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Sir,

Pleomorphic adenoma is the most common epithelial tumour of the lacrimal gland, accounting for half of all epithelial lesions in the lacrimal fossa.1 It is extremely rare in childhood. Only eight cases are previously reported in the literature.2,3,4,5,6,7 We report a further case in a young teenager whose presentation mimicked that of an inflammatory or infectious dacryoadenitis rather than a neoplastic process.

Case report

A 14-year-old boy had a 3-year history of intermittent swelling of the right eyelid.

This had become progressively worse over the last 6 months. It was unresponsive to broad-spectrum antibiotics prescribed by his general practitioner for a presumed infectious dacryoadenitis.

Examination revealed normal visual acuity in both eyes, an S-shaped ptosis of the right eyelid and mild erythema (Figure 1a). A firm nontender immobile mass was palpable at the superolateral rim extending from the lacrimal fossa. There was 2 mm of proptosis and ocular motility was normal. A CT scan revealed a well-circumscribed mass (Figure 1b and c) in the lacrimal fossa. There was bony remodelling of the fossa suggesting chronicity, although no evidence of bony erosion. A fine-needle aspiration cytology using a 23 gauge 0.6 mm × 30 mm needle was performed as an outpatient procedure. Cytologically, the aspiration showed a cellular smear consisting of plasmacytoid myoepithelial cells with little myxoid stroma and no evidence of malignancy. This was consistent with a pleomorphic adenoma of the lacrimal gland (Figure 2a).

Figure 1
figure 1

(a) Clinical appearance of 14-year-old boy with erythema, swelling, and S-shaped ptosis of the right upper eyelid. (b) and (c) Coronal (b) and Axial (c) CT scan shows a lacrimal gland mass with bony remoulding suggesting chronicity but no bony erosion.

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Figure 2
figure 2

(a) FNAC of the lesion shows many plasmacytoid myoepithelial cells and some pink stained myxoid stroma. No epithelial cells are present in this field (Giemsa stain). (b) Histology of the lesion shows a few ductules embedded in myxoid stroma on the right and a proliferation of plasmacytoid myoepithelial cells on the left (H and E stain).

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The patient had excision of the tumour in toto with the capsule intact by a lateral orbitotomy approach and made an uneventful recovery. Macroscopically, the tumour measured 40 mm × 40 mm × 30 mm. Histologically, the tumour was a cellular lesion with a few ductules lined by benign epithelial cells and many plasmacytoid myoepithelial cells, and myxoid stroma focally, consistent with a cellular pleomorphic adenoma (Figure 2b).

Discussion

Pleomorphic adenoma typically presents in middle age and is rare in childhood. It usually manifests as a slowly progressive, painless superotemporal mass without bony erosion.

There are only eight cases reported of pleomorphic adenoma in childhood. Dacryoadenitis accounts for most cases of an enlarged lacrimal gland in children. The clinical appearance of recurrent eyelid erythema in our case suggested an inflammatory process also. In two similar case reports of pleomorphic adenoma in childhood, signs of intermittent eyelid swelling were present for extended periods, as long as 4 years, and were attributed to presumed allergy.

A fine-needle aspiration cytology (FNAC) established the diagnosis in this case. This was a rapid, accurate, and valuable tool for tissue diagnosis.

An open incisional biopsy is not recommended for a suspected pleomorphic adenoma as removal with an intact capsule has prognostic implications. The likelihood of recurrence within 5 years is 3% if the lesion is removed with an intact capsule. This compares to a 30% chance of recurrence within 5 years if the lesion is incompletely removed or the capsule is not intact.3

Although pleomorphic adenoma of the lacrimal gland is rare in childhood, it must be considered in the differential diagnosis of an expansile lacrimal fossa mass in this age group.