Abstract

In the World Health Organization 2022 classification, a nomenclature change from pituitary adenoma to pituitary neuroendocrine tumor(PitNET)was introduced to indicate rare but potentially malignant behavior. No grading system is available for PitNETs; therefore, the establishment of a system that corresponds to their clinical behavior is an urgent issue. Presently, this change has no direct influence on therapeutic strategies. Recently, the therapeutic outcomes of most patients with PitNETs have significantly improved owing to marked advancements in both surgical and medical treatments. The former includes the evolution of endoscopic surgery and technical refinements, whereas the latter includes the introduction of new effective drugs and increased knowledge and experience regarding their use, leading to personalized and/or precision medicine. Consequently, the treatment goals have advanced, encompassing endocrinological remission, successful management of comorbidities, increased health-related quality of life, and a normalized mortality rate. However, management of some aggressive and metastatic PitNETs remains difficult. Although temozolomide(TMZ)is considered a promising sole therapeutic option, recent reports have shown that TMZ does not provide long-term control in many cases. A multidisciplinary approach is necessary for the reliable prediction and successful management of aggressive tumors.

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