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
Summary
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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