An analysis of craniopharyngioma patients in Malta : epidemiology, patient characterisation and long-term sequelae

Abstract

BACKGROUND: Despite being benign, craniopharyngiomas are challenging tumours to manage and can cause significant morbidity and mortality in both the paediatric and adult population.

METHOD: Our aim was to analyse epidemiology, patient characteristics and long-term sequalae through a population-based study in Malta. A thorough research was carried out to identify patients who were diagnosed with craniopharyngioma in our local population. Subjects were identified from various hospital databases. Presenting features, patient and tumour characteristics, treatment modalities, long-term sequelae and epidemiology were analysed.

RESULTS: From a cohort of 29 patients, 62.1% were male. The mean age at presentation was 32.4 years (s.d. ± 19.0). 11 patients (37.9%) were diagnosed with childhood onset craniopharyngioma (age at presentation < 20 years) whilst 18 patients (62.1%) had adult-onset craniopharyngioma. Median follow up period since time of diagnosis was 13.0 years (IQR 5–25). For incidence estimates, 13 patients who were diagnosed between June 2008 and June 2019 were included. The background population formed 4.8 million patient-years at risk. The overall SIR was 3.0/1,000,000/year, with the highest SIR in the 10–19 year age group. The estimated prevalence rate was 52.7/1,000,000 people, with lower prevalence rates for childhood- compared to adult-onset (20.3/1,000,000 vs 32.4/1,000,000 people). Visual disturbances and symptoms secondary to raised intracranial pressure were the commonest presenting complaints. Most tumours were multi-cystic (42.9%) and were commonly located in the intrasellar region with suprasellar extension. The median longest tumour diameter was 31.0 mm (IQR 21–41), with statistically significant difference between childhood- and adult-onset disease; 43.0 mm (IQR 42.5–47.25) vs 27.0 mm (IQR 20.55–31.55) (P = 0.011). All 24 patients who underwent neurosurgical intervention (82.8%) had adamantinomatous craniopharyngioma. 58.6% of patients required radiotherapy. The commonest long-term sequalae were hormone deficiencies (93.1%), followed by obesity (20.7%). Most patients required hormonal supplementation of more than one pituitary axis. 7 patients (30.4%) had evidence of tumour regrowth or recurrence during follow-up. 3 patients passed away throughout their follow up.

CONCLUSION: From a cohort of 29 patients, 62.1% were male. The mean age at presentation was 32.4 years (s.d. ± 19.0). 11 patients (37.9%) were diagnosed with childhood onset craniopharyngioma (age at presentation < 20 years) whilst 18 patients (62.1%) had adult-onset craniopharyngioma. Median follow up period since time of diagnosis was 13.0 years (IQR 5–25). For incidence estimates, 13 patients who were diagnosed between June 2008 and June 2019 were included. The background population formed 4.8 million patient-years at risk. The overall SIR was 3.0/1,000,000/year, with the highest SIR in the 10–19 year age group. The estimated prevalence rate was 52.7/1,000,000 people, with lower prevalence rates for childhood- compared to adult-onset (20.3/1,000,000 vs 32.4/1,000,000 people). Visual disturbances and symptoms secondary to raised intracranial pressure were the commonest presenting complaints. Most tumours were multi-cystic (42.9%) and were commonly located in the intrasellar region with suprasellar extension. The median longest tumour diameter was 31.0 mm (IQR 21–41), with statistically significant difference between childhood- and adult-onset disease; 43.0 mm (IQR 42.5–47.25) vs 27.0 mm (IQR 20.55–31.55) (P = 0.011). All 24 patients who underwent neurosurgical intervention (82.8%) had adamantinomatous craniopharyngioma. 58.6% of patients required radiotherapy. The commonest long-term sequalae were hormone deficiencies (93.1%), followed by obesity (20.7%). Most patients required hormonal supplementation of more than one pituitary axis. 7 patients (30.4%) had evidence of tumour regrowth or recurrence during follow-up. 3 patients passed away throughout their follow up.