Clinical Characteristics, Including Endocrinological Changes, of Optic Glioma in Children

ABSTRACT

PURPOSE: Optic glioma is a primary tumor arising from the optic nerve and/or chiasm. When located in the hypothalamus, it can produce endocrine signs such as diencephalic syndrome, diabetes insipidus, and panhypopituitarism, before and after treatment. We reviewed the clinical features of optic gliomas, especially the endocrinologic manifestations before/after treatment. METHODS: Retrospective clinical characteristics were reviewed. Thyroid function test, combined anterior pituitary hormone test, and growth hormone provocation test were performed before and after surgical resection or radiotherapy. RESULTS: Twenty one patients (male: female 9:12, mean age, 6 yr) diagnosed by pathologic specimens were included. Initial manifestations were decreased visual acuity (47.6%), headache/vomiting (33.3%), diencephalic syndrome (28.6%), nystagmus (23.8%), strabismus (9.5%), proptosis (4.8%), and hydrocephalus (4.8%). Ninteen optic gliomas (90.5%) were in the intracranial location. The other 2 optic gliomas were confined in the orbital cavity, which were associated with neurofibromatosis-1. Endocrinologic review: There were no endocrinologic symptoms or signs in all patients before operation. But there were multiple hypothalamic-pituitary hormonal deficiencies, including growth hormone deficiency (85.7%; complete 71.4%, partial 14.3%), hypothyroidism (64.7%), diabetes insipidus (53%; persistent 41.2%, transient 11.8%), ACTH deficiency (28.6%), and sexual precocity (5.9%), postoperatively. CONCLUSION: We suggest that early-onset of tumor, especially with diencephalic syndrome, and/or intracranial involvement can be considered as a bad prognostic factor. While optic glioma is a pathologically benign tumor, it is not so clinically benign as thought, because of possible permanent hypopituitarism and diencephalic syndrome. Therefore, we have to carefully follow up the patients to look for the complications, such as hypopituitarism, of this tumor after operation.