Background: Craniopharyngiomas are rare embryogenic malformations of the sellar area with low-grade histological malignancy. Its incidence is 0.52.0 cases per million persons per year, 3050% of all cases become apparent in children.
Objective and hypotheses: The aim of the study was to analyse the clinical symptoms and treatment efficacy in children diagnosed with Craniopharyngioma.
Method: A retrospective analysis included 100 children with craniopharyngioma treated in Institute in years 19992011.
Results: The sex ratio was 1:1 and the median age at primary diagnosis was 8.5 years (2 weeks 18 years old). The symptoms observed included: cranial hypertension (57%), endocrine disorders (70%), vision disorders (54%), a growth failure and decrease of growth rate (50%), diabetes insipidus (8%), delayed puberty (including menstrual disorder) (22%), secondary hypothyroidism (14%), adrenal insufficiency (7%). Complete resection of tumour was achieved in 90% of patients, partial resection in 10%. Overall 23 patients underwent a second surgery due to a local recurrence with a median recurrence time of 2 years (7 months to 8 years old). Postoperative radiotherapy received 19 patients. Most of surgical interventions was made by the same operator (89%). A second recurrence was seen in 2 patients with a median time of 5 years. These patients needed decompression a cystic part of tumour. Postoperative complications included adenohypophysis (100%), diabetes insipidus (89%), visual acuity deterioration (73%), overweight and obesity (62%), and cognitive deficits (19%). The 5-year survival in our series is 90%, 89% at 10 years. However, 7 patients died between 0.55 years after surgery (median 2 years), 5 died due to severe hypothalamo-hypophyseal disturbances, two because of neurosurgical complications.
Conclusion: Craniopharyngiomas are tumours, which can cause many different symptoms depending on tumour localisation, size and the diagnosis promptness. Surgical treatment is effective in most cases, especially if performed by experienced neurosurgeon. Long term survival is high, however complications have serious implications on the quality of life.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology