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Abstract. INTRODUCTION: Pleomorphicadenoma of the lacrimal gland is uncommon but itis the most common benign epithelial tumor ofthis gland. In the literature few cases have beenreported in patients aged between 6 years and 80years with a mean age of 39 years. A correct diag-nosis and treatment is fundamental in order toavoid a relapse and sometimes their malignanttransformation. An incisional biopsy is better to beavoided because it could injure the capsule, lead-ing to dissemination of tumoral cells in the orbitaltissues with a recurrence rate of 30% over 5 years.

AIM: This papers want to support the use ofmini-invasive surgery for the treatment of orbitallesions when it is possible.

MATERIALS AND METHODS: We report twoclinical cases of pleomorphic adenoma affectingthe lacrimal gland treated with two differentsurgery approaches. The radiographic and photo-graphic documentation of the patients was col-lected in the pre-and post-operatively. All patientsunderwent a CT scan and MRI.

CONCLUSIONS: This lesions requires a well-grounded clinical and therapeutic protocol to avoidthe risk of malignant transformation or disease re-currence, very dangerous at this site. CT scan andMRI scan are very important to recognize differenttypes of lesions involving the lacrimal gland andfossa. A mini-invasive surgery reduces hospitaliza-tion, risk of complications, surgical times andbleedings and guarantees an excellent functionaland esthetic result when performed by a skilledsurgeon.

Key Words:Pleomorphic adenoma, Lacrimal gland, Epithelial

tumor, Mini-invasive surgery.

Introduction

The lacrimal gland shares similar origins tothe salivary glands. It is an exocrine gland andconsists in an orbital lobe and in a palpebral lobeseparated by the aponeurosis of the levator palpe-brale superioris muscle. There is also the acces-

Pleomorphic adenoma of the lacrimal gland:two clinical cases

C. RINNA, G. REALE, F. CALVANI, V. CALAFATI, F. FILIACI, E. RICCARDI,V. RAMIERI, F. CASCINO, P. CASCONE, C. UNGARI

Department of Maxillo-Facial Surgery, Policlinico Umberto I, Sapienza University of Rome, Italy

Corresponding Author: Emiliano Riccardi, MD; e-mail: emiliano.riccardi@hotmail.it

sory lacrimal gland located in the lamina propriaof conjunctiva. It helps producing the tear fluid,very important for the maintenance of a healthyocular surface. In the lacrimal gland pathology awide variety of causative factors are assumed tobe possibly involved, like: aging, sex steroid hor-mone, neurotrophic factors, apoptosis, pituitary-depend factors, infections, smoke, autoimmunereaction.Pleomorphic adenoma (PA) is the most com-

mon benign epithelial tumor of the lacrimalgland1-5. It is rare and risk factors are still un-known. It always develops by the deep orbitallobe, rarely from the palpebral lobe6-8, extremelyrarely from the accessory lacrimal gland9, but itcan be located everywhere there is glandular tis-sue. The authors present two cases of lacrimalgland PA treated by different surgery approaches,in the Department of Cranio Maxillo FacialSurgery, University of Rome Sapienza.

Case IA 50 years-old female was referred to our De-

partment with a four months history of diplopiaand progressive loss of sight in the left eye. Onthe examination she had an exophthalmus, a defi-ciency in the left upward gaze (Figure 1A) andorbital dystopia. The conjunctiva was normal.She referred no pain and a history of surgery (inanother hospital) for a left orbital mass definedas a fibrolipoma by its histology. After a carefulexamination, CT scan of the orbits was neededfor a better comprehension of the problem and itshowed: by the upper-external corner of theleft orbit there is an oval solid inhomogeneousmass with regular margins that compresses anddisplaces anteriorly the ocular globe (Figure2A). Thus, in order to better define the extensiontrough surrounding soft tissues, a MRI scan wasperformed and it showed: an expansive ovalmass in the left orbit with regular margins thatcompresses and displaces anteriorly the ocular

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Pleomorphic adenoma of the lacrimal gland: two clinical cases

Figure 1. A, Patient with pleomorphic adenoma of the lacrimal gland showing shift of the leveling of the interpupillary line,conjunctival chemosis and limitation of the left eye in the upward movement. B, Patient three months postoperatively.

Figure 2. A, Axial computed tomography demonstrates an encapsuled mass localized in the upper external portion of the leftorbital cavity. B, Postoperative CT scan didnt show any residual lesion.

globe without evidence of local infiltration.Because there wasnt any radiological and clini-cal evidence of malignancy and a total excisionwas possible, we didnt perform a pre-operativebiopsy. By a coronal approach, after a partial re-section of the temporal muscle, we exposed andperformed an osteotomy on the left orbit lateralpillar. Once the oval mass was exposed, with itsregular margins and a good cleavage plane, wecompletely removed it without drop the capsule.Surgery ended with the orbital pillar reposition-ing, fixed by plates and screws. Histopathologyrevealed that it was a pleomorfic adenoma (PA).After four days the patient was discharged. Fol-lowing CT and MRI scans showed signs of theprevious surgery without any evidence of recur-

rence (Figure 2B). There have been no signs ofrecurrence after a 7 years follow-up and shesstill free of any ocular signs and symptoms(Figure 1B).

Case IIA 44 years-old woman presented at our Depart-

ment with a short history (3 months) of left exoph-thalmos (Figure 3A). The patient was also visitedby an oculist who performed ocular ultrasound thatshowed the presence of a left retrobulbar cysticmass. Ocular examination was unremarkable ex-cept for the left exophthalmos. The conjunctivawas normal. No pain was referred. So the patientunderwent a MRI scan that revealed: an expan-sive mass on the upper-lateral region of the left eye

Figure 3. A, Preoperative photograph shows exophthalmus of the left eye. B, Postoperative photograph after four months: thescar is well hide in the eyelid fold.

Figure 4.A, Coronal CT demonstrating a lobulated mass of the orbital lobe of the left lacrimal gland. B, Postoperative CT scan.

we reached a good functional and esthetic resultwithout any complications (Figure 3B). Macro-scopically the tumor was 2.3 cm long and waswell delimited within a thin capsule. A pathologi-cal diagnosis of PA of the lacrimal gland wasmade. After 3 years the patient remains free fromdisease as showed in post-operative CT and MRIscans (Figure 4B).

Discussion

Pleomorphic adenoma (PA) is the most com-mon benign tumor of the lacrimal gland10. In theliterature few cases have been reported in patientsaged between 6 years11 and 80 years12 with a meanage of 39 years13. A correct diagnosis and treat-ment is fundamental in order to avoid a relapseand sometimes their malignant transformation. Anincisional biopsy is better to be avoided because itcould injure the capsule, leading to dissemination

with definite and regular margins, without any evi-dence of infiltration through the lateral and superi-or rectus muscles. Moreover in order to betterdefine the bone extension of the mass, we per-formed a CT scan that showed: an expansivemass on the upper-lateral region of the left eye(about 2.2 cm) without erosion of the orbitalwalls (Figure 4A).Considering the imaging studies we chose to

remove the lesion again without a pre-operativebiopsy because the mass didnt show malignancyon the clinical and radiological exams as sur-rounding soft tissue invasion or bone erosion orirregular margins. So we performed a skin inci-sion following the skin crease of the superioreyelid, then an osteotomy of the superior-exter-nal corner of the left orbit in order to have a bet-ter sight of the surgery field. We completely re-moved the mass without damaging the capsuleand shortening both the surgery time and post-operative time of discharge (just 2 days!). Also

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Pleomorphic adenoma of the lacrimal gland: two clinical cases

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Conclusions

This paper aims to support the use of mini-in-vasive surgery for the treatment of orbital lesionswhen it is possible. With the advent of brain CTand MR scans, the orbital tumors are more easilydisclosed and characterized. These studies arevery important to recognize different types of le-sions involving the lacrimal gland and fossa, butits very difficult to differentiate each specificdisease on the basis of image characteristicsalone. A careful clinical evaluation and a patho-logic study (when it is indicate and possible) areneeded.In the first case we performed an invasive sur-

gical approach in order to have a wide vision ofthe operating field and avoiding a recurrence or amalignant trasformation, because the patient hadalready been treated. In the second case after acareful clinical and radiological study, we per-formed miniinvasive approach with a shortsurgery, completely removing the tumor andwithout intra and postoperative complications anda short hospitalization (2 days). The functionaland esthetical results were very good. Therefore,after an appropriate clinical and radiologicalstudy, wed like to promote, when possible, a mi-ni-invasive surgery, which is better tolerated,shortens the hospitalization, reduces the risk ofcomplications, surgical times and bleedings andguarantees an excellent functional and esthetic re-sult when performed by a skilled surgeon.

References

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of tumoral cells in the orbital tissues with a recur-rence rate of 30% over 5 years5,14. Though, it isvery important performing a complete removalwithout injuring the capsule, the lacrimal arteryand the lacrimal nerve, a branch of the ophtalmicdivision of the trigeminal nerve. The diagnosis isachieved by definitive histological examination af-ter the excision of the mass16.About the surgical approach for orbital le-

sions, it varies depending on the site of origin,the extension and the histopathological diagno-sis (when it is indicate and possible). In 1996Rubin et al17described different approaches forthe treatment of orbital disease by ophthalmolo-gists, neurosurgeons, and maxillofacial sur-geons. Seema et al18 treated 32 cases of PA ofthe lacrimal gland with anterolateral orbitotomythrough a Stallard-Wright incision and removedthe tumor along with the periosteum and a rimof normal tissue.We prefer, when possible, not to use these kind

of complex surgical approaches that raise the riskof post-operative complications, prolonge the hos-pitalization with a mediocre esthetic result and along surgical time. Porter et al19, like us in the sec-ond case, excised en bloc a PA of the palpebrallobe of the lacrimal gland with a superior skincrease incision and the patient had no complica-tions and remains asymptomatic at the 8 monthsfollow-up. Clinically PA presents as a well cir-cumscribed, slowgrowing, painless swelling massthat can cause ptosis, proptosis, exophthalmus,enophthalmus, orbital distopy, diplopia and re-stricted eye motility20. In our first case the patientpresented with four months history of progressiveloss of vision and diplopia. In the second case theonly sign was exophthalmus. We both treatedthem by tumor excision without previous biopsyto avoid recurrences and malignant transforma-tions. We also performed pre-operative CT andMRI scans in order to better define the lesion. CTscan is very important because its high sensibilityfor the presence of calcification and bony erosion,sclerosis and destruction. A disadvantage of CT isthe inevitable exposure of the lens to irradiation.Magnetic resonance is superior in delineating theextent of soft tissue involvement. Disadvantagesare the lower sensitivity for the presence of calcifi-cation, the imaging of the bone and the long imageacquisition times.In our opinion, in order to perform an appro-

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C. Rinna, G. Reale, F. Calvani, V. Calafati, F. Filiaci, E. Riccardi, et al.