The role of skull base surgery for the treatment of adenoid cystic carcinoma of the sinonasal tract

Abstract

Background Adenoid cystic carcinoma (ACC) of the sinonasal tract is an aggressive malignancy associated with a poor 5‐year survival rate. The role of skull base surgery for the treatment of patients presenting with sinonasal ACC and its impact upon their survival has not previously been evaluated. Methods A retrospective review of 35 patients with ACC of the sinonasal tract who were treated with surgery and radiation therapy at the University of Pittsburgh Medical Center was performed to evaluate patient outcome. Results Local recurrence of tumor following surgery and radiation therapy was observed in 36% of the patients originally treated at the University of Pittsburgh Medical Center. Fourteen percent of these patients developed a regional tumor recurrence, and 21% developed distant metastases. We did not identify any tumor‐related factors that predicted patient outcome. Local recurrences were treated with salvage surgical excision, and, despite aggressive management, only 1 of 17 patients with local recurrence was considered cured (NED) at 24 months (follow‐up after salvage surgery). Overall, disease‐free survival was 46.4%, at a median follow‐up of 40 months. Conclusions ACC of the sinonasal tract is an aggressive malignancy. Skull base surgery has facilitated the gross total excision of advanced lesions that were deemed inoperable in the past, but has not resulted in an overall improvement in disease‐free survival. Local recurrence portends a very poor prognosis, despite aggressive salvage regimens. Alternative therapies for local recurrences warrant further investigation. Prospective, randomized studies are necessary to evaluate the outcome of patients treated with aggressive multimodal treatment regimens, including chemotherapeutic regimens. © 1999 John Wiley & Sons, Inc. Head Neck 21: 402–407, 1999.

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

“…When the skull base was affected, overall survival is much shorter and more patients died with disease (p = 0.005): 26 patient had skull base involvement, surviving an average of 6.5 years and 80.8 % dead of disease versus patients without skull base involvement having an average survival of 12.1 years and 41.7 % dead of disease. These findings are similar to other reviews [6,52,84].…”

“…However, tumors in the sinonasal tract are fragmented and often in multiple parts, making a calculation of tumor ''volume'' more difficult. Further, while higher tumor grade was associated with an overall shorter survival (Grade 1: 12.5 years vs. Grade 3: 7.5 years), this difference did not reach statistical significance (p = 0.089), a finding similar to other authors [84]. Interestingly, many of the parameters (necrosis, bone invasion, perineural invasion, dominant pattern) when separately evaluated were significant (see below) while others are not significant (mitoses, atypical mitoses, pleomorphism) or not valid (circumscription) in this anatomic site.…”

“…A highly characteristic feature of salivary gland ACC is the tendency to show perineural invasion [6,142]; however, nerve symptoms were uncommon in STACC (12.8 %). Perineural invasion was not a constant feature in STACC, with perineural or intraneural involvement seen in 54.7 % of cases, within the range of other reported series (40-91 %) [6,84]. Neural invasion did not correlate to stage, bone invasion or lymphovascular invasion.…”

“…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.…”

“…Because the tumor is slow growing and often has skip lesions, skull base disease is often a late finding. As the skull base has a limited blood supply, chemotherapy is not effective in this setting, although radiation may be palliative [6,52,84,86,152]. When the skull base was affected, overall survival is much shorter and more patients died with disease (p = 0.005): 26 patient had skull base involvement, surviving an average of 6.5 years and 80.8 % dead of disease versus patients without skull base involvement having an average survival of 12.1 years and 41.7 % dead of disease.…”