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Background We examined the assumption in conventional teaching about metastatic adenoid cystic carcinoma (ACC) being an indolent type of disease. Methods A single center analysis of 105 cases of ACC was performed. Radiographs were reviewed and tumor response to chemotherapy was measured. Distant disease-free survival (DDFS) and time to death since distant metastases diagnosis were analyzed. Results Forty-two percent of the patients were diagnosed with distant metastases. DDFS showed significant negative associations with advanced T classification, N+ classification, solid type tumor, and positive surgical margins. Distant metastases (91%) developed in the first 5 years after presentation. Median distant metastatic survival was 13.8 months. The most frequent organ sited was the lung. Solid type ACC showed a preponderance for multiorgan metastases (17/28; 61%). Distant metastases seemed not to occur in case of clear surgical margins. Solid type ACC had a significant poorer survival after development of distant metastases. Conclusion Metastatic ACC is not always an indolent disease. © 2016 Wiley Periodicals, Inc. Head Neck 39: 456-463, 2017 [ABSTRACT FROM AUTHOR]

Summary: Background: Adenoid cystic carcinoma is a rare salivary gland malignancy with a poor disease free survival due to frequent distant metastases and late local recurrences. Previous single-center reports on outcome mostly encompass small series. In this report a relative large series of 105 cases is analyzed, all treated at the VU University Medical Center, Amsterdam, The Netherlands over a 30-year period in which treatment strategies remained unchanged. Methods: All cases of ACC of the head and neck between 1979 and 2009 at our institution were analyzed through a medical chart review. Recurrence patterns and possible prognostic factors (T-stage, N-status, age, gender, type of salivary gland involved, histological grade, surgical margins, perineural invasion (PNI) and postoperative radiotherapy (RT)) were analyzed. Results: One-hundred and five cases of ACC of the head and neck were identified. Five-, ten- and twenty-year survival rates for overall survival were 68%, 52% and 28%, respectively. T-stage, N-status, surgical margins, histological subtype and age were highly significant predictors for survival. PNI was not a negative prognosticator. Conclusions: T-stage, N-status, surgical margins, histological grade and age are the main predictors of survival-outcome in ACC of the head and neck. Distant metastasis frequently develop, mainly in the first 5years post treatment. Local recurrences often develop even later on, warranting long term follow up of patients treated for ACC. Grade III ACC should be considered a specific entity within the group of ACC due to its typical aggressive biological behavior and relatively poor outcome, implicating the need for an improved adjuvant treatment. [Copyright &y& Elsevier]

Background: To analyse outcome with regard to histological features in patients treated for adenoid cystic carcinoma (ACC) in the VU University Medical Center, Amsterdam, The Netherlands. Material–methods: All cases of ACC of the head and neck treated between 1979 and 2009 were analysed through a medical chart review and histopathological revision. Recurrence patterns and possible histological prognostic factors such as histological subtype according to Perzin and Szanto’s grading system (grade I: predominantly tubular, no solid component, grade II: predominantly cribriform, less than 30% solid and grade III: solid component >30%), surgical margins (positive, close and clear) and perineural invasion (PNI) were analysed. Uni- and multivariate statistical tests were performed with SPSS version 15.0® (IBM, New York, USA). Results: One-hundred and five cases of ACC of the head and neck were identified. The most affected major salivary gland was the parotid gland. With regard to the minor salivary glands, the oral cavity and oropharynx were the predominant sites for ACC. Five-, ten- and twenty-year survival rates for disease specific survival (DSS) were 73%, 53% and 45%, respectively. For overall survival (OS) values were 68%, 49% and 25%, respectively. 89% of cases showed positive or close surgical margins, where the group of clear surgical margins mainly consisted of T1–2 major salivary gland tumors. Surgical margins were a significant factor for DSS (p =0.004) as well as for OS (p <0.000). Hazard Ratios (HR) for OS in patients with positive and close surgical margins were 8.1 and 2.3, respectively. With regard to histological subtype, 26% of cases were grade I, 47% grade II and 27% grade III. DSS (p <0.000) and OS (p <0.000) were highly significant. On multivariate analysis, HR for OS was 2.7 for grade II and 3.8 for grade III respectively. PNI was seen in 70% of cases and was a negative prognosticator in terms of DSS and OS on univariate analysis (p =0.033 and 0.042 respectively) and showed a HR of 4.9 on multivariate analysis. Conclusions: Adequate surgical margins, although difficult to achieve, are an important prognosticator. A predominantly tubular histology has the best prognosis, followed by cribriform and solid subtype. The latter can best be considered a high grade malignancy. PNI is a negative prognosticator regardless of named nerve involvement. There is a need for improved adjuvant treatment after surgery in ACC of the head and neck. [Copyright &y& Elsevier]