• Tumor invades periorbita, nerves, and bone. Intracranial   spread is major cause of death.
• Treatment is controversial. Most aggressive treatment   consisting of exenteration, orbitectomy, and radiation   therapy has failed to demonstrate increased survival.   Despite aggressive treatment, most recur locally or with   metastasis.
• Recurrence 5 to 20 years after initial diagnosis.
• New treatment modalities – proton beam radiation therapy,   selective intra-arterial chemotherapy.