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In the last years, molecular genetic features have been integrated into the diagnosis of several types of the parasellar tumours enhancing diagnostic accuracy and providing information of the value for targeting therapies. In this review, we will present histopathological and molecular genetic features, updated classification criteria, recent advances in the diagnostics and rationale for novel pharmacological therapies of selected types of parasellar neoplasms. © 2020 S. Karger AG, Basel.BACKGROUND Malignant pleural effusion (MPE) pos