Macroprolactinoma with an atypical course

Prolactinomas are among the most common pituitary adenomas. The typical symptoms of hyperprolactinemia associated with a prolactinomaprimarily include symptoms of hypogonadism resulting from the suppression of the hypothalamic-pituitary-gonadal axis.In men and postmenopausal women, symptoms related to the mass effect of the tumor usually predominate. In some cases, however,prolactinomas may initially present with atypical symptoms, which can make it difficult to establish the correct diagnosis. An extensive literature search covering the past 30 years was conducted using the PubMed database. The following search terms were applied: "macroprolactinoma", "atypical prolactinoma", and "giant prolactinoma manifestations". Only atypical cases of pituitary macroadenomasassociated with laboratory-confirmed hyperprolactinemia (prolactin concentrations > 100 ng/mL) were included. In ourstudy, we also included 3 cases of macroprolactinoma with an atypical presentation at the time of diagnosis, which we encountered inour department. Overall, 15 articles met the inclusion criteria. Including the cases described in this study, 28 patients with macroprolactinoma presenting with atypical symptoms at diagnosis were identified. Most patients were men, and the majority were aged 40-60 years. The most commonatypical manifestations included neurological symptoms such as epilepsy, cranial nerve palsy, hydrocephalus or behavioral disorders,as well as otolaryngological symptoms such as recurrent epistaxis or nasal obstruction. Macroprolactinomas were most commonlymisdiagnosed as skull base tumors, sinonasal malignancies, or primary neurological disorders. In most cases, the initiation of dopamineagonist therapy led to rapid clinical improvement and partial or complete tumor regression, even in cases of giant macroprolactinomas. A proportion of well- or moderately differentiated olfactory neuroblastomas may, in fact, be misdiagnosed macroprolactinomas. If other endocrine tumors are present, the typical symptoms of hyperprolactinemia may be masked. In some cases, macroprolactinomas are diagnosedwhen the condition becomes life-threatening. Serum prolactin levels should be measured in any patient with a sellar or parasellar tumor.