Importance: Acinic cell carcinoma is a rare salivary neoplasm that is generally associated with a good prognosis, although a subset of patients develops local and distant recurrences. Given the rarity of the disease, factors to identify patients at risk for recurrences or decreased survival are not clearly defined.
Objectives: To identify clinicopathologic factors associated with adverse survival in patients with acinic cell carcinoma and to assess the effect of local, regional, and distant recurrences on survival.
Design, setting, and participants: Retrospective medical record review in a tertiary care cancer center of 155 patients treated for acinic cell carcinoma from January 1990 through February 2013.
Main outcomes and measures: Primary outcomes evaluated were overall and disease-free survival. The end points assessed were age at diagnosis, sex, size of primary tumor, presence of positive surgical margins, postoperative radiation therapy, and development of local, regional, or distant recurrences.
Results: The median survival was 28.5 years, with 13 patients (8.4%) dying of their disease. Women (n = 104) were affected twice as often as men (n = 51) but had an improved survival (P < .001). Patients diagnosed as having acinic cell carcinoma before or at the age of 45 years had an improved survival (P = .02) compared with their elder counterparts, a finding that was independent of sex. Neoplasms larger than 3 cm at presentation were associated with a decreased overall survival compared with smaller lesions (P = .02). The development of distant metastases was most associated with death from the disease (odds ratio, 49.90; 95% CI, 6.49-2246.30; P < .001) compared with local and regional recurrences.
Conclusions and relevance: Although patients with acinic cell carcinoma generally have a favorable prognosis, we have identified several factors associated with decreased survival, including male sex, age older than 45 years, neoplasms larger than 3 cm, and the development of a distant recurrence. These results suggest that maximizing local and regional control for this disease can offer substantial benefit when no distant disease is detectable.