Optic Chiasm Astrocytomas of Childhood

Abstract

We reviewed 61 patients seen from 1975 to 1994 with a biopsy-confirmed optic chiasm astrocytoma or a characteristic clinical and imaging presentation. The mean age at diagnosis was 72 months, including 30 who were less than 5 years old. The mean tumor diameter at presentation was 31 mm among 54 tumors measured. Tumors involved the optic nerve in 41, hypothalamus in 38, optic tract in 15, and optic radiations in 2. Four patients, all with neurofi-bromatosis, received no treatment. Forty patients received conventional radiation therapy at a mean age of 89 months. Chemotherapy was given to 19 children and 7 of these were followed for greater than 1 year. Six of these 7 had progressive disease which required subsequent surgery and/or radiation therapy. Thirty-six patients underwent surgery. We restricted our analysis of outcome to those 38 patients followed for longer than 60 months. Development was normal in 13%, mildly disabled in 53%, severely disabled in 21% and dead in 13%. Children were severely disabled or dead in 47% if younger than 5 years at diagnosis, and 21% if older. There was no endocrine dysfunction in 18% and new endocrine failure was seen in 61%. More posterior tumors did not portend a poorer prognosis. Children less than 5 years old had a poorer prognosis. Neurofibromatosis did not have a marked effect on outcome. Intracranial hypertension was an indicator of a poor prognosis. No specific treatment modality was clearly superior in terms of long-term survival or improvement in quality of life.

Brain Tumors in Children Under 3 Years of Age

“…Chiasm and hypothalamic gliomas are common in this age group and are often referred to as low-grade tumors [34, 38, 40, 41, 42]. However, in our experience as well as that of others, they still represent a complex medical and surgical challenge in young children [1, 41].…”

“…Brown et al [13]surveyed 88 children under 2 years of age and reported that all deaths in patients operated for benign tumors occurred in those with optic chiasm/hypothalamic tumors. Larger series have suggested that mortality and morbidity rates may be reduced when surgery is limited to debulking large symptomatic exophytic or cystic tumors, relieving hydrocephalus or providing a histological diagnosis for further adjuvant treatment [40, 41, 42]. …”

Late Effects of Therapy of Thalamic and Hypothalamic Tumors in Childhood: Vascular, Neurobehavioral and Neoplastic

“…The main risk factors of permanent neurologic and endocrinologic injury are young age, extensive tumor resections and RT [2, 10, 13]. …”

“…Several studies have attempted to measure quality of life and performance of children surviving brain tumors, even though the quality of life measures lack standardization [12, 13, 14, 15, 16]. Ellenberg et al [10]conducted a prospective study of intellectual outcome in 73 children with brain tumors consecutively admitted to the hospital over a period of 3 years.…”

Hypothalamic-Opticochiasmatic Gliomas Mimicking Craniopharyngiomas

“…HOCGs have distinct age-related presentations, with infants usually exhibiting macrocephaly, failure to thrive and profound visual loss. Children less than 5 years of age harboring HOCGs commonly present with endocrinopathies, while older children primarily complain of visual loss with endocrinopathies as a secondary complaint [5]. Preoperative evaluation includes thorough endocrine and ophthalmologic examination in conjunction with neuroimaging studies, including skull radiographs, computed tomography (CT) and magnetic resonance imaging (MRI).…”