British Journal of Ophthalmology, 1986, 70, 681-685

Lacrimal sac mucoepidermoid carcinomaJ BLAKE,' JOAN MULLANEY,' AND J GILLAN2

From the 'Royal Victoria Eye and Ear Hospital, and 2Rotunda Hospital, Dublin, Ireland

SUMMARY A single case of mucoepidermoid carcinoma of the lacrimal sac was considered worthreporting as there are only four other cases described in the literature. The importance of thecorrect histological diagnosis and the management are discussed. This appears to be the first studyof the ultrastructure of lacrimal sac mucoepidermoid tumour.

Mucoepidermoid carcinoma is a tumour composed ofneoplastic mucin-producing cells and epidermoidcells. Although it most frequently affects the salivaryglands it has also been reported in the ophthal-mological literature in the conjunctiva'-3, lacrimalgland,4, and lacrimal sac2".

Case history

A 78-year-old woman was seen in December 1979 forexamination of a swelling in the region of the leftlacrimal sac which had been present for somemonths. Her corrected visual acuity was right eye2/60, left eye 6/6. Uncomplicated intracapsularCorrespondence to Joan Mullaney, MD, National OphthalmicPathology Laboratory and Registry of Ireland, Royal Victoria Eyeand Ear Hospital, Adelaide Road, Dublin 2, Ireland.

cataract extraction had been performed on the righteye in 1977, but the central vision had remained poorowing to maculopathy attributed to injury some yearsbefore.A firm, solid, painless mass was palpable in the left

lacrimal fossa. There was no proptosis and no dis-charge on pressure on the sac. X-rays of the orbitsand paranasal sinuses revealed one large ethmoid cellon the left side with smooth margins, but tomographyof maxillary5 and orbital areas revealed no evidenceof bone destruction. At surgery the mass was foundto be adherent to skin and firmly fixed to underlyingperiosteum. When removed it measured 14x8x6mm.

Pathological examination showed a mass of fibroustissue in which lay scattered numerous islands ofsquamous cells (Fig. 1) in different configurations.

Fig. 1 Squamous cell clumps.(H& E, x32).

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Fig. 2 Squamous cells andmicrocystic area. (H & E, x 32). -

i'VThere were foci of microcystoid (Fig. 2) areas whichstained positively for mucin, and many scatteredclear cells could be seen. Bundles ofvoluntary musclewere scattered throughout, with evidence of muscleinvasion as well as some degree of secondary

myositis. In focal epithelial aggregations the cellscould be described as being transitional in type.There was a moderate amount of mitotic activity.The histological diagnosis was that of a typicalmucoepidermoid carcinoma.

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Fig. 3 Electron micrograph. Columnarandpolygonalshaped cells, bound by a basallamina (BL) andforming a lumen (L).The luminal surfaces bear microvilli (MV) and desmosomes are identifiable. Mucin (M) and seromucin (SM) vacuoles arepresent in some ofthe cells (insert). (x2600, inset x9400).

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Lacritnal sac mucoepidermoid carcinoma

Fig. 4 Appearance afterfirst operation-slight epiphora.

Tumour tissue in 1 mm cubes was removed fromthe paraffin embedded specimen and processed forelectron microscopy. The tumour cells, which werepolygonal, elongated, and columnar, were sur-rounded by a basal lamina, to which they appearedattached. Both mucous cells and intermediate cellswere found in close association with occasionallumen formation (Fig. 3). The mucus-secreting cellscontained membrane bound mucin, filled vacuoles,and occasional seromucinous secretory vacuoles.Some cells also contained endoplasmic reticulum.Mitochondria, fine filaments, and glycogen granuleswere either absent or inconspicuous. Desmosomesand tonofilaments were easily identified. The luminalsurface of both the mucus-secreting and the inter-mediate cells were characterised by microvilli.

Examination by a specialist physician revealed nosign of metastatic spread and an ear, nose, and throatexamination revealed no abnormality (Eric Fenelon,FRCSI). Thereafter she remained symptom-free

(Fig. 4) for 31/2 years apart from a brief period ofslight epiphora.

In December 1983 the patient attended again witha firm mass which displaced the eye upwards andforwards and was disposed in crescentric mannerfrom the inner half of the left upper eyelid, aroundthe inner canthus and into the medial two-thirds ofthe lower eyelid.

Ultrasonography showed two deposits on themedial side of the orbit, the more anterior of whichwas situated 6 mm behind the anterior corneal plane.Computerised tomography scan (Fig. 5) (after intra-venous contrast) disclosed an infiltrating, enhancing,soft tissue mass along the medial margin of the leftorbit, extending laterally to the globe and anteriorlyalso towards the medial canthus, the bulk of thetumour being located in the medial interior part ofthe orbit. There was no evidence of bone destruction.A biopsy showed a similar appearance to the original.specimen, the squamous cells being morphologicallyvery prominent. Mucin was also identified.At subsequent operation (Fig. 6), in March 1984,

as much of the tumour as could be removed withoutdamage to the eye was excised. Microscopic exami-nation of this most recent specimen showed muco-epidermoid tumour with considerable dedifferentia-tion and with hyperchromatism and mitotic activity.Normal lacrimal sac mucosa was identified, withtumour in the deeper layers, and there was extensiveinfiltration of muscle.

In December 1984, five years after the patient firstpresented, she retained a visual acuity of 6/9 in theleft eye, though that had now become immobile andshowed lagophthalmos and early signs of exposurekeratitis. She declined further surgery on grounds ofage and general frailty.

rig. D InJultratng, ennancing, soft tissue mass along medial ig. 6 Recurrence oftumour-early stage in surgicaimargin oforbit. exposure.

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Table 1 Summary ofreported cases oflacrimal sac mucoepidermoid carcinoma

Authors Age, Mass Pain Discharge Duration Proptosis Extra- Bone Size of Exent- Follow-up orSex, of ocular destruc- mass in eration presentstatusRace Watery Purulent Bloody symptoms muscle tion on mm

(years) restriction X-ray

Bambirra 58 + + + - - 2 + + - 10x8 + Well2yearsetal.5 M (max. later

Brazilian diam.)Nietal.2 60 + - - - - 3 - - + 20x20 + Died I year

M x 15 laterofChinese cardiovascular

disease. Norecurrence

Nietal.2 40 + - + + + 2 + + + 30x25 + No recurrenceF x25 after l7yearsChinese

Nietal.2 19 + - - + - 1 - - + 30x30 + No recurrenceF x 15 after 5 yearsChinese

Blake 78 + - - - - 1 + + - 14x8 - Well5yearsetal.,this F x6 later, but eyepaper Irish fixed, exposure

keratitis

Discussion

The occurence of this tumour in other sites is welldefined in both the pathological and general surgicalliterature. Seven cases have been reported in theconjunctiva' 3 6. Harry and Ashton7 reviewed lacrimalsac tumours in the files in the Department ofPathology at the Institute of Ophthalmology inLondon over a 20-year period (1948-1967). Thirteencases were found, eight being epithelial in origin,and they concluded that all of these were of trans-itional cell type. They proposed a classification of:type 1 (transitional cell papilloma); type 2 (inter-mediate transitional cell tumour); and type 3 (trans-itional cell carcinoma). Squamous metaplasia was afeature of many of the growths, but mucoepidermoidtumours per se were not described. So far as can beascertained, there are only four reports of muco-epidermoid tumours arising in the lacrimal sac, thefirst case being described in Brazil in 1981 byBambirra et al.5 Three more were reported by Ni et al.(1983) from Shanghai.2 Table 1 summarises thesereports and includes the present case.Ni et al.2 postulate that mucoepidermoid

carcinoma in the lacrimal sac area may arise fromeither lacrimal sac mural serous gland epithelium orfrom the columnar epithelium of the conjunctiva withits goblet cells. These tumours may infiltrate locallyand rarely metastasise; only in very few cases doesinvasiveness assume serious proportions.8 Allpatients reported upon with mucoepidermoidcarcinoma underwent exenteration, and the earlyinvasion of near-by voluntary muscle would tend to

confirm that this may be the required procedure. Thisoption was not readily open to us, since from thestandpoint of visual acuity we were dealing with anonly eye. The advanced age of the patient also arguedin favour of a conservative approach, which in theevent allowed her five years of good vision. Prog-nosis, now that further surgery has been declined, ispoor: vision will be lost through exposure keratitisand optic atrophy.A diagnosis of squamous cell carcinoma was made

in a general pathology laboratory which received thespecimen from the present patient before it wasreferred to this centre, when the mucoid element wasidentified. We believe that more of these growthsmay on further investigation and examination befound to be of mucoepidermoid type, but could havebeen missed because of the rarity of reports of suchneoplasms at this site.

We thank Mr R Lester and Miss M Hurley for technical help, Mr STravers for photographic assistance, and Miss C Tyner forsecretarial work.

References

1 Brownstein S. Mucoepidermoid carcinoma of the conjunctivawith intraocular invasion. Ophthalmology (Rochester) 1981; 88:1226-30.

2 Ni C, Wagoner MD, Wang WJ, Albert DM, Fan CO, RobinsonN. Mucoepidermoid carcinomas of the lacrimal sac. ArchOphthalmol 1983; 101: 1572-4.

3 Searl SS, Kriegstein HJ, Albert DM, Grove AS. Invasivesquamous cell carcinoma with intraocular mucoepidermoidfeatures: conjunctival carcinoma with intraocular invasion anddiphasic morphology. Arch Ophthalmol 1982; 100: 109-11.

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4 Font RL, Gamel JW. Epithelial tumours of the lacrimal gland: ananalysis of 265 cases. In Jakobiec FA, ed. Ocular and adnexaltumours. Birmingham, Ala: Aesculapius, 1978: 787-805.

5 Bambirra EA, Miranda D, Rayes A. Mucoepidermoid tumour ofthe lacrimal sac. Arch Ophthalmol 1981; 99: 2149-50.

6 Rao NA, Font RL. Mucoepidermoid carcinoma of the con-junctiva: a clinicopathologic study of five cases. Cancer 1976; 38:1699-709.

7 Harry J, Ashton N. The pathology of tumours of the lacrimal sac.

Trans Ophthalmol Soc UK 1968; 18: 19-35.8 Thackray AC, Sobin LH. International Histological Classificationof Tumours, No. 7. Histological typing ofsalivary gland tumours.Geneva: WHO, 1972.

Acceptedforpublication 30 December 198S.

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