Question: I have Nelson’s syndrome (aggressive enlargement of an ACTH secreting pituitary tumor following a bilateral adrenalectomy) and radiation has not been effective. The tumor is creating problems with my vision. What are my treatment options?

Answer: Since the pituitary tumor has enlarged to affect the nerves that control eye movements or eyesight, the first line of treatment is surgery to remove as much of the tumor as possible. Medical treatment with the oral chemotherapy temozolamide (Temodar) can be tried. Two recently published studies, one from France and one from the University of Virginia, showed that patients with different types of aggressive pituitary tumors (including some patients with Nelson’s syndrome) had a 50% “response rate” (either a decrease in tumor size or no growth over time). Because this is a chemotherapy drug, treatment with Temodar should be prescribed by and supervised by a Neuro Oncologist.

By Dr. Mary Lee Vance MD (Winter, 2010)