Sinonasal adenoid cystic carcinoma: systematic review of survival and treatment strategies.

Objective: This study reviews the published outcomes related to sinonasal adenoid cystic carcinoma (SNACC). Clinical presentation, radiographic diagnosis, pathology, treatment, and management outcomes of this uncommon disease are reported. Data Sources PubMed database.

Methods: A systematic review of studies for SNACC from 1960 to 2012 was conducted. A PubMed search for articles related to SNACC, along with bibliographies of those articles, was performed. Articles were examined for both individual patient data (IPD) and aggregate patient data (APD) that reported survivability. Demographics, disease site and spread, treatment strategies, follow-up, outcome, and survival were described for IPD, and a meta-analysis for survival rates was performed for APD.

Results: A total of 55 journal articles were included. Individual patient data were reported in 39 journal articles, comprising a total of 88 cases of SNACC. Sixteen articles, totaling 366 patients that reported aggregate 5-year survivorship pertaining to SNACC, were also included. Average follow-up in the IPD was 51.2 months (range, 1-198 months), and 5-year survivorship was 63.5%. In the studies reviewed, surgery followed by postoperative radiotherapy was the most common therapy used and resulted in the highest percentage of survivors. Aggregate patient data meta-analysis revealed a 5-year survival rate of 62.5%.

Conclusion: This study contains the largest pool of SNACC patients to date. The data suggest that SNACC has a poor overall prognosis. It also suggests that surgery with postoperative radiotherapy is the most commonly used and may possibly be the most effective therapy.