BCAC typically arise in adults older than 60 years and have no gender predominance [4]. Patients present with a slow-growing mass that unlike this case, usually presents without ulceration [1]. Local pain is present in about 25% of patients. The 2005 World Health Organization classification categorizes BCAC as a low-grade tumour with a favourable prognosis [5]. These tumours were first recognized in 1978 and account for approximately 1.6% of all salivary gland neoplasms [3]. The vast majority occur in the parotid gland (about 90%) [6–8], followed by the submandibular gland and minor salivary glands [1].
BCAC is believed to arise from pluripotent ductal reserve cells. Grossly, these tumours appear solid grey-tan and grow as large as 7 cm in diameter [9]. BCAC has four major histologic growth patterns: solid, tubulotrabecular, cribriform and membranous. The solid pattern is the most common and the most likely to present with perineural invasion, though the prognostic significance of perineural invasion in BCAC is unknown. The membranous type has an appearance resembling dermal eccrine cylindromas. A syndrome of numerous basal cell adenomas, dermal cell cylindromas, and trichoepitheliomas has been described called Brooke-Spiegler Syndrome [4, 9]. Most cases of BCAC are believed to develop de novo but as many as 25% of cases may arise from a pre-existing basal cell adenoma [10, 11].
The major pathologic differential diagnostic considerations for BCAC are basal cell adenoma and adenoid cystic carcinoma. As its malignant counterpart, BCAC shares many histologic characteristics with basal cell adenoma but is distinguished from basal cell adenomas often only by invasion of local structures or by perineural or angiolymphatic invasion [12]. Distinguishing BCAC from adenoid cystic carcinoma is important due to the poorer prognosis and higher prevalence of the latter disease. Major distinguishing features of adenoid cystic carcinoma are the presence of dark hyperchromatic angulated nuclei which are in contrast with the vesicular nuclei of BCAC. Additionally BCAC often have prominent peripheral palisading of the outer layer that adenoid cystic carcinomas lack [9]. Immunostains are of uncertain value in this differential.
BCAC of a minor salivary gland is an extremely rare tumour with 22 reported cases in the literature. In recent series' of minor salivary gland tumours, BCAC account for <1% of all minor salivary gland tumours [12–16]. Overall, minor salivary gland tumours typically are located on the hard palate [16]; however, in the few available cases of minor salivary gland BCAC, they arise commonly from the buccal mucosa (9) in addition to the palate (7), followed by lip and tongue [17]. Patients present with a slow-growing mass in an area of minor salivary gland distribution. The prognosis of BCAC of a minor salivary gland appears similar to that of BCAC of other salivary glands. The treatment of choice is wide local excision. Since these tumours seldom metastasise to cervical lymph nodes, routine neck dissection is not recommended. The mortality rate for this tumour is also low, although reported local recurrence rates are high. Parashar et al. [17] noted that of 17 patients with BCAC of minor salivary glands with follow-up between 2 and 14 years, 2 patients died of the disease, both within 4 years. As with minor salivary gland malignancies in general, postoperative radiation is recommended for close surgical margins or following surgical excision of recurrent disease.