Is exenteration must in each case of adenoid cystic carcinoma of lacrimal gland?

Abstract

Purpose: Adenoid cystic carcinoma is the most common malignant epithelial tumor of the lacrimal gland. As adenoid cystic carcinomas are relatively rare tumor and the prognosis is poor, comparison of different treatment approaches is difficult. Controversy still remains regarding the appropriate treatment modality for adenoid cystic carcinoma of the lacrimal gland. The aim of this study was to report the clinicopathological profile and management options for adenoid-cystic-carcinomas of lacrimal gland at a tertiary care referral centre.

Methods: This is a retrospective review of all histopathologically proven adenoid cystic carcinoma of the lacrimal gland diagnosed between years 2009-2013. Total 20 cases were retrieved from clinical and pathological archive.

Results: 20 cases of adenoid-cystic-carcinomas were studied. The age ranged from 17-75 years. Nine out of 20 were males. Duration of symptoms ranged from 2month-3years. Expansion of the lacrimal fossa was evident in 16/20 (80 %) of the cases, bony erosion or invasion of bone was seen in 45% cases (9/20). Calcification was detectable at soft-tissue CT window settings in 4 (20%) patients. Histologically Cribriform pattern was most common, perineural invasion was seen in 2 case & 2 cases were high grade. Eleven cases with tumor confined to mid-orbit which appeared benign on radiology were managed with local en-bloc excision f/b chemotherapy and radiotherapy. Two cases with orbital apex involvement were managed with chemoreduction f/b debulking & radiotherapy. One case with extensive orbital involvement even after 6-cycle of chemotherapy underwent exenteration. Primary exenteration followed by radiotherapy was done in 5/20 cases all of which showed ACC on incision biopsy. Two cases where complete local resection was done showed early recurrence [<3 months] which were exenterated. No recurrence has been noted in 18 cases till follow-up of 3month-2years. Two patients died of intracranial recurrence.

Conclusions: A combination of surgery with chemotherapy and radiotherapy can be considered as an alternative treatment modality for well demarcated and localised adenoid-cystc-carcinomas of lacrimal gland with encouraging short term outcomes avoiding exenteration.

Keywords: 631 orbit • 624 oncology