Intracranial extension of meibomian gland carcinoma with pagetoid changes

A 41-year-old man presented with exophthalmos and loss of visual acuity. Neuroradiological studies showed a large mass extending from the intraorbital region to the frontal lobe. In addition, it also involved the middle cranial and infratemporal fossae with accompanying skull destruction. The tumor was almost totally removed. The histological diagnosis was sebaceous carcinoma with pagetoid changes. Despite surgery and local irradiation, intracranial metastases were recognized one year later, and the patient underwent total tumor removal and whole-brain irradiation. Although multiple lung metastases were detected one year after the second operation, three years post-surgery he remains free of intracranial tumor recurrence. Sebaceous carcinoma of meibomian gland origin with pagetoid changes is a distinct, highly aggressive clinical entity. Early diagnosis and appropriate treatment are essential to improve the prognosis of patients with meibomian gland carcinoma with intracranial extension.