Pituitary tumors: epidemiology and clinical presentation spectrum

Pituitary tumors (PTs) are a heterogeneous group of lesions of the central nervous system that are usually benign. Most of them occur sporadically, but 5% can do so within family syndromes, usually at a young age. There are differences by sex, age, race, and genetic factors in the prevalence of different tumor cell types and clinical presentation. Functioning-PTs (FPTs) are usually diagnosed earlier than non-functioning PTs (NFPTs). However, this depends on the PT type. Headaches and visual disturbances are the most frequent mass-effect symptoms, but seizures or hydrocephalus may also occur. Pituitary apoplexy is another possible mode of presentation, and it requires special attention because of its potential severity. PTs in pregnancy, childhood, and old age present a series of clinical peculiarities that must be taken into account when evaluating these patients. Ectopic PTs (EPTs) are uncommon and share the same clinical-epidemiological data as eutopic PTs, but, depending on their location, other types of clinical manifestations may appear. Silent PTs are often detected as an incidentaloma or due to neurologic symptoms related to mass-effect. Aggressive PTs and pituitary carcinomas (PCs), which are very rare, are characterized by multiple local recurrences and metastases, respectively. This review addresses the epidemiology and clinical presentation of PTs, from the classical hormonal and mass-effect symptoms to the different rare presentations, such as pituitary apoplexy, hydrocephalus, or diabetes insipidus. Moreover, special situations of the presentation of PTs are discussed, namely, PTs in pregnancy, childhood, and the elderly, EPTs, silent and aggressive PTs, and PCs.