Gregory A Kaltsas1,2, Dionysia Kolomodi1, Harpal Randeva2, Ashley Grossman3. 1. First Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece. 2. WISDEM Centre, University Hospital of Coventry and Warwickshire, Coventry, United Kingdom. 3. Centre for Endocrinology, Barts and the London School of Medicine, Queen Mary University of London, London, United Kingdom.
Abstract
CONTEXT: Although most sellar lesions are related to pituitary adenomas, the region gives rise to a variety of neoplasms that can be associated with substantial morbidity and/or mortality. DESIGN: Information from reviews and guidelines of relevant societies dealing with such neoplasms, as well as articles that have provided new developments that made important contributions to their pathogenesis and treatment up to 2018, were obtained: public indexes such as PubMed/MEDLINE were used with the relevant search items. RESULTS: Sellar neoplasms have a worse outcome than pituitary adenomas that is related not only to their natural history but also to side effects of therapies and evolving endocrine and/or hypothalamic deficiencies. Recent imaging advances have established the radiological fingerprint of some of these neoplasms, and several chromosomal aberrations have also been identified. Although established approaches along with new surgical and radiotherapeutic approaches remain the main treatment modalities, recent evidence has provided insight into their molecular pathogenesis involving, other than chemotherapy, treatments with targeted agents as in gliomas and craniopharyngiomas bearing BRAF mutations. Development of predictive markers of recurrences may also identify high-risk patients, including proliferative markers and expression of the progesterone receptor in meningiomas, and lead to less aggressive surgery. Owing to the rarity and complexity of these neoplasms, patients should be managed in dedicated centers. CONCLUSIONS: The diagnosis and management of sellar neoplasms necessitate a multidisciplinary approach. Following evolving recent advances in their diagnosis and therapy, such a multidisciplinary approach needs to be extended to establish evidence-based diagnostic and management plans.
CONTEXT: Although most sellar lesions are related to pituitary adenomas, the region gives rise to a variety of neoplasms that can be associated with substantial morbidity and/or mortality. DESIGN: Information from reviews and guidelines of relevant societies dealing with such neoplasms, as well as articles that have provided new developments that made important contributions to their pathogenesis and treatment up to 2018, were obtained: public indexes such as PubMed/MEDLINE were used with the relevant search items. RESULTS:Sellar neoplasms have a worse outcome than pituitary adenomas that is related not only to their natural history but also to side effects of therapies and evolving endocrine and/or hypothalamic deficiencies. Recent imaging advances have established the radiological fingerprint of some of these neoplasms, and several chromosomal aberrations have also been identified. Although established approaches along with new surgical and radiotherapeutic approaches remain the main treatment modalities, recent evidence has provided insight into their molecular pathogenesis involving, other than chemotherapy, treatments with targeted agents as in gliomas and craniopharyngiomas bearing BRAF mutations. Development of predictive markers of recurrences may also identify high-risk patients, including proliferative markers and expression of the progesterone receptor in meningiomas, and lead to less aggressive surgery. Owing to the rarity and complexity of these neoplasms, patients should be managed in dedicated centers. CONCLUSIONS: The diagnosis and management of sellar neoplasms necessitate a multidisciplinary approach. Following evolving recent advances in their diagnosis and therapy, such a multidisciplinary approach needs to be extended to establish evidence-based diagnostic and management plans.