Abstract

Abstract Introduction Adenoid cystic carcinoma (ACC) is a rare cancer of the head and neck that primarily occurs in the salivary glands. Sino-nasal ACC (SNACC) is a much rarer entity; this paper presents an exceedingly rare case of SNACC with both intracranial and intradural extension. In addition to this, a review of the literature has been performed. Case Study Our case describes the diagnosis of SNACC with both intracranial and intradural extension in a 55yr old female which was subsequently managed with palliative radiotherapy. Review of literature and Conclusions Currently, there are only few larger studies that have been conducted on SNACC and only isolated case reports of SNACC with intracranial extension. Aggregated data suggests that SNACC tends to present late at T3-T4 stage, has a 5-year survival of 60% and holds the best prognosis when surgical resection is feasible. From our case and the four other cases published on SNACC with intracranial extension it can be seen there is a clear distinction with management of patients with or without intradural extension. Intradural extension is a contraindication to surgical resection and once found deems the disease stage as primarily palliative. The focus for treatment of SNACC particularly with intracranial extension should catch disease early and prevent intradural spread. This maximises the number of patients who can undergo surgical resection and improves prognosis.

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