Aggressive Pituitary Adenomas: The Dark Side of the Moon

BACKGROUND: Although pituitary adenomas are consi-dered benign lesions, a small group may show clinically aggressive behavior, sometimes independently from the classic markers of aggressiveness, including the Ki67 la-beling index or p53 expression. METHODS: We selected 7 patients harboring a pituitary tumor with clinical features of aggressiveness. Patients underwent a full preoperative and postoperative endocri-nologic and neuroradiologic workup. Two were nonfunc-tioning, 2 prolactin-secreting, 2 adrenocorticotrophic hormoneesecreting, and 1 a growth hormoneesecreting adenoma. RESULTS: The 7 patients underwent a total of 17 surgical procedures. At the first surgical procedure, gross total removal was achieved in none of the patients, whereas subtotal removal (> 90% of tumor removed) was achieved in 4/7 cases and partial removal (< 90% of tumor removed) in 3/7 cases. At first operation, 4/7 patients showed a Ki67 index <= 3% and 2/7 > 3%; this information was not available for 1 patient. Postoperatively, all patients underwent radiation therapy. Three patients received chemotherapy with temozolomide. Three patients underwent peptide receptor radionuclide therapy. To date, 1 patient has died of tumor progression, and 2 patients are in a poor general condition. The remaining 4 patients are in a fair/good condition, without any major complaints. The mean follow-up is 43.42 months. CONCLUSIONS: Aggressive pituitary adenomas repre-sent a specific and still underestimated entity, often diag-nosed late. Clinical and neuroradiologic rapid progression is often the only marker of aggressiveness. Surgical debulking remains the first therapeutic option. Multidisci-plinary management is mandatory to offer these patients targeted therapeutic options.