Endocrine Abstracts

Background: Pituitary adenomas in children/adolescents represent around 3% of all intracranial neoplasms. They are more frequently hormone-secreting lesions, usually diagnosed in early childhood and late adolescence. Female gender is generally prevalent, because of most evident symptoms (i.e., irregular periods, galactorrhea, etc.). Giant pituitary adenomas (GA) very rarely occur in pediatric age, posing frequent challenges in their management.

Patients and methods: We retrospectively evaluated the records of 7 teenager patients (5 males, median age 16.7 ± 1.6 yrs) with GA, referred to the Endocrine Unit of the University Hospital of Messina (Italy) from 1990 to 2020. All patients underwent biochemical, clinical and neuroradiological workup. The median follow-up was 16.1 ± 9.9 years.

Results: GAs were clinically characterized as follows: five functioning PAs (4 PRL-secreting, 1 ACTH-secreting) and two non-functioning pituitary adenomas (NFPAs). Median pituitary tumor diameter was 43 ± 2.2 mm. Genetic analysis revealed only 1 carrier of MEN1 mutation (a patient with PRL-secreting tumor), while no changes of AIP gene were detected. At diagnosis, main presenting symptoms were headache (n.5), visual disturbances (n.4), menstrual irregularities (n.2), and growth delay (n.1). In terms of pituitary function, 1 patient presented with panhypopituitarism, 2 with multiple pituitary deficits. One patient with NFPA was lost to follow-up, of the remaining 6 patients: 3 (1 PRL, 1 ACTH, 1 NFPA) were referred to first-line surgery by endoscopic trans-nose-sphenoidal approach, 3 PRL-omas were treated with medical therapy (cabergoline) exclusively or prior to surgery. After first-line treatment, stable remission (no tumor remnant and/or no hormone hypersecretion) was observed in 3 subjects, progression (growing tumor remnant and/or persistent hormonal hypersecretion) in 3 cases. In these last cases, second-line treatment was radiotherapy. At last follow-up visit: 5 patients were in remission while 1 patient with PRL secreting tumor was still under effective cabergoline treatment; 2 patients had panhypopituitarism, 3 single/multiple pituitary deficits, while pituitary function was preserved in one subject. GH deficiency (GHD), isolated as well in association with other deficits, was the most frequent pituitary hormone deficit (n.5/7 patients). During follow-up, the following comorbidities were diagnosed: metabolic syndrome (n.3), osteoporosis (n.2), second tumors (n.1).

Conclusions: Giant pituitary adenomas management in children/adolescents is challenging and require a multidisciplinary approach.