Facial pain and multiple cranial palsies in a patient with skin cancer

In January 2005, a 78-year-old male was referred from general practice to the Danish Headache Center for investigation and treatment of right-sided facial pain. He had a recurrent cancer in his right buccal region, and received curettage and electrodesiccation in August 2001, in January 2002, and in October 2002. Each time skin biopsies were obtained. The first and second biopsy showed basal cell carcinoma (BCC), and the third suggested SCC. He was subsequently examined twice (March 2003 and September 2004) by a dermatologist and declared clinically cured. In May 2003, he developed a white spot, numbness, and burning pain in the right buccal region. In September 2004, he complained of increased pain and experienced pain after non-painful stimuli (allodynia) in the region supplied by the maxillary branch of the trigeminal nerve. In October 2004, he developed incomplete right-sided facial nerve palsy (decreased nasolabial fold). In February 2005, MRI scans with focus on the fifth and seventh cranial nerves and cerebrospinal fluid (CSF) examinations were unremarkable. The patient complained of constant burning and intermittent stabbing facial pain with average intensity 7 (0–10 pain intensity numeric rating scale). He was treated with gabapentin (3,600 mg daily), codeine (125 mg daily), amitriptyline (50 mg daily) and ketobemidone (125 mg daily as need), but shortly after stopped with all medications because of adverse events. Two months later, he still complained of severe facial pain and developed diplopia. The patient was placed on pregabalin (450 mg daily) and reported a decrease in pain intensity by 40–50%. In May 2005, a neurological examination revealed unilateral (right sided) third and sixth cranial nerve palsies, complete loss of sensory function of the right trigeminal nerve (V1, 2, 3), temporal muscle atrophy, and ninth cranial nerve palsy. The perineural spread of his cutaneous cancer was suspected. A MRI scan showed no signs of perineural spread, and CSF and whole body positron emission tomography examinations were normal. A biopsy of the lingual nerve was unremarkable and a biopsy of the right supraorbital nerve showed axonal degeneration and subtotal loss of the myelinated nerve fiber, but no signs of perineural spread. A biopsy of the infraorbital nerve revealed infiltration of both blood vessels and nerve by SCC (Fig. 1). In July 2005, MRI scans showed pathological perineural enhancement of the right maxillary nerve and around the cavernous sinus and the Meckel cave (Fig. 2). A re-evaluation of the skin biopsies from 2001 to 2002 revealed SCC. The patient underwent radiation therapy without adjuvant chemotherapy over a period of two months. On a follow-up examination, 2 months after start of radiation, the neurological status and mild facial pain were unchanged. Three months later, the patient died of pneumonia.