Survivors of pediatric optic pathway hypothalamic glioma (OPG) have a high risk of long-term endo-metabolic and neuro-behavioral comorbidities, according to a study presented at the 20th International Symposium on Pediatric Neuro-Oncology (ISPNO).

The study showed that the etiology of these comorbidities depends on hypothalamic tumor location and treatments received.

Researchers evaluated specific risk factors for poor long-term outcomes occurring between 1981 and 2020 in 90 patients diagnosed with OPG before their third birthday.

Continue Reading

Overall, 57.8% of patients had endo-metabolic dysfunction, occurring at a median of 3.4 years (range, 0.05-13.64 years) after OPG diagnosis. The endocrine event-free survival rate was 54.1% at 5 years, 31.4% at 10 years, and continued to decline up to 13.6 years.

As far as anterior pituitary dysfunction outcomes, growth hormone deficiency occurred in 41.1% of patients (at a median of 5.3 years), central precocious puberty in 26.7% (at 3.8 years), central hypothyroidism in 23.3% (at 5.9 years), central hypocortisolism in 22.2% (at 5.3 years), and gonadotropin deficiency in 18.9% (at 12.7 years). 

The occurrence of anterior pituitary dysfunction was increased by diencephalic syndrome presentation (P =.001), hypothalamic involvement (P =.005), radiotherapy (P =.017), and surgery (P =.015).

As for posterior pituitary dysfunction outcomes, inappropriate antidiuretic hormone secretion syndrome occurred in 11.1% of patients (at a median of 2.8 years), central diabetes insipidus in 8.9% (at 0.8 years), and cerebral salt wasting syndrome in 1 patient (at 15.28 years).

Posterior pituitary dysfunction occurred in 15 patients (16.7%). It only occurred after surgery and/or as a consequence of hydrocephalus in patients with chiasmatic/post-chiasmatic location and hypothalamic disease.

Regarding metabolic dysfunction, obesity occurred in 25.5% of patients, at a median of 6.8 years after OPG diagnosis. Diabetes or impaired glucose tolerance occurred in 12.2% of patients, at a median of 8.1 years after OPG diagnosis.

Endo-metabolic dysfunction was more likely to occur in patients with neuro-behavioral complications (P <.0001).

Neuro-behavioral deficits were observed in more than half of the cohort (n=52) and were associated with hypothalamic involvement (P =.043) and radiotherapy (P =.003).

Based on these findings, the researchers concluded that early referrals for endocrine and neuro-developmental care are likely needed to improve long-term quality of life in this patient population.

Picariello S, Cerbone M, D’Arco F, et al. A 40-year cohort study of evolving hypothalamic dysfunction in 90 infants and young children (<3y) with optic pathway gliomas. Presented at ISPNO 2022; June 12-15, 2022. Abstract LGG-33.