OSTROSKY A, KLURFAN FJ, GONZALEZ MJ, CAMALY D, VILLA D. PLEOMORPHIC ADENOMA OF THE LACRIMAL GLAND. CASE REPORT. MED ORAL PATOL ORAL CIR BUCAL 2005;10:86-9.

ABSTRACT

Lacrimal gland tumors are rare. We present a case of a 69 year old male patient with a one year history of an orbital tumor (Pleomorphic Adenoma). The tumor was surgically enucleated and patient recovery was very satisfactory, with no signs of recurrence after one year of follow-up. A review of the clinical symptoms, radiologic features, treatment and histopathology of this kind of tumors is also made.

Key words: Pleomorphic Adenoma, benign mixed tumor, lacrimal gland.

INTRODUCTION

Pleomorphic adenoma (“ benign mixed tumor”) is one of the most frequent tumors of the lacrimal gland and constitutes 50% of tumors of epithelial origin (1).

The incidence of this neoplasm is slightly higher in men (60%) than in women (40%) (2,3) and it is more frequent between the third and seventh decades of life, with an average age of 39 years. Cases of pleomorphic adenoma of patients between 6 and 80 years have been reported in the literature (4).

The lacrimal gland and adnexal ocular structures are anatomically comparable to salivary glands and may give rise to the same kind of tumors. Both tumor groups show noticeable similarities with regard to their local recurrence rate (5). As in salivary glands, these neoplasms clinically presents as a painless, slow- growing mass. It also produces slowly progressive proptosis, diplopia, impairment of vision and inferonasal displacement of the ipsilateral eye (6). Most patients have had their symptoms for more than a year (7).

The diagnosis is achieved by clinical and radiographic examination and is confirmed by definitive histological examination after the removal of the mass (4). The dissemination of neoplastic cells in the orbital cavity, which a biopsy could cause, should be carefully avoided (7).

Histopathologically, the pleomorphic adenoma of the lacrimal gland is identical those originating in the salivary glands, as well as in other serous glands of the body (5). The tumor is composed of cords of well-differentiated epithelial tubules, derived from the ducts of the lacrimal gland, within loose mixomatous connective tissue (6).

The most widely accepted treatment by most authors for this kind of tumors is surgical enucleation of the tumoral mass with conservation of its capsule.

A case is reported and a review of the clinical symptoms, radiologic characteristics, treatment and histopathology of this kind of tumors is also made.

CASE REPORT

A 69 year-old white male patient came to our hospital complaining of a firm, nontender, freely movable mass located on the external portion of the right upper eyelid, which had been slowly growing for a year. The mass was not fixed to skin, tarsus or periostium. Visual acuity and globe motility were both normal and the patient did not complaint of diplopia. The patient did have ptosis and slight proptosis (Fig.1). Past medical history was unremarkable.

Computed tomographic scans and magnetic resonance studies showed a well-circumscribed, round, anterior superior orbital mass, with the bulk of the lesion anterior to the lateral and superolateral orbital rim. No bony erosion was found, and there was only slight globe displacement (Figs.2 and 3).

The differential diagnosis included, besides the pleomorphic adenoma, a dermoid cyst and a lacrimal gland duct cyst (dacryop). However, orbital dermoid cysts present early in life and on CT, they classically produce a discrete rounded osteolytic orbital rim defect with sclerotic margins. On the other hand, dacryops characteristically exhibit a grey-blue appearance subconjunctivally, is softer to palpation owing to a thin capsule and may periodically fluctuate in size.

The clinical and radiologic evidence was consistent with a diagnosis of benign mixed tumor arising in the palpebral lobe of the lacrimal gland, and because of that, no incisional biopsy was made. Under general anesthesia and by means of a trans-septal approach ( anterior orbitotomy), the tumor was excised with extreme caution to avoid damage to its capsule. A 3 cm lid incision was performed laterally in the superior lid crease and both sharp and blunt dissection were carried down through orbicularis muscle. A firm, encapsulated mass was removed along with lacrimal gland tissue (Fig.4).

The sample was sent for histological examination and the typical characteristics for mixed tumor were reported: cords of well-differentiated epithelial tubules within loose mixomatous and condroid connective tissue (Figs.5 and 6). The tumor was well circumscribed, and there was no evidence of malignancy.

Four weeks postoperatively the results were very satisfactory (Fig.7). No recurrences have appeared after a year of follow up.

DISCUSSION

As it was previously mentioned, the lacrimal gland and modified gland structures present in the ocular adnexae are anatomically comparable to salivary glands and give rise to the same kind of tumors. The histologic features of epithelial tumors of the salivary glands look like those found in the lacrimal gland. In addition, both tumor groups show similarities with regard to their local recurrence rate. Many controversies have existed regarding the management of lacrimal gland tumors. Various authors such as Rose and Wright (7) have published algorithms for the management of this kind of tumors. Due to the characteristics of our case, an incisional biopsy was obviated and an excisional one was done. A correct diagnosis includes a clinical phase and a radiographic phase (4). The most frequent signs and symptoms of a clinical phase have already been described. A computed axial tomographic scan, especially with a coronal projection, is of great aid. In fact, besides obtaining positive results in more of 80% of cases, it gives more precise information about the real extension and relations of the neoplasia with surrounding structures (4). In cases of pleomorphic adenoma, CT usually shows a well-circumscribed mass with clear definition of the lacrimal fossa (8). Malignant epithelial tumors typically appear as an ill-defined mass and occasionally invade the surrounding orbit. Lymphoid tumors tend to mold to the orbital bone and globe (5). It is then essential a correct clinical and radiological diagnosis in order to avoid an incisional biopsy. An incisional biopsy could injure the capsule, leading to dissemination of tumoral cells in the adjacent orbital tissues and exposing the patient to a risk of relapse of the neoplasia (1,4,6,7), and sometimes to malignant transformation (9,10).

Surgery is essential in the management of pleomorphic adenoma of the lacrimal gland (11) and different types of approaches have been proposed. Depending on the case, an anterior or lateral orbitotomy could be used. In this particular case and due to the anterior location of the tumor, and being a palpebral lobe tumor, an anterior transpalpebral approach was employed, which also avoids some of the associated morbidity of the greater dissection entailed by a lateral orbitotomy (1).

Various kind of lesions can affect the lacrimal gland. The differential diagnosis includes other benign and malignant intrinsic lacrimal gland tumors, and inflammatory lesions (1). In the case described above, the differential diagnosis was not complicated due to the eloquence of the clinical findings and the radiological examinations. The pleomorphic adenoma can be easily differentiated from inflammatory conditions, lymphoid lesions or malignant tumors because these have a short duration of symptoms. Epithelial malignancies are also painfull and there may be bony abnormalities on radiographic studies (1,2,5,7,11).

The presented case has the typical clinical and radiological characteristics of pleomorphic adenomas. It has already been described the importance of a correct diagnosis to avoid, as most authors recommend, the incisional biopsy and the associated recurrences. It is also considered of great importance a careful surgical technique to prevent damaging the tumoral capsule, as well as to perform a close follow up of the patient follow.

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