Pleomorphic adenoma is the most common epithelial tumour of the lacrimal gland, representing 12% of lacrimal fossa lesions.1 It typically presents in middle age and is rare in children under the age of 16 years, with only a few previously reported cases.2–6 We present an additional case in a 10 year old girl with photographic documentation of longstanding orbital asymmetry.
Case report
A 10 year old girl was referred with a 3 month history of painless right eyelid swelling, presumed to be allergic in nature. The unaided right visual acuity was 6/24, improving to 6/9 with correction of astigmatism (+1.00/−2.25 × 020) and the unaided left visual acuity was 6/5. Pupil responses and colour perception were normal. Ocular motility was normal but there was 3 mm of right proptosis, with inferonasal globe displacement, and a non-tender lacrimal gland mass. Periorbital sensation was intact and ocular examination was otherwise unremarkable. Family photographs demonstrated an asymmetry in appearance of the two eyes for at least 2 years previously (Fig 1).
Figure 1.
Photographs taken at (A) 6 years, (B) 8 years, (C) 9 years, and (D) 11 years, showing inferonasal displacement of the right globe, right ptosis, and superolateral orbital swelling present for at least 2 years before presentation.
Computed tomograph (CT) scan demonstrated a well defined 1.8 × 1.8 cm mass arising from the right lacrimal gland with mild smooth bony erosion of the lacrimal fossa, consistent with a pleomorphic adenoma. Lateral orbitotomy was performed under general anaesthetic with intact excision of the orbital lobe of the right lacrimal gland. Histology confirmed benign pleomorphic adenoma with intact pseudocapsule.
Comment
Pleomorphic adenoma of the lacrimal gland is rare in children with only few previously reported cases. Nevertheless, even in the paediatric age group, it is important to consider the diagnosis in the presence of a painless, gradually progressive lacrimal gland mass. A published system for evaluation of lacrimal gland masses scores the duration of symptoms, presence of persistent pain or sensory loss, as well as radiological features, in order to determine the appropriate surgical management.7 The case described scored the maximum 8 points, where a score higher than 3 suggests pleomorphic adenoma requiring excision without previous incisional biopsy. Complete excision with an intact pseudocapsule has prognostic importance in avoiding later pervasive recurrence and malignant transformation.
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