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Cited by 633Pituitary Gland Disorders and TreatmentsNeuroendocrine Tumor Research AdvancesMeningioma and schwannoma management

Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up

Karavitaki, N., Brufani, C., Warner, J. T., Adams, C. B. T., Richards, P., Ansorge, O., Shine, B., Turner, H. E. +1 more·2005·Clinical Endocrinology
DOI 10.1111/j.1365-2265.2005.02231.x
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Abstract

BACKGROUND: Craniopharyngiomas account for 2-5% of all primary intracranial tumours. Despite their benign histological appearance, they are often associated with an unfavourable prognosis and their optimal treatment remains controversial. AIM: To analyse the natural history and treatment outcome of children and adults presenting to the Departments of Paediatrics and Endocrinology with craniopharyngioma between 1964 and 2003. PATIENTS AND METHODS: The records of 121 patients (age range 2.5-83 years, 42 aged < 16 and 79 aged > or = 16) were identified. The mean follow-up period since presentation was 103 months (8.6 years) (range 0.3-468 months). Sixteen patients underwent gross total removal (A), 3 gross total removal + radiotherapy (B), 51 partial removal (C), 33 partial removal + radiotherapy (D), 6 cyst evacuation alone (E) and 3 cyst evacuation + radiotherapy (F). The clinical, imaging and endocrinological data at presentation and during follow-up were analysed. RESULTS: Headache and visual field defects were the most common presenting clinical features (64% and 55%, respectively). Ninety-four per cent of the tumours had an extrasellar component and 23% of them were associated with hydrocephalus. There was a significant difference in the recurrence-free survival rates between groups A-D [at 10 years: 100% (A), 100% (B), 38% (C) and 77% (D), P < 0.01], which persisted even when analysing patients operated after 1980. The median time of first recurrence was 2.5 years (range 0.5-36). The peri-operative mortality of the patients who had any type of neurosurgical intervention due to recurrence was higher than that observed after primary surgery (24%vs. 1.8%) (P < 0.01). The rate of re-accumulation of the cyst fluid was 58% during the first year in patients of group E, whereas none of the subjects of group F experienced such an event during their follow-up period. There was no reversal of pre-existing pituitary hormone deficits after any surgical intervention. The probabilities of GH, FSH/LH, ACTH, TSH deficiency and diabetes insipidus at the 10-year follow-up were 88%, 90%, 86%, 80% and 65%, respectively. After excluding the non-tumour-related deaths, the 10-year survival rate following presentation was 90%. Patients with recurrence had a significantly lower probability for survival compared with those without it (at 10 years: 70%vs. 99%, P < 0.01). At the 10-year follow-up the probability of the presence of major visual field defects was 48%, hyperphagia/obesity 39%, epilepsy 12% and hemi-/monoparesis 11%. In this large series no substantial differences in the outcome of tumours diagnosed during childhood or adult life were found. CONCLUSIONS: Craniopharyngiomas remain tumours associated with significant morbidity. Gross total removal provides favourable results in terms of recurrences. If this cannot be achieved safely, adjuvant radiotherapy is beneficial in preventing tumour re-growth. Childhood- and adult-onset lesions generally behave similarly.

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Authors
Karavitaki, N., Brufani, C., Warner, J. T., Adams, C. B. T., Richards, P., Ansorge, O., Shine, B., Turner, H. E., & Wass, J. A. H.
Affiliations
  • Churchill Hospital (GB)
  • John Radcliffe Hospital (GB)
  • University of Oxford (GB)
Corresponding author
Wass, J. A. H.
ORCID
Venue
Clinical Endocrinology
Publisher
Wiley
Published
2005-03-24
Volume
62
Issue
4
Pages
397-409
ISSN
  • 0300-0664 (ISSN-L)
  • 1365-2265
DOI
10.1111/j.1365-2265.2005.02231.x
OpenAlex
W2090331475
Cited by
633
References
66
Citations by year
2026: 18 · 2025: 28 · 2024: 34 · 2023: 35 · 2022: 31 · 2021: 29 · 2020: 44 · 2019: 38 · 2018: 30 · 2017: 31 · 2016: 30 · 2015: 40 · 2014: 36 · 2013: 27 · 2012: 38
FWCI
1.58
Type
journal article
Language
en
Open access
closed
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • Clinical Endocrinology (publishedVersion)
  • PubMed (publishedVersion)
  • University of Birmingham Research Portal (University of Birmingham) (submittedVersion)
Topics
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Epidemiology › Meningioma and schwannoma management
Keywords
craniopharyngioma, pediatric craniopharyngioma, natural history (disease), recurrence-free survival, surgical treatment, radiotherapy, cyst evacuation, growth hormone deficiency, visual field defects, hydrocephalus, long-term follow-up, treatment outcomes, perioperative mortality, children versus adults, prognosis, endocrinology
MeSH terms
Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Craniopharyngioma, Female, Follow-Up Studies, Humans, Male, Middle Aged, Neoplasm Recurrence, Local, Pituitary Neoplasms, Survival Rate, Chi-Square Distribution, Disease-Free Survival, Statistics, Nonparametric

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