A denoid cystic carcinoma (ACC) is a locally aggressive salivary gland malignancy prone to perineural invasion and metastasis. Local recurrences often involve the skull base, whereas distant metastases most frequently involve the lung, bones, and liver. 3,4,27,43,45 Standard therapy for this malignancy has most commonly included a strategy of local resection followed by adjuvant radiation therapy. 6,13,16,17,23,24,28,30,35,38,39,43,46,47,52 Chemotherapy using agents such as carboplatinum and paclitaxel has been used, but with limited results. 2,11,12,15,26,27,36,41,44,[48][49][50] ACC can arise in any salivary gland, both major and minor. Consequently, these tumors can be located in the tongue, trachea, palate, larynx, and paranasal sinuses.
27Tumors in the sinonasal cavity can remain undetected for long periods of time with minimal symptoms. Only after sufficient soft-tissue involvement, bone destruction, or cranial nerve involvement does the malignancy become manifest. Therefore, a strategy of maximal resection with minimal morbidity often presents a substantial therapeutic challenge given the complex anatomy of lesions that involve the paranasal sinuses and extend intracranially into the anterior or middle fossa.abbreviatioNs ACC = adenoid cystic carcinoma; EGFR = epidermal growth factor receptor; ICA = internal carotid artery; OS = overall survival; PFS = progression-free survival; VEGF = vascular endothelial growth factor. submitted October 28, 2014. accepted January 5, 2015. iNclude wheN citiNg Published online August 7, 2015; DOI: 10.3171/2015.1.JNS142462. disclosure The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. results Median follow-up for study patients was 6.75 years. The 5- and 10-year overall survival (OS) rates were 78% and 50%, respectively. Sixty-six percent of patients had progression of their disease. The 5- and 10-year progressionfree survival (PFS) rates were 46.7% and 21.0%, respectively. Gross-total resection was achieved in 75% of patients, with 49% having microscopically negative margins at the time of first operation. On univariate analysis, resections with microscopically negative margins were associated with a significant OS advantage (20.1 ± 3.3 years) compared with resections that left residual disease, even if microscopic (10.3 ± 1.6 years, p = 0.035). In patients who underwent reoperation, the effect persisted, with improved OS in those with negative margins (21.4 ± 0.0 vs 16.7 ± 4.0 years, p = 0.06). The use of adjuvant radiotherapy was associated with an OS advantage (16.2 ± 2.5 vs 5.5 ± 2.2 years, p = 0.03) at initial diagnosis and improved PFS (7.8 ± 1.0 vs 2.1 ± 0.62 years, p = 0.005), whereas repeat irradiation provided no benefit. The use of adjuvant chemotherapy at diagnosis or at recurrence was not associated with any significant advantage. Multivariate analysis revealed margin-negative resection at initial operation and at recurrence retained OS significance, even after controlling...