Histological-pathological and clinical T stage of Primary Adenoid Cystic Carcinoma of the Lacrimal Gland in a Chinese population

Background To present clinical presentations, histological-pathological patterns, clinical T stage, divergent treating methods, and outcomes of primary adenoid cystic carcinoma (ACC) of the lacrimal gland in a Chinese population. Methods This case series study enrolled patients with primary ACC of the lacrimal gland admitted to authors’ Hospital in China between 2003 and 2014. Results A total of 38 patients included 16 men and 22 women with a median age of 46.3 years were enrolled. Sixteen patients (42.11%) had local recurrence, while nineteen patients (50%) had distant metastasis at the time of presentation. Twelve patients (31.58%) were in T1-3 stage and twenty-six (68.42%) were in T4 stage. The median disease-free survival for the entire group was 30.4 months. Nineteen patients (73.08%) in T4 stage had a predominantly solid histological pattern, and only three (25%) in T1 ~ T3 stage had a predominantly solid histological pattern. Twelve patients (46.15%) in T4 stage underwent an exenteration with removal of the bone of the superior and lateral orbit and radiation therapy. Fourteen patients (53.85%) underwent globe-preserving apparent gross tumor resection with radiation therapy. The median disease-free survival time for the entire group was 30.4 months. The 5-year survival rate was 52.63%. Conclusions The solid pattern might be associated with more aggressive biologic tumor behaviors and this stage of adenoid cystic carcinoma of the lacrimal gland may be considered as an indicator of poor prognosis.