MANAGEMENT AND LONG-TERM OUTCOME OF ADENOID CYSTIC CARCINOMA WITH INTRACRANIAL EXTENSION - A NEUROSURGICAL PERSPECTIVE

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 mana ging these tumors has been limited. The growth of interdisciplinary cr anial base surgical approaches has now increased the involvement of ne urosurgeons in the management of these tumors. We present a series of 16 patients with adenoid cystic carcinomas with cranial base and intra cranial extension, the largest series reported in the neurosurgical li terature. 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 i nvolved by tumor, a preliminary cerebral revascularization procedure w ith a cervical carotid to middle cerebral artery vein bypass graft sho uld be performed before tumor resection. 2) The management of the cave rnous sinus and orbit: Cavernous sinus tumor should be removed as full y as possible, but every effort should be made to preserve the IIIrd a nd IVth cranial nerves to achieve optimal functional and cosmetic resu lts. The orbit should be exenterated when there is intraconal involvem ent; otherwise, intraorbital tumor can be removed with orbital preserv ation. 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 the long-term survival of many patients with advanc ed local disease and even of those with metastatic disease. 4) The lon g-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, eig ht patients died of disease, with a mean survival of 137 months, and o ne patient died of complications after surviving for 63 months.