ESPE2021 Meet the Expert Diagnosis and management of a child with pituitary adenoma (1 abstracts)
Odense University Hospital, Odense, Denmark
A prolactinoma is the most common pituitary adenoma, but a pituitary adenoma is rarely diagnosed in children. Corticotropinomas and somatotropinomas are observed, whereas clinically non-functioning pituitary adenomas, TSH-adenomas and gonadotroph adenomas are very rare in children; a minor percentage of pituitary tumours are aggressive. Genetic cause and syndromic disease are more often seen in children and adolescents with a pituitary adenoma compared to adults, and it is necessary to perform genetic screening in most pediatric patients with a pituitary adenoma to optimize therapy and follow-up. Dopamine-D2-receptor-agonist therapy is first-line treatment also in pediatric patients with a prolactinoma, and impaired gonadal function will usually be restored, when prolactin levels are normalized. Surgery is primary therapy in Cushings disease, gigantism or acromegaly, but a considerable number of patients will need additionally medical therapy and in some cases radiotherapy. Hypopituitarism needs to be considered at the time of diagnosis of a pituitary adenoma, after therapy and during follow-up, especially after radiotherapy. The 2017 World Health Organization (WHO) classification system on the pituitary is based on tumour cell lineage and transcriptional factor nuclear staining. Sometimes we need no more than a scan of the pituitary region to decide, that the tumour is aggressive, however, in selected cases, it is necessary to assess tumour proliferative potential by mitotic count and Ki-67 index. Experimental medical therapy, e.g. temozolomide, is indicated in few pediatric patients with an aggressive or malignant pituitary tumour, and we need O6-methylguanine-DNA methyl transferase (MGMT) status for the clinical decision making. Close collaboration between the pediatrician and endocrinologist is very fruitful during transition to adulthood.