Lacrimal Gland Tumors
While 9–15% of orbital tumors arise in lacrimal gland, inflammatory or infiltrative diseases – such as sarcoidosis, Wegener’s granulomatosis, IgG4 disease, or other (nonspecific) dacryoadenitis – comprise two-thirds of lacrimal gland masses and can presen
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David H. Verity and Geoffrey E. Rose
Introduction While 9–15% of orbital tumors arise in lacrimal gland, inflammatory or infiltrative diseases – such as sarcoidosis, Wegener’s granulomatosis, IgG4 disease, or other (nonspecific) dacryoadenitis – comprise two-thirds of lacrimal gland masses and can present with signs and symptoms similar to tumors [1, 2]. Often a firm diagnosis can be reached only with tissue biopsy. Inflammatory lesions typically present with acute or subacute symptoms that can include a painful, tender swelling in the lacrimal gland area, an “S-shaped” deformity of the upper eyelid, or conjunctival redness and injection (Fig. 13.1). Lymphomas tend to produce chronic, painless globe displacement, although some present with inflammatory features, which portend a worse prognosis [3]. Benign or malignant tumors can present similarly, and both enter the differential diagnosis for many lacrimal masses. About a half of primary tumors of the lacrimal gland are benign, most being
D. H. Verity Adnexal Service, Moorfields Eye Hospital, London, UK G. E. Rose (*) Adnexal Service, Moorfields Eye Hospital, London, UK NIHR Biomedical Centre, Institute of Ophthalmology, London, UK e-mail: [email protected] © Springer Nature Switzerland AG 2019 C. J. Hwang et al. (eds.), Clinical Ophthalmic Oncology, https://doi.org/10.1007/978-3-030-13558-4_13
of epithelial in origin; however, other very rare benign tumors – such as hemangiopericytoma, neurilemmoma, neurofibroma, lymphangioma, and other vascular malformations – can be centered on the gland. In addition to a thorough history and examination, high-resolution imaging plays a key role in establishing an appropriate treatment plan.
Epidemiology Almost all benign lacrimal gland tumors are pleomorphic adenomas (Table 13.1) [4], and adenoid cystic carcinoma is the commonest malignant epithelial tumor [5]. Carcinoma arising in prior pleomorphic adenoma (“malignant mixed tumor”) represents the second most common lacrimal gland malignancy [1, 5, 6], whereas mucoepidermoid carcinomas, primary adenocarcinomas, and squamous carcinomas are rare. Lymphoma, associated with systemic disease in a minority, accounts for about 10–14% of all lacrimal gland masses [1, 2], while metastases to the lacrimal gland are very uncommon; the latter tend to mimic the primary lesion, most such masses being fast growing and associated with a poor prognosis.
Clinical Features Pleomorphic adenomas present from childhood [7] to old age, with a peak incidence in middle age and without a significant gender bias [4]. 165
D. H. Verity and G. E. Rose
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a
b
Fig. 13.1 S-shaped deformity of the right upper lid caused by subacute dacryoadenitis (a). CT showing right lacrimal gland enlargement with molding around the globe (b)
Table 13.1 Common primary lacrimal gland tumors Types Benign tumors Malignant tumors
Nomenclature Pleomorphic adenoma Myoepitheliomaa Adenoid cystic carcinoma Malignant mixed tumor (usually adenocarcinoma arising in pleomorphic adenoma) Mucoepidermoid carcinom
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