This is a major review of the management of lacrimal gland carcinoma, focusing mainly on adenoid cystic carcinoma. Relevant literature published in English since 1970 was included amounting to some 40 articles after filtering. Overall mortality for all lacrimal gland carcinomas was estimated at 50% at five years. Adenoid cystic carcinoma was the most common lacrimal gland epithelial tumour, accounting for 62% of all carcinomas. The authors describe several unique features including a younger age at diagnosis, at least 10 years earlier than other carcinomas, and pain occurring more frequently and earlier in the disease. Survival rates between different carcinomas were hard to compare due to lack of standardisation in tumour staging, but adenoid cystic carcinoma characteristically had high local and distant recurrence rates, often after many years of dormancy. One series had 45% survival at five years, 23% at 10, and 14% at 15 years. Younger age carries a better prognosis, whereas solid, basaloid tumours fare worse than other histological sub-types. Up to 85% will have perineural invasion which is associated with a higher risk of local recurrence and skull base involvement. Bone invasion was found in 81% in a series from the Mayo clinic. Tumour size is a strong indicator of local recurrence and metastasis, with T3 or larger tumours having significantly worse outcomes. Treatment options remain controversial, in particular whether exenteration improves survival compared with local excision; although the authors suggest that local, globe sparing surgery should be explored further for small tumours. Adjuvant radiotherapy has a role as the tumours are more radio-sensitive than other lacrimal gland carcinomas. Chemotherapy seems less effective, both as adjunctive treatment and as neoadjuvant therapy. This is an interesting and comprehensive review which also benefits from the inclusion of a useful management algorithm for all lacrimal gland masses.
Lacrimal gland carcinoma
Reviewed by James Hsuan
Management of lacrimal gland carcinoma: Lessons from the literature in the past 40 years.
