Eric David Miller MD, PhD, Presenter: Nothing to Disclose

Dukagjin Blakaj MD, PhD, Abstract Co-Author: Nothing to Disclose

John Christopher Grecula MD, Abstract Co-Author: Research Grant, Teva Pharmaceutical Industries Ltd Research Grant, Soligenix, Inc Stockholder, Pfizer Inc Stockholder, F. Hoffman-La Roche Ltd Stockholder, WebMD Health Corp Stockholder, Merck & Co, Inc Stockholder, Amgen Inc Stockholder, AstraZeneca PLC Stockholder, Baxter International Inc Stockholder, Bristol-Myers Squibb Company Stockholder, EntreMed, Inc Stockholder, Express Scripts Holding Company Stockholder, General Electric Company Stockholder, Eli Lilly and Company Stockholder, Medtronics

Purpose/Objective(s): Sinonasal adenoid cystic carcinoma (SNACC) is a rare cancer that typically presents with nonspecific symptoms and at an advanced stage. The purpose of this study is to evaluate the treatment outcomes and complications of patients treated for advanced SNACC at our institution.Materials/Methods: The medical records of 14 patients with SNACC treated between 1994 and 2012 were reviewed. Thirteen patients (93%) had advanced disease (T3, T4) at diagnosis and 1 patient had metastatic disease. The maxillary sinus (50%) was the most common primary tumor site. The most common presenting symptoms were pain (46%) and eye symptoms (38%). All patients received primary surgery and 11 patients (79%) received postoperative radiation therapy (PORT) with 1 patient receiving concurrent chemotherapy. PORT doses ranged from 40 to 68 Gy (median 59.7 Gy). The median follow-up time for all patients and for living patients was 37.5 months and 37 months, respectively.Results: The 3 and 5-year actuarial survival outcomes were 70% and 40%, and the 3 and 5-year progression-free survival outcomes were 54% and 43% for all patients, respectively. Seven patients (50%) failed primary treatment with 3 local failures (21%), 2 distant failures (14%), and 2 locoregional and distant failures (14%). The median time to progression was 26 months (range, 3-61 months). In the patients who developed recurrent disease, the majority had T4 disease (85%) and positive surgical margins (85%). All of the patients who developed local recurrence received a PORT dose of less than 60 Gy. Three of the patients who developed a first recurrence had a second recurrence with a median of 25 months (range, 1-30 months) after the initial recurrence. Of the 7 patients who failed primary treatment, 1 patient (7%) was alive at last follow-up. Six patients (43%) have been disease-free since primary treatment and were alive at last follow-up. In this group, 4 of the patients had T4 disease (67%) and 3 of the patients (50%) had positive surgical margins. All of the patients who have been disease-free since primary treatment received a PORT dose of 60 Gy or more, although this did not reach statistical significance (p = 0.104). Three patients (21%) have long-term complications from treatment including a nasocranial fistula, osteoradionecrosis of the frontal bone requiring hyperbaric oxygen therapy, and nasolacrimal duct obstruction.Conclusions: Surgery and PORT is the current standard for treatment of SNACC. Patients who have negative surgical margins and receive a PORT dose of 60 Gy or more had a more favorable prognosis than patients who received a PORT dose of less than 60 Gy. Long-term complications were observed in 21% of patients following treatment. We are currently evaluating HPV status and prognostic significance in our SNACC patients as HPV has been shown to be associated with sinonasal tract tumors.

Miller, E, Blakaj, D, Grecula, J, Treatment Outcomes of Advanced Sinonasal Adenoid Cystic Carcinoma.  Radiological Society of North America 2014 Scientific Assembly and Annual Meeting, - ,Chicago IL. http://archive.rsna.org/2014/14045824.html