Endocrine manifestations of pediatric CNS tumors at diagnosis and as sequalae of treatment.

Overview

Pediatric CNS tumors may be associated with endocrinopathies at the time of initial diagnosis and as a sequalae of their treatment. Endocrine dysfunction is highly prevalent among tumors located along the hypothalamic pituitary axis and optic pathway, with manifestations such as precocious puberty, diabetes insipidus, or growth failure presenting initially without neurologic symptoms. Posterior fossa tumors, which are more common in pediatrics, can also present with endocrine dysfunction despite their relatively more distant location due to their propensity for causing hydrocephalus. The various treatment modalities for CNS tumors portend additional risks for developing endocrinopathies. Acute endocrine dysfunction often follows surgery involving the HP axis, while endocrine late effects, particularly following radiation exposure of the HP axis, can develop more insidiously years to decades after completion of treatment. Chemotherapy and newer targeted and immunotherapies can cause peripheral endocrine gland as well as HP axis dysfunction. With an increasing number of childhood cancer survivors in the population, recognition and treatment of endocrine late effects is increasingly important. We review here the common endocrine dysfunction associated with various CNS tumors at diagnosis and as a consequence of their treatment.