Abstract
Background/Purpose: A prolactinoma is the most common pituitary adenoma, but it is relatively rare in childhood and adolescence. There is only limited research about the clinical spectrum, treatment, and outcomes of prolactinomas in childhood and adolescence. In this single-center cohort study, we assessed the clinical, hormonal, and neuroradiological characteristics and therapeutic outcomes of children and adolescents with prolactinomas.Methods: This retrospective cohort study included 25 patients with prolactinomas diagnosed before 19 years of age, who presented at Samsung Medical Center during a 15-year period (March 2005 to August 2019).Results: The median age at diagnosis was 16.9 (range 10.1–18.5) years, and 80% of the patients were female. The common clinical manifestations at diagnosis were galactorrhea (10/20, 50%) and amenorrhea (9/20, 45%) among females and visual field defects (3/5, 60%) and headaches (2/5, 40%) among males. In our cohort, macroadenomas accounted for 56% of cases, and the rate of overall responsiveness to dopamine agonists (DAs) was 56% (10/18). Male gender, the prolactin (PRL) level at diagnosis, and the presence of panhypopituitarism were positively correlated with maximum tumor diameter (r = 0.443, P = 0.026; r = 0.710, P < 0.001; and r = 0.623, P = 0.001, respectively). After the trans-sphenoidal approach (TSA), 53% (8/15) of patients showed normalization of the PRL level. Three patients, who underwent gamma knife surgery (GKS) owing to either resistance or intolerance to DAs or recurrence after the TSA, achieved a normal PRL level accompanied with marked tumor reduction and symptom remission.Conclusions: A macroprolactinoma is more prevalent than a microprolactinoma in children and adolescents than in adults. Male gender, increased PRL levels, and the presence of panhypopituitarism at diagnosis are closely related to macroprolactinomas in children and adolescents.
Highlights
A prolactinoma is the most common pituitary adenoma, which is characterized by lactotroph cells secreting prolactin (PRL) and monoclonal expansion of single cells in the pituitary [1]
Hyperprolactinemia inhibits the pulsatile secretion of gonadotropin-releasing hormone (GnRH) from the hypothalamus, which is required for follicle-stimulating hormone (FSH) and luteinizing hormone (LH) secretion from the pituitary gland
The diagnosis of a prolactinoma was based on typical clinical signs and symptoms, brain magnetic resonance imaging (MRI) findings, a PRL level above the normal range in at least two evaluations, and lactotroph adenoma confirmed by immunohistochemistry in patients who received Trans-sphenoidal surgical approach (TSA)
Summary
A prolactinoma is the most common pituitary adenoma, which is characterized by lactotroph cells secreting prolactin (PRL) and monoclonal expansion of single cells in the pituitary [1]. It usually occurs sporadically; it is dominantly inherited with germline mutations in the AIP or MEN1 gene in ∼5% of cases [2, 3]. Clinical manifestations of a prolactinoma can result from the overproduction of prolactin and mass effects. Macroprolactinoma, more often diagnosed in boys, may be associated with headaches, visual field defects, or other neurologic deficits that are mainly caused by mass effect [1, 12]
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