Cranial neuropathy as a presenting sign of recurrent aggressive skin cancer

The purpose of this study was to identify and characterize recurrent skin cancers of the head and neck presenting with cranial neuropathies and to review the presentation and the management for this rare subset of cutaneous neoplasms.

A retrospective review was performed for all patients with previous related cutaneous neoplasms presenting with cranial neuropathies referred to a single academic tertiary-care head and neck tumor program from 1999 to 2007. Six cases of head and neck carcinoma with demonstrable cranial neuropathy were identified and analyzed by clinical history, radiographic and surgical findings, and treatment and survival data. A review of the literature, pertinent anatomy, imaging studies, and surgical/nonsurgical management are summarized for these aggressive neurotropic malignancies.

Cranial neuropathy was the presenting symptom of recurrent disease in all six patients. Four presented with multiple cranial neuropathies. All exhibited neuropathy of the trigeminal nerve (cranial nerve V). The tumors involved were squamous cell carcinoma (4) and melanoma (2). All patients were multiply symptomatic, presenting with a mean of three neurologic symptoms, including facial numbness (5), facial paralysis or weakness (3), facial pain (3), diplopia (3), paresthesia (3), hearing loss (1), or formication (2). Symptoms were present for an average of 7 months prior to diagnosis of perineural recurrence. Cranial nerve involvement was confirmed in all patients by magnetic resonance imaging, and five patients manifested histologic evidence of perineural tumor infiltration. Treatment consisted of various combinations of surgery, radiation, and chemotherapy for five patients, and one patient declined any intervention. Death rate subsequent to disease was 50%, and follow-up has continued within our institution on all patients for an average of 25.5 months (range, 3-72 months).

Cranial neuropathy is a rare presentation of recurrent cutaneous neoplasms of the head and neck. Given this infrequent occurrence and shared features of presentation, these highly morbid tumors are often mistakenly diagnosed as Bell's palsy or trigeminal neuralgia. Our findings corroborate previous reports of diagnostic delay, increased tumor burden, and worsened morbidity and mortality associated with such cutaneous malignancies. The critical utility of radiologic imaging for staging and tumor delineation are also supported by our institutional data.