Adenoid Cystic Carcinoma of the Sinonasal Tract: Treatment Results

Abstract

Objectives: Malignancies arising from the sinonasal tract, which includes the nose, paranasal sinuses, and nasopharynx, are uncommon. Although adenoid cystic carcinoma (ACC) is the second most common cancer occurring in the sinonasal tract, only few studies have been reported. This retrospective review was performed to identify the clinical features and treatment outcomes of sinonasal ACC. Methods: Thirty-five patients diagnosed and treated for ACC of the sinonasal tract were included in this study. Medical records, radiographs, and pathologic slides were retrospectively reviewed. Results: In two thirds of the patients, the maxillary sinus was the site of origin and cribriform was the most common histologic subtype (61%). Seventy-one percent of the patients had advanced disease (T3, T4) at the time of diagnosis. Five-year overall survival rate was 86% and treatment failure occurred in 18 patients (51%). Five-year local recurrence rate and distant metastasis rate were 30% and 25%, respectively. Adjunctive radiotherapy appeared to reduce local recurrence. Presence of distant metastasis correlated with decreased 5-year survival (P ‫؍‬ .001). Five-year survival rate after development of distant metastasis or local recurrence were 17% and 58%, respectively. Conclusions: Based on our findings, we suggest that sinonasal ACC be treated by a combined modality of radical surgery followed by postoperative radiation. The prognosis of sinonasal ACC seems to be determined by the presence of distant metastasis.

Sinonasal Tract and Nasopharyngeal Adenoid Cystic Carcinoma: A Clinicopathologic and Immunophenotypic Study of 86 Cases

“…These findings are similar to major salivary gland survival which is 76.9 and 61.6 % for 5-and 10 year overall survival rate, and 44.2 and 23.0 % for 5-and 10-year disease free survival rates, respectively [7,10,11,56,59,142]. Patients may, therefore, live with local disease and/or metastatic disease for extended periods of time, indicating the progressive and indolent nature of STACC, suggesting the 10-year survival rate may be more important and further underscoring the requirement for long-term clinical followup and management.…”

“…Even when surgical margins may be negative, skip lesions along peripheral nerve trunks invalidate a negative margin. In this study, as in many others [7,52,84,86,151], determination of margins could not be reliably performed, but perhaps apropos if the data is dearth. Perhaps, therefore, prudence would suggest discontinuing or greatly reducing the submission of multiple frozen section margins which unnecessarily prolong surgery, introduce frozen section artifacts, and provide a false sense of security to the clinician and patient about the success of extirpation.…”

“…Most of the tumors involved multiple locations (52 %), although the nasal cavity alone (29 %) was a common finding. Tumor involvement of multiple sites correlated with a worse patient outcome (p \ 0.0001) [7,86]. This is also probably correlated to the majority of patients showing higher stage (III and IV) disease at presentation, involving sensitive structures of the area (optic nerve, brain stem, chiasm).…”

“…It is possible that tumors managed by surgery alone were more localized and thus prone to a better prognosis without any additional therapy. Since the disease is progressive and indolent, radiation is probably at best palliative, delaying rather than preventing recurrences and providing symptomatic relief while not significantly altering long term outcome [7,106]. Modifications of radiation regimens (carbon ion boost and photo intensity-modulated radiation therapy) may yield a better response, but requires more careful evaluation [24,86,92].…”

“…Squamous cell carcinoma is the most common sinonasal tract cancer, but adenoid cystic carcinoma (ACC) is the second most common malignancy and the most common salivary glandtype tumor of the sinonasal tract (nasal cavity, paranasal sinuses, and nasopharynx, hereinafter referred to collectively as the sinonasal tract, i.e. STACC) [7]. ACCs comprise between 10 and 15 % of all malignant salivary gland tumors.…”