Management and Long-term Outcome of Adenoid Cystic Carcinoma with Intracranial Extension: A Neurosurgical Perspective

Abstract

Adenoid cystic carcinoma is a tumor of minor and major salivary glands that often invades the cranial base and intracranial cavity via local and perineural spread. In the past, the role of neurosurgeons in managing these tumors has been limited. The growth of interdisciplinary cranial base surgical approaches has now increased the involvement of neurosurgeons in the management of these tumors. We present a series of 16 patients with adenoid cystic carcinomas with cranial base and intracranial extension, the largest series reported in the neurosurgical literature. We have focused our approach on a neurosurgical perspective and made recommendations for the treatment of these tumors in relation to the following specific aspects of this disease. 1) The management of the carotid artery: In our experience, when the carotid artery is involved by tumor, a preliminary cerebral revascularization procedure with a cervical carotid to middle cerebral artery vein bypass graft should be performed before tumor resection. 2) The management of the cavernous sinus and orbit: Cavernous sinus tumor should be removed as fully as possible, but every effort should be made to preserve the IIIrd and IVth cranial nerves to achieve optimal functional and cosmetic results. The orbit should be exenterated when there is intraconal involvement; otherwise, intraorbital tumor can be removed with orbital preservation. 3) The use of palliative surgery: We have found that the use of palliative surgery can be considered even in patients whose extent of local disease precludes a surgical cure. The slow progression of the disease allows for long-term survival of many patients with advanced local disease and even of those with metastatic disease. 4) The long-term survival of these patients: In our series, six patients had no evidence of local disease and a mean survival of 72 months, one living patient had evidence of local disease and has survived 56 months, eight patients died of disease, with a mean survival of 137 months, and one patient died of complications after surviving for 63 months.