althoughthey are rare in the very young, the age variation iswide (Chavis et al., 1978). Most commonly thesigns and symptoms are similar to those seen inacute dacryoadenitis, though the patient is apyrexialwith a normal white cell count. Some patients havea slowly enlarging mass which may not be tender,though the pain is frequently noticed. Plain radio-graphs are invariably normal, and CT scans willdemonstrate the size and extent of the mass. Ultra-sonic scans can provide useful information, thepattern of echoes placing the tumour in the lym-phoma-pseudotumour group.Lymphomatous lesions include a broad spectrum

from reactive lymphoid hyperplasia to malignantlymphomas of various types. They most commonlyoccur in adults with a progressive swelling of theeyelid over a period of several months. Inflammatorysigns and symptoms are extremely uncommon. Afirm nontender mass can usually be palpated in theregion of the lacrimal gland. The presence of apurple pink fleshy lesion underneath the conjunctivaof the upper fornix is characteristic of this lesion.The results of investigations are similar to those seenin inflammatory pseudotumours.Dermoid cysts may also occur in the region of

the lacrimal gland and cause some confusion. Peakincidence is in the first decade. Characteristically,

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a smooth mass may be palpated in the upper outerquadrant of the orbit, but in some cases the cystmay lie deeply within the orbit, and the onlyabnormal physical sign is proptosis. The patientspresent with a swelling of the upper eyelid or withprogressive proptosis. On rare occasions the cystmay become inflamed and produce a similar clinicalpicture to acute dacryoadenitis. Plain radiographsprovide the diagnosis. A sharply defined cyst-likebone defect, sometimes with sclerotic margins, ischaracteristic. Benign mixed tumours and some

malignant lacrimal tumours cause a more genera-lised expansion of the lacrimal fossa. An invasivecarcinoma will produce an ill-defined osteolysisquite unlike the picture of a dermoid. CT scans

frequently aid the diagnosis by revealing the cysticnature of the lesion and in some cases a fluid levelwithin the cyst produced by sebaceous material andlipid. Ultrasonic scans should also show the cyst,so that in most cases a preoperative diagnosis iscorrectly made.

Patients with lacrimal fossa lesions can be conve-

niently divided into 2 groups on clinical grounds.The first group are patients with a suspected benignmixed tumour which is confined to the lacrimalfossa. These patients have a history of longer than12 months, radiographic evidence of expansion ofthe lacrimal fossa, and no history of pain. Thesecond group comprises patients with a variety oflesions, lacrimal gland carcinomas, lymphomas,pseudotumour, and other inflammatory lesions.They have a short history, often with pain, and mayor may not have abnormal radiographs.The importance of recognising the first group on

clinical grounds cannot be emphasised too often.The temptation to biopsy the lesion should beavoided, and total removal of the whole of thelacrimal gland through a lateral orbitotomy can beplanned. The choice of approach to the gland is ofprime importance, for the exposure should be suchthat the palpebral lobe and the structures of theeyelid can be dissected out under an operatingmicroscope. It is essential that in removing thelacrimal gland plus tumour the capsule of the tumouris not touched and that periosteum overlying thetumour is removed at the same time. The modifiedlateral orbitotomy approach offers the only way toachieve the correct and adequate removal of thetumour. A transcranial anterior orbitotomy or theBerke lateral orbitotomy makes it extremely difficultor impossible to avoid a subtotal or piecemealremoval of the gland and tumour.

Rupture of the capsule of a benign mixed celltumour affects the prognosis adversely because ofseeding of the tumour cells into the surroundingtissues. Recurrences are thus inevitable, and in

many cases, although such recurrences are histo-logically nonmalignant, malignant changes canoccur. In patients in whom the recurrences remainbenign the outcome is often indistinguishable fromthat seen in a true carcinoma. The tumour cellsinvade the apex of the orbit and the surroundingbone. A painful and lingering death ensues, for inmost cases the tumour spreads beyond the line ofsurgical resection, and, although radiotherapy maybe used as a palliative measure, benign mixed celltumours are relatively radioresistant.

Patients with a very short history of a lacrimalfossa swelling and associated inflammatory signscan reasonably be treated with a short course ofantibiotics. Resolution within 2 to 3 weeks wouldbe the expected course. Unless the mass shows aprogressive decrease in size, early incisional biopsyshould be done. Patients with a history of a fewmonths' swelling in the region of the lacrimal glandwith or without inflammatory signs or abnormalx-rays should be biopsied forthwith.One other group of patients can be identified in

whom an early biopsy is indicated. These arepatients who have a history of longer than 12months but do not fit into the clinical patternpresented by the benign mixed cell tumours. Theyoften complain of pain and show radiological evi-dence of invasion of the overlying bone or evidenceof calcification within the tumour. A biopsy shouldbe obtained in all these cases through a transseptalincision. The extraperiosteal approach shouldnever be used, for the integrity of the periostealbarrier must be maintained to prevent possibleseeding of the extraperiosteal space by malignantcells. By following this regimen a definite diagnosiswill be reached at an early stage in the clinicalevaluation. Such diverse lesions as inflammatorymasses, lymphomas, and carcinomas will be accur-ately identified and the appropriate treatmentstarted.

Patients found to have adenoid cystic carcinomasand other malignant epithelial neoplasms should beevaluated to determine the extent of the tumour.Where there is evidence of restriction of the tumourmass within the periosteal barrier and withoutinvolvement of the orbital apex, a radical resectionof the area can be undertaken. The skills of aneurosurgeon and plastic surgeon as well as the ofophthalmic surgeon should be united in such asurgical operation. Surgical resection in these casesshould include portions of the lateral and superiororbital walls as well as removal of the lids andorbital contents. A report describing the criteria ofoperability, recommended surgical approaches, andthe results of the experience with malignant lacrimalgland neoplasms at Moorfields Eye Hospital is in

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Clinical preseintationi anid matnagemenit of lacrimal glaiid titmoiars

Table 2 Flowsheet of macnagement of lacrimal gland/fossa masses based on durationi of symptoms andradiographic findings

Duirationi0/ X-raysylmp/tomilis findinigs

Withinflammatorysigns

INormalIWithoLutI inflammatory

Short |signs

Pressurechanges

CAbnormalI Destructive

Long

C changes

Normal --

0 Pressure*|changes -

Abnormal gDestructive

l changes -

lImprovedwithoutmass

2-3 Weeks Jantibiotictreatment Improvcd,

unimprovedswith mass

Incisional biopsy

Withregression

Observe for )- regressionof mass

Without Incisional( regression -biopsy

Incisional biopsy

Incisional biopsy

En bloc excisional biopsy

En bloc excisional biopsy

Incisional biopsy

Short duration less than 12 months. Long - duration greater than 12 months. Incisional biopsies done via trans-septal approach. En blocexcisional biopsy done via lateral orbitotomy.*Characteristic presentation of benign mixed cell lacrimal gland tumouir.

preparation. Radiotherapy and chemotherapy may

be considered in those cases in which spread hasoccurred beyond even these wide surgical margins.The outlook for these patients is extremely poor

(Forrest, 1954; Zimmerman et al., 1962; Forrest,1971; Henderson, 1973; Ashton, 1975).This approach, graphically summarised in Table

2, diminishes the need for histological diagnosis byfrozen section and permits decisions on extensivesurgical removal of a neoplastic lesion to be basedon evaluation of permanent appropriately fixedand stained microscopic sections. Furthermore, therisks of incisional biopsy of a benign mixed tumourand its subsequent potential recurrence are mini-mised. This risk cannot be completely eliminated,for occasionally a patient with a benign mixed-celltumour arising in the palpebral lobe will presentwith a short history of a slowly enlarging mass

similar to a chalazion, and in these circumstancesinvariably the lesion is approached directly.No protocol can provide foolproof solutions to

the complex decisions facing the surgeon. Nonethe-less, this diagnostic approach based primarily on

clinical history and radiographic findings, doesprovide a logical approach to the problem oflacrimal gland and fossa lesions. In part the dismalprognosis of malignant lacrimal gland tumours isrelated to delay in diagnosis, with subsequent spread

beyond the limits of surgical excision. We thinkthat an improved survival rate can be achieved byan aggressive attitude towards early biopsy followedby extirpative surgery.

Our thanks are due to the various consultant ophthalmicsurgeons who referred the cases to the Orbital Clinic, toDr G. A. S. Lloyd for his help with the interpretation of theradiographs, the Department of Medical Illustration forthe preparation of the clinical photographs, and to Mrs S.J. Cole for her assistance in the preparation of the paper.

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Davies, W. S. (1954). Pleomorphic adenoma and adeno-carcinoma of the lacrimal gland with report of thirteencases. Transactions of the A merican OphthaltnologicalSociety, 52, 467-495.

Duke-Elder, S., and MacFaul, P. A. (1974). The ocularadnexa. In Duke-Elder, S. (ed.), Systetn of Ophtihallnology,vol. XIII, pt. 2, pp. 601-622.

Forrest, A. W. (1954). Epithelial lacrimal gland tumours;pathology as a guide to prognosis. Transactions of theA merican Academy of Oplitlialniologi' anid Otolartngology,58, 848-866.

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