Abstract

Retrospective analyses were done on 19 lacrimal adenoid cystic carcinoma (ACC) patients who underwent curative treatment between 1997 and 2013. Nine patients (47.4%) had T1-2 disease and ten (52.6%) had T4 disease. Surgical procedures were globe-preserving tumor resection in 11 patients (57.9%), incisional biopsy in five (26.3%), and orbital exenteration was undertaken in three (15.8%). Residual tumor burdens were R0/1 in 12 patients (63.2%) and R2 in seven (36.8%). Radiation therapy (RT) was recommended to all patients, and 16 (84.2%) completed RT (median 60 Gy). After median follow-up of 57.5 months, seven (36.8%) developed progression and three (15.8%) died. Local recurrence occurred in four patients (21.1%), distant metastasis in one (5.3%), and combined local recurrence and distant metastasis in two (10.5%). Progression-free survival and overall survival rates at 5-years were 64.5% and 82.6%, respectively. Among 12 patients following R0/1 resection, two (16.7%) developed local recurrence and none died, while among seven following R2 resection, five (71.4%) developed progression and three (42.9%) died. RT following R0/R1 resection could reduce progression. Globe-preserving surgery and RT seemed optimal strategy for T1-2 disease. Careful attention should to be paid to minimize residual tumor burden at surgery and effort for safe radiation dose escalation would be desired.

Highlights

  • Adenoid cystic carcinoma (ACC) is a rare, slowgrowing malignancy of the secretory glands with a prolonged clinical course [1,2,3,4,5]

  • We retrospectively reviewed the treatment outcomes of lacrimal ACC patients accrued in a single institute to evaluate the prognostic influences of histological subtype, surgical extent, residual tumor burden, and role of radiation therapy (RT)

  • Based on the current observations, two important summaries could be made: first, globe-preserving surgery followed by RT seemed an optimal treatment strategy for those having T1-2 disease; second, the addition of RT following R1 resection could decrease the risk of progression, but not following R2 resection

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Summary

Introduction

Adenoid cystic carcinoma (ACC) is a rare, slowgrowing malignancy of the secretory glands with a prolonged clinical course [1,2,3,4,5]. According to the Surveillance, Epidemiology, and End Results (SEER) database, ACC rising in the eye and orbit accounted for only 1.8% of total patients, among who the lacrimal gland was most commonly involved in over 80%, and was associated with poor survival [5]. Manual has defined T stage based on tumor size and extension to surrounding tissues [6, 7] (Table 1). Surgical resection has been the most important and orbital exenteration has been commonly recommended to the patients with T3-4 disease [5]. Based on the invasiveness of primary tumor and the surgical extent, post-operative radiation therapy (RT)

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