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BMC Cancer  2007 

An unusual presentation of adenoid cystic carcinoma of the minor salivary glands with cranial nerve palsy: a case study

DOI: 10.1186/1471-2407-7-157

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Abstract:

A 49-year-old African American female presented to the emergency room complaining of severe right-sided headache, photophobia, dizziness and nausea, with diplopia. The patient had a 14 year history migraine headaches, hypertension, and mild intermittent asthma. Physical examination revealed right lateral rectus muscle palsy with esotropia. There was numbness in all three divisions of the right trigeminal nerve. Motor and sensory examination of extremities was normal. An MRI of the brain/brain stem was obtained which showed a large mass in the clivus extending to involve the nasopharynx, pterygoid plate, sphenoid and right cavernous sinuses.Biopsy showed an ACC tumor with a cribriform pattern of the minor salivary glands. The patient underwent total gross surgical resection and radiation therapy.This is a case of ACC of the minor salivary glands with intracranial invasion. The patient had long history of headaches which changed in character during the past year, and symptoms of acute 5th and 6th cranial nerve involvement. Our unique case demonstrates direct invasion of cavernous sinus and could explain the 5th and 6th cranial nerve involvement as histopathology revealed no perineural invasion.Adenoid Cystic Carcinoma (ACC) is a rare tumor entity and comprises about 1% of all malignant tumor of the oral and maxillofacial region [1]. It is a slowly growing but highly invasive cancer with high recurrence rate. Lymphatic spread to local lymph nodes is rare. Hematogenous spread, however, occurs often in the course of the disease [2]. Intracranial ACC even is more rare and has been reported as 4 – 22% of ACC [3]. It could be primary or secondary which could occur either by direct invasion like in our case, hematogenous spread, or perineural spread [4,5]. Perineural spread of ACC has long been recognized. The literature revealed that the region of Gasserian ganglion to be the most common site of involvement (35.8%) [2,3,6,7], while cavernous sinus was involved in 15.1% [3-5

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