Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics

Abstract

Pituitary tumors are rare in childhood and adolescence, with a reported prevalence of up to one per 1 million children. Only 2–6% of surgically treated pituitary tumors occur in children. Although pituitary tumors in children are almost never malignant and hormonal secretion is rare, these tumors may result in significant morbidity. Tumors within the pituitary fossa are mainly of two types: craniopharyngiomas and adenomas. Craniopharyngiomas cause symptoms by compressing normal pituitary, causing hormonal deficiencies and producing mass effects on surrounding tissues and the brain; adenomas produce a variety of hormonal conditions such as hyperprolactinemia, Cushing disease and acromegaly or gigantism. Little is known about the genetic causes of sporadic lesions, which comprise the majority of pituitary tumors, but in children, more frequently than in adults, pituitary tumors may be a manifestation of genetic conditions such as multiple endocrine neoplasia type 1, Carney complex, familial isolated pituitary adenoma and McCune–Albright syndrome. The study of pituitary tumorigenesis in the context of these genetic syndromes has advanced our knowledge of the molecular basis of pituitary tumors and may lead to new therapeutic developments.

Keywords::

• acromegaly
• brain tumor
• Cushing disease
• MEN
• multiple endocrine neoplasia
• prolactinoma

Financial & competing interests disclosure

The present work was supported by the US NIH, National Institute of Child Health & Human Development (NICHD) intramural project Z01-HD-000642-04 to Constantine A Stratakis. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.