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The Iranian Journal of Otorhinolaryngology Vol.17, No.41,Autumn-2005

Recurrence of Lacrimal Gland Pleomorphic Adenoma

(Two case report with review of literature)

1Etezad Razavi M.MD,2Saber MoghaddamA.MD, 3KargozarA.MD, 4Sharifi N.MD,5Yazdani A.MD

1,2,3Associated Professor of Ophthalmology,4Assistant Professor of Pathology, 5Assistant Professor of Ophthalmology-Mashhad University of Medical Sciences

Abstract Introduction: In cases with lacrimal gland mixed tumor preoperative delicate clinical and radiological diagnosis lead to proper surgical approach. Incomplete resection of lacrimal gland mixed tumor may be complicated by severe tumor recurrence with the risk of malignant transformation. In this case report, we present 39 years old man with history of transcranial excision of lacrimal gland tumor. Six months before admission progressive proptosis and inferomedial displacement of right globe (approximately 3 cm) has been developed. Soft tissue hypertrophy of eyelids and corneal leukoma developed due to the chronic progressive course of proptosis with chronic corneal exposure. The other case also was a 38-year-old man with significant proptosis and history of two time lacrimal gland tumor excision, one from transcranial approach. The surgical procedure was performed in both cases through anterolateral orbitotomy without bone removal and the whole lesions were removed with pseudo capsule. The vision of the first patient improved from hand motion preoperatively to one meter finger count after surgery, and there was no recurrence in both cases 6 months postoperatively. In general, regarding risk of malignant degeneration and recurrence after incomplete excision or incisional biopsy of lacrimal gland mixed tumor, it is strongly recommended to perform complete excision with psudocapsule in the first surgery. Key words: Pleomorphic adenoma, Lacrimal gland, Tumor recurrence, Benign Mixed Tumor

Introduction

linically, the large majority of lacrimal gland masses represent as idiopathic

inflammatory disease (dacryadenitis), which usually responds to anti-inflammatory medication and does not require surgical intervention and biopsy. Of those lacrimal gland tumefactions which do not present with inflammatory signs and symptoms, approximately half will represent lymphoproliferative disorders, and the other half are epithelial neoplasm's.

Computed tomography scanning is very helpful in evaluating lesions in the lacrimal gland region. CT contour analysis can be used to differentiate inflammatory conditions and lymphoid proliferations from frank larimal gland neoplasm's. Inflammatory and lymphoid proliferations within the lacrimal gland tend to cause it to expand diffusely, making it appear elongated, whereas neoplasm's appearing as isolated globular masses, tend to displace and indent the globe (1).

C

Etezad Razavi M.MD Address: Mashhad University of Medical Sciences. Khatamolanbia Eye Hospital Acceptation date: 81/10/22 Confirmation date: 82/6/16

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The Iranian Journal of Otorhinolaryngology No.41, Autumn-2005

Approximately 50% of epithelial tumors are benign mixed tumors (pleomorphic adenoma), and about 50% are carcinomas. In this report, we introduce recurrence of two cases of benign mixed tumor after incomplete resection by neurosurgical subfrontal craniotomy approach. Both of them operated with lateral orbitotomy approach and complete resection performed with no sign of recurrence after six months. Case report The first case was a 39-years-old man who presented to our clinic with a huge superlateral orbital mass with rapid progression during the last sixth months. The patient had a history of lacrimal gland tumor resection from the orbital roof (by Transcranial approach), ten years ago in the neurosurgery setting. Recently the mass progressively enlarged with displacement of globe inward and downward.In external eye examination, a large nodular mass appeared in the superolateral aspect of the right orbit (in the lacrimal gland region) displacing the globe nasally and inferiorly, accompanied by axial proptosis.Significant protrusion of the right eye with lagophthalmos and incomplete closure of eyelids led to corneal ulcer and corneal opacity (leukoma) (Fig 1a).

A: preoperative

Fig.1: Photographs of the first case with recurrence of pleomorphic adenoma of lacrimal gland Visual acuity of the right eye was perception of hand movement and for the left eye was 20/20. Significant enlargement of the right upper lid and lower lid was related to the chronic and prolonged course of the disorder (Localized Gigantism).

Examination of ocular motility showed significant limitation of ductions of the right eye in upward and outward rotation. In computerized tomography, a large multilobulated lesion with involvement of superolateral region of right orbit can be seen. The tumor diameter was approximately 4 cm. defect of orbital roof bone noted on CT and heterogeneous radiodensity with area of calcification are visible. The globe displaced inferiorly about 3cm that presented on CT and there is no significant sign of any bone erosion. (Fig 2 a&b)

Fig. 2A: Axial View

Fig.2B: Coronal View

Fig 2: Computerized Tomographic Scan of the

first case Regarding to longavity and chronic course with progressive growth of the tumor after primary neurosurgical removal, and also the lesion was painless and CT scan shows no bone erosion with multilobulated pattern, we highly suspected to recurrence of plaomorphic adenoma. So, complete resection of the tumor was done with psuedocapsule performed through the anterolateral orbitotomy incision. The lesion extended from behined and superior of globe to the orbital apex. After removal the size of the tumor was 3x4x4 cm with multilobulated pattern on the surface.

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Recurrence of Lacrimal Gland Pleomorphic Adenoma Etezad Razavi M, and

(Fig 3: a & b).

Fig.3:Intraoperative view of huge lacrimal gland tumor. After complete tumor excision the visual acuity improved from hand motion to one meter counting finger. The eyelids laxity and lateral canthus displacement reconstructed with another surgery (Fig 1b).

B: postoperative

The entire lesion was sent for histopathologic exam, and the report was compatible with pleomorphic adenoma of the lacrimal gland. Light microscopic exam with hematoxilineosin stain revealed epithelial elements with round monomorph- us nucleus without mitosis mesenchymal elements with hyalinzed mixoid stroma with cartilageneous and osteoid differentiation and degenerated bone particles were also reported (Fig 4).

Fig.4:Light microscopy of pleomorphic adenoma (H&E stain, mag 100x) In serial section there was no neural or vascular invasion or mitosis and necrosis related to malignancy(Fig5).

Fig.5: Light microscopy of pleomorphic adenoma (H&E stain, mag 400x) All of the histopathologic findings correspo-nded to recurrence of pleomorphic adenoma of the lacrimal gland after incomplete tumor removal. The second case was a 38-years-old man that came to us with complaints of proptosis and infra displacement of the left eye from a few months prior to his visit. He had a history of two times surgery for lacrimal gland tumor, one from the transcranial approach. Magnetic resonance imaging revealed a large mass extending superior and behind the globe up to orbital apex. Our surgical approach was the same as the first case and after complete removal pathologic exam confirmed recurrence of pleomorphic adenoma of lacrimal gland. The patient has no sign of recurrence after six months.

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Discussion Patients with lacrimal gland benign mixed tumor present with a progressive painless downward and inward displacement of the globe with axial proptosis. Symptoms are usually present for more than 12 months. A firm lobular mass may be palpated near the superior lateral orbital rim, and orbital imaging often reveals enlargement or expansion of the lacrimal fossa. On CT Scan the lesion appears well circumscribed but may have a slightly nodular configuration. Microscopic examination shows varied cellular structure consisting primarily of proliferations of benign epithelial cells and a steroma composed of spindle-shaped cells with occasional cartilaginous, mucinous, or even osteoid degeneration or metaplasia. This variability accounts for the mixed tumor characterization of this lesion. A pseudocapsule circumscribes the lesion, but microscopic nodular extensions into the pseudocapsule account for the tendency of the lesion to recur if an appropriate margin of surrounding orbital tissue is not also removed at the time of excision. Treatment should consist of complete removal of the tumor with its pseudocapsule and a surrounding margin of orbital tissue without a preliminary biopsy. If the capsule of the pleomorphic adenoma is incised for direct biopsy, there is a 32% rate of recurrence, and these recurrences have a significant risk of malignant degeneration. Analysis of the clinical presentation of the lesion and its CT contour helps the surgeon determine if the lesion is likely to be a benign mixed tumor, in which case lateral orbitotomy is required for complete excision as the initial approach.There are confidential research results that show incomplete resection of epithelial lacrimal gland tumors and incisional biopsy of these lesions is hazardous for patients (1). If there are sufficient clinical and radiological criteria leading to encapsulated

epithelial lacrimal gland tumors, primary intervention should consist of complete removal of the tumor without a preliminary biopsy (2, 3,4). Incisional biopsy of the lesion and violation of the capsule of the pelomorphic adenoma increased recurrence rate and risk of malignant degeneration (5). Regarding anatomical location of lacrimal gland in superolateral region of the orbit beneath the superior orbital rim, in spite of extension of large tumors to orbital apex, preferred surgical approach is though anterolateral orbitotomies. When a pleomorphic adenoma is suspected on the basis of clinical and radiological appearances,the tumor with a rim of surrounding normal tissues should be removed without prior biopsy. This ensures excision of any nodules extending outside the main tumor; recurrence following incomplete removal takes the form of multiple scattered nodules (5,6,7,8). Although in our cases the surfaces of lesions are also multilobulated, histologically the tumor is biphasic, with epithelial and stromal elements.The epithelial portion is composed of small ductules with an inner cuboidal to columnar layer and an outer spindle-shaped layer that often contains clear cells. Squamous metaplasia may be present.The outer layers show a gradual transition to mesenchymal tissues, which may display mioxoid, cartilaginous, bony or adipose features (9). Ultra structural examination has shown that ductular cells in pleomorphic adenoma have the characteristic of ductular cells of the normal, and that the stromal cells retain epithelial features in the from of tonifilaments and desmosomes, although occasional cells show myoepithelial features with this filaments and dense bodies (7,8,9,10). In our two cases also, surgery was performed through the lateral orbitotomy with complete resection of tumor.

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Essentially, in all orbital lesions in which the orbital apex and osseous optic canal were involved, the transcranial approach needed to unroof the canal and totally resected the tumor.Otherwise in all orbital lesions limited to the orbital cavity various methods of anterolateral orbitotomies may give the best results. Conclusion The crucial aspect of management of all lacrimal fossa tumors is to suspect pleomorphic adenoma. Although this tumor is histologically benign, incomplete excision will likely result in relentless recurrence and even malignant transformation. Therefore, when pleomorphic adenoma is suspected, a lateral orbitotomy is mandatory. The entire tumor with its pseudocapsule, surrounding levator aponeurosis, conjunctiva and periorbita must be excised enbloc (11,12,13,14,15,16) to avoid recurrences and long-term misery for patients. References 1- Academy of Ophthalmology: Basic and Clinical Science course. Section 7.2000. P.72-73. 2- Albert and Jakobiec. Principle and practice of ophthalmology. Vol3,4. 1998. P. 1956, 2349t,1955-1956. 3- Ostrosky A, Klurfan FJ et al. Pleomorphic adenoma of the lacrimal gland.Case report.Med Oral Patol Oral Cir Bucal 2005 Jan-Feb;10(1):88-9; 86-8. 4- Marshall AF, White DR, Shockley WW. Pleomorphic adenoma in the palpebral lobe of the lacrimal gland. Otolaryngol Head Neck Surg 2005 Jan; 132(1):141-3. 5-Wright JE. Factors affecting the survival of patients with lacrimal gland tumors. Can J Ophthalmol 1983;17:3. 6- Miyazaki T, Yamasaki T et al. Unusual progression of pleomorphic adenoma of the lacrimal gland: case report. Neurol Med Chir (Tokyo) 2005 Aug; 45(8):407-1. 7- Sadick H, Riedel F et al. Benign mixed tumor of the lacrimal gland. Clinical diagnosis and surgical management. ORL J

Otorhinolaryngol Relat Spec 2003 Sep-Oct;65(5):295-9. 8- Tsunoda S, Yabuno T. Pleomorphic adenoma of the lacrimal gland manifesting as exophthalmos in adolescence case report.Neurol Med Chir (Tokyo) 1994 Dec;34(12):814-6. 9- Auran J, Jakobiec FA, Kerbs W. Benign mixed tumor of the palpebral lobe of the lacrimal gland. Ophthalmology 1988;95:90 . 10- Dardick I, Van nastrand A WP. Jeans MTD et al. Pleomorphic adenoma. 1: ultrastructural organization of epithelial regions. 2: ultra structural organization of stromalregions. Hum pathol 1983;14: 780. 11- Henderson j. Orbital tumors. Philadelph- Ia :WB saunders, 1973. P. 402-442. 12-Jones IS. Surgical Consideration in the management of lacrimal gland tumors.Clin plast surg 1978;5:561. 13-Wright JE. Surgical exploration of the orbit in Stewart WB (ed):Ophtalmic plastic Reconstructive surgery. Sanfransisco: American Academy of Ophthalmology ;1984. 14- Sadick H, Riedel F. Benign mixed tumor of the lacrimal gland.Clinical diagnosis and surgical management. ORL J Otorhinolaryn gol Relat Spec 2003 Sep-Oct;65(5):295-9. 15- Fichter N, Schittkowski M, Guthoff RF. Diseases of the lacrimal gland Ophthalmologe 2005 Apr;102(4):399-423; quiz 424-5. 16- Ohtsuka K, Hashimoto M, Suzuki Y. A review of 244 orbital tumors in Japanese patients during a 21-year period: origins and locations. Jpn J Ophthalmol 2005 Jan-Feb;49(1):49-55.

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