medwireNews: Tailored management aimed at minimising hypothalamic morbidity has resulted in patients developing fewer hormone deficiencies following surgery for paediatric craniopharyngiomas, research from the UK suggests.

For 100 patients treated between 1973 and 2000, the aim was to achieve complete resection where possible. By their last follow-up, more than 90% of patients were deficient in growth hormone (GH), thyroid-stimulating hormone (TSH) and gonadotrophins, and 85% in adrenocorticotrophic hormone (ACTH). Additionally, 80% had diabetes insipidus and 68% had panhypopituitarism.

A further 85 patients treated between 1998 and 2011 received tailored management with conservative surgery where possible and radiotherapy (RT) where needed “in keeping with a worldwide shift” in paediatric craniopharyngioma management.

“Importantly, the less radical surgical approaches employed and the greater use of adjuvant RT over time were associated with reduced endocrine morbidity”, report Timothy Shao Ern Tan (Royal Manchester Children’s Hospital) and co-researchers in the European Journal of Endocrinology.

The proportions of children deficient in GH, TSH and ACTH were not significantly different to those among patients treated in the earlier time period, but the rate of gonadotrophin deficiency fell to 59%, the rate of diabetes insipidus to 65% and that of panhypopituitarism to 43%.

“The move towards a more conservative approach with adjuvant RT as well as the increased use of the trans-sphenoidal route was likely to have contributed to preserving the pituitary stalk and the hypothalamic–pituitary integrity”, says the team, adding: “This has benefits for survival and reproductive capacity.”

And these improvements did not come at the cost of increased tumour recurrence, with rates of 38% and 32% in the earlier and later eras, respectively.

However, the prevalence of hypothalamic symptoms at last follow-up actually increased between the two treatment eras, from 58% to 80%. Although the researchers attribute the increase to greater awareness among healthcare professionals, they say the finding suggests that “hypothalamic morbidity continues to be a challenging burden”.

Obesity also remained a problem, with the median follow-up body mass index (BMI) standard deviation score (SDS) being 1.33 and 1.94 in the earlier and later treated eras, respectively, and the proportion of patients with a BMI SDS greater than 2.0 being 36% and 46%. Visual morbidity also remained common, although data were available for only 36 patients in the more recent cohort.

“[R]educing the burden of hypothalamic and visual morbidity remains a challenge and more effective targeted therapies are necessary to improve outcomes”, say the researchers.

By Eleanor McDermid

Eur J Endocrinol 2017; Advance online publication

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