Perineural spread of cutaneous squamous cell carcinoma via the orbit: Clinical features and outcome in 21 cases

Objective: To describe the clinical features, treatment, and outcome of a series of patients with perineural spread (PNS) of cutaneous squamous cell carcinoma (SCC) via the orbit. Design: A cohort study. Participants: Twenty- one patients identified with PNS of cutaneous SCC via the orbit. Intervention: Patients were treated with various combinations of conservative or radical surgery, external beam radiation therapy, and chemotherapy. Results: Of 21 cases, 17 (81%) were male. Age at presentation with PNS ranged from 38 to 82 years (median, 66 years). The forehead and eyebrow were the most common site of the primary lesion. All but one had altered or decreased sensation, but only nine presented with pain. Fourteen (67%) had ophthalmoplegia at presentation, and 14 (67%) had evidence of PNS involving branches of the facial nerve. Despite combinations of radical surgery, conservative surgery, and radiation therapy, no method of treatment appeared more effective, and 14 patients died from 9 months to 5 years after presentation with PNS (median, 3 years), usually from local and intracranial disease. Two survived to 14 and 18 years, one is alive at 3 years with recurrent local and distant disease, and four are alive without evidence of disease at 2, 3, 4, and 12 months after radiation therapy. Conclusions: Perineural spread of cutaneous SCC via the orbit carries a poor prognosis. Early radiation therapy may offer the best form of palliation. The role of radical surgery probably is limited once orbital involvement is apparent, as the cavernous sinus and facial nerve branches usually are involved.