Stijn van Weert1, Rinze Reinhard2, Elisabeth Bloemena3, Jan Buter4, Birgit I Witte5, Marije R Vergeer6, C René Leemans1. 1. Department of Otolaryngology - Head and Neck Surgery, VU University Medical Center, Amsterdam, The Netherlands. 2. Department of Radiology, VU University Medical Center, Amsterdam, The Netherlands. 3. Department of Pathology, VU University Medical Center/Academic Center for Dentistry (ACTA), Amsterdam, The Netherlands. 4. Department of Medical Oncology, VU University Medical Center, Amsterdam, The Netherlands. 5. Department of Epidemiology and Biostatistics, VU University Medical Center, Amsterdam, The Netherlands. 6. Department of Radiation Oncology, VU University Medical Center, Amsterdam, The Netherlands.
Abstract
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.
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.
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