Very long-term sequelae of craniopharyngioma

Eur J Endocrinol. 2017 Jun;176(6):755-767. doi: 10.1530/EJE-17-0044. Epub 2017 Mar 21.

Abstract

Objective: Studies investigating long-term health conditions in patients with craniopharyngioma are limited by short follow-up durations and generally do not compare long-term health effects according to initial craniopharyngioma treatment approach. In addition, studies comparing long-term health conditions between patients with childhood- and adult-onset craniopharyngioma report conflicting results. The objective of this study was to analyse a full spectrum of long-term health effects in patients with craniopharyngioma according to initial treatment approach and age group at craniopharyngioma presentation.

Design: Cross-sectional study based on retrospective data.

Methods: We studied a single-centre cohort of 128 patients with craniopharyngioma treated from 1980 onwards (63 patients with childhood-onset disease). Median follow-up since craniopharyngioma presentation was 13 years (interquartile range: 5-23 years). Initial craniopharyngioma treatment approaches included gross total resection (n = 25), subtotal resection without radiotherapy (n = 44), subtotal resection with radiotherapy (n = 25), cyst aspiration without radiotherapy (n = 8), and 90Yttrium brachytherapy (n = 21).

Results: Pituitary hormone deficiencies (98%), visual disturbances (75%) and obesity (56%) were the most common long-term health conditions observed. Different initial craniopharyngioma treatment approaches resulted in similar long-term health effects. Patients with childhood-onset craniopharyngioma experienced significantly more growth hormone deficiency, diabetes insipidus, panhypopituitarism, morbid obesity, epilepsy and psychiatric conditions compared with patients with adult-onset disease. Recurrence-/progression-free survival was significantly lower after initial craniopharyngioma treatment with cyst aspiration compared with other therapeutic approaches. Survival was similar between patients with childhood- and adult-onset craniopharyngioma.

Conclusions: Long-term health conditions were comparable after different initial craniopharyngioma treatment approaches and were generally more frequent in patients with childhood- compared with adult-onset disease.

Publication types

  • Comparative Study

MeSH terms

  • Age of Onset
  • Aging*
  • Child
  • Child, Preschool
  • Cohort Studies
  • Combined Modality Therapy / adverse effects
  • Craniopharyngioma / complications
  • Craniopharyngioma / physiopathology*
  • Craniopharyngioma / surgery
  • Craniopharyngioma / therapy
  • Cross-Sectional Studies
  • Female
  • Follow-Up Studies
  • Humans
  • Hypopituitarism / epidemiology
  • Hypopituitarism / etiology*
  • Male
  • Neoplasm Recurrence, Local / epidemiology
  • Neoplasm Recurrence, Local / prevention & control
  • Netherlands / epidemiology
  • Obesity / complications*
  • Obesity / epidemiology
  • Pituitary Neoplasms / physiopathology*
  • Pituitary Neoplasms / surgery
  • Pituitary Neoplasms / therapy
  • Prevalence
  • Retrospective Studies
  • Risk
  • Survival Analysis
  • Vision Disorders / epidemiology
  • Vision Disorders / etiology*

Supplementary concepts

  • Combined Pituitary Hormone Deficiency