Epidemiology of craniopharyngiomas: a population-based study in Malta

in Endocrine Oncology
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  • 1 Department of Medicine, Faculty of Medicine and Surgery, University of Malta, Mater Dei Hospital, Msida, Malta
  • 2 Department of Medicine, Mater Dei Hospital, Msida, Malta

Correspondence should be addressed to S Craus: crausarah@gmail.com

Background

Despite being benign tumours, craniopharyngiomas are challenging to manage and can cause significant morbidity and mortality in both the paediatric and adult population. The aim of the study was to analyse the epidemiology of craniopharyngiomas, patient and tumour characteristics through a population-based study in Malta, enabling a better quantification of the disease burden.

Methods

Thorough research was carried out to identify the number of patients who were diagnosed with craniopharyngiomas. Epidemiological data, including both standardised incidence rates (SIR) and prevalence rates, were established in a well-defined population. For incidence estimates, patients who were diagnosed between 2008 and 2019 were included. The background population formed 4.8 million patient-years at risk.

Result

Twenty-nine subjects were identified and included in our study. The overall SIR was 0.3/100,000/year, with a higher SIR for males compared to females (0.4/100,000/year and 0.2/100,000/year, respectively). The highest SIR was recorded in the 10–19 year age group. The estimated prevalence rate amounted to 5.27/100,000 people, with a lower prevalence rate for childhood-onset when compared to the adult-onset category (2.03/100,000 vs 3.24/100,000 people). The median longest tumour diameter was 31.0 mm (IQR 21–41), with a 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).

Conclusion

Through this population-based study, accurate and up-to-date prevalence and incidence rates for craniopharyngiomas are reported. These provide a clearer reflection of the true health burden of the disease.

Abstract

Background

Despite being benign tumours, craniopharyngiomas are challenging to manage and can cause significant morbidity and mortality in both the paediatric and adult population. The aim of the study was to analyse the epidemiology of craniopharyngiomas, patient and tumour characteristics through a population-based study in Malta, enabling a better quantification of the disease burden.

Methods

Thorough research was carried out to identify the number of patients who were diagnosed with craniopharyngiomas. Epidemiological data, including both standardised incidence rates (SIR) and prevalence rates, were established in a well-defined population. For incidence estimates, patients who were diagnosed between 2008 and 2019 were included. The background population formed 4.8 million patient-years at risk.

Result

Twenty-nine subjects were identified and included in our study. The overall SIR was 0.3/100,000/year, with a higher SIR for males compared to females (0.4/100,000/year and 0.2/100,000/year, respectively). The highest SIR was recorded in the 10–19 year age group. The estimated prevalence rate amounted to 5.27/100,000 people, with a lower prevalence rate for childhood-onset when compared to the adult-onset category (2.03/100,000 vs 3.24/100,000 people). The median longest tumour diameter was 31.0 mm (IQR 21–41), with a 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).

Conclusion

Through this population-based study, accurate and up-to-date prevalence and incidence rates for craniopharyngiomas are reported. These provide a clearer reflection of the true health burden of the disease.

Introduction

Craniopharyngiomas are rare but challenging tumours. Despite being a serious condition, population-based epidemiological data on craniopharyngiomas are limited in other studies (Bunin et al. 1998, Haupt et al. 2006, Nielsen et al. 2011, Zacharia et al. 2012). Epidemiological studies enable the researcher to adequately quantify the disease burden, disease severity stratification and utilisation of health care resources (Gruppetta et al. 2013). The aim of this study was to analyse variable factors related to craniopharyngiomas, focusing mainly on epidemiology and tumour characteristics by carrying out a population-based study.

Materials and methods

Study population and data collection

Malta is an archipelago of islands situated in the middle of the Mediterranean Sea with almost 500,000 inhabitants. The fact that Malta is an island with a high population density is advantageous when conducting epidemiological studies (Gruppetta et al. 2013). Since we only have one central National Health Service hospital to which all patients are referred, the whole Maltese population was taken as the background population for this study.

Permission for data collection was granted through our local institutional review board. To ensure that data collection was accurate and complete, we analysed multiple data sources. Subjects were identified from several hospital databases including the outpatient departments, the neurosurgical registry, the radiology department MRI registry and the neuro-endocrine paediatric and adult clinic records. Furthermore, all MRI scans (c. 100,000) that in any way visualised the parasellar area were reviewed. This multi-method data acquisition allowed for data triangulation. Patients with pituitary pathology other than craniopharyngioma on imaging were excluded from the study. The diagnosis of craniopharyngiomas was done using the most universally accepted radiological characteristics of craniopharyngiomas on MRI (Friedman et al. 2003), after discussion with different specialists including experienced neuro-radiologists, neurosurgeons, neuro-endocrinologists and tertiary centre referral specialists. This was confirmed by histology in those patients who were operated. Demographic data including gender, age at presentation, the onset of symptomatology and diagnosis were collected from hospital case notes and clinical records. The cohort was subdivided into patients with childhood-onset craniopharyngioma (presentation at < 20 years of age) and adult-onset (presentation at ≥ 20 years of age).

Data on the population at risk for each calendar year was obtained from the National Statistics Office. For prevalence estimates, patients who were alive and fulfilled the inclusion criteria on 30 June 2019 were included, while to estimate incidence rates, patients diagnosed between the beginning of 2008 and the end of 2018 were selected. During this study period, the annual number of inhabitants in the country varied from 413,609 to 493,579. The background population during this study period gave rise to 4.8 million patient-years at risk.

Tumour characteristics

We studied tumour size, localisation, consistency as well as any involvement of surrounding structures. Findings were obtained by analysing all available imaging techniques, followed by confirmation with neuroimaging and neurosurgery reports. Anteroposterior, craniocaudal and transverse dimensions were measured, enabling us to calculate the median longest tumour diameter as well as to assess any statistically significant differences between childhood-onset and adult-onset patients.

We categorised craniopharyngiomas into four groups based on tumour location and its relation to surrounding structures on MRI, mainly the sellar diaphragm, optic chiasm and mammillary bodies. As shown in Fig. 1, group 1 craniopharyngiomas were strictly intrasellar, group 2 tumours were supra-diaphragmatic and infra-chiasmatic, whilst group 3 tumours were supra-chiasmatic (referred to as group 3A or 3B depending on if they were located anteriorly or posteriorly to the mamillary bodies, respectively). Group 3B tumours were further examined for any evidence of brainstem involvement. This grading system was adapted from Flitsch et al. (2011) who suggested a relationship between pre-operative tumour grade and post-operative morbidity (Zoicas & Schöfl 2012, Müller 2014). We also analysed if there was evidence of optic chiasm involvement on imaging, and if present, it was further determined whether the compression was from the superior, inferior or posterior aspect, with the latter resulting in splaying of the chiasm. Tumour consistency was studied, and tumours were characterised as unicystic, multicystic, solid with cystic components or as a predominantly solid lesion.

Figure 1
Figure 1

Midline sagittal MR contrast enhanced T1 weighted images. (A) Normal view, (B) classification system of craniopharyngioma. Intra and suprasellar region is divided into three sections. Region 1 is limited intrasellarly, region 2 is beneath the optic chiasm and mamillary bodies, region 3 is above the optic chiasm and mamillary bodies. Region 3 is divided in two: anterior (3A) and posterior (3B) to the mammillary bodies. (C–P) Tumours in our study were classified as explained in (B). (C) Grade 2; (D–H) Grade 3A; (I–L) Grade 3B; (M–P) Grade 3B + brainstem compression.

Citation: Endocrine Oncology 1, 1; 10.1530/EO-21-0006

Statistical analysis

Prevalence figures together with the standardised incidence rates (SIR) were calculated, standardising with the World Health Organization 2001 standard population using the direct method (Ahmad et al. 2001). CIs (95% CI) were calculated using Wilson’s method. Results were expressed as mean when the data were normally distributed, together with s.d.s.d.), whilst median was used when data were not symmetrical. Non-parametric assessments were used. Statistical analyses were carried out using IBM SPSS® Statistics for Windows, Version 25.0 (IBM Corp.). Two-sided P-values below 0.05 were considered statistically significant.

Results

Epidemiology

Twenty-nine patients were identified as having craniopharyngioma among the Maltese population, with 26 patients being alive at the end of the study period (30 June 2019). Hence, the estimated prevalence rate amounted to 5.27/100,000 people. The prevalence rate for childhood onset craniopharyngiomas was lower than for the adult-onset category (2.03/100,000 vs 3.24/100,000 people). Females had a lower prevalence rate compared to males (3.31/100,000 and 7.15/100,000 people respectively). For incidence estimates, a total of 13 patients, who were diagnosed between beginning of 2008 and end of 2018, were included. The overall SIR was 0.3/100,000/year, with a higher SIR for the male compared to the female group (0.4/100,000/year and 0.2/100,000/year, respectively). The highest overall SIR was recorded in the 10–19 year age group (SIR of 1.01/100,000/year). From the adult-onset group, the highest SIR was recorded in the 50–59 year group (0.35/100,000/year) (Fig. 2). Further details regarding the prevalence and incidence of craniopharyngiomas are found in Table 1.

Figure 2
Figure 2

Standardised incidence rates according to 10-year age groups (2008–2018).

Citation: Endocrine Oncology 1, 1; 10.1530/EO-21-0006

Table 1

Prevalence and incidence of craniopharyngiomas.

All craniopharyngiomasChildhood-onset (<20 years)Adult-onset
Prevalence estimates (30 June 2019)
Number of craniopharyngiomas (n)26*1016
Overall prevalence (/100,000) (95% CI)5.27 (3.59–7.72)2.03 (1.10–3.73)3.24 (1.99–5.27)
Male (n)18711
Female (n)835
Prevalence male (/100,000) (95% CI)7.15 (4.52–11.3)2.78 (1.35–5.74)4.37 (2.44–7.82)
Prevalence female (/100,000) (95% CI)3.31 (1.68–6.53)1.24 (0.42–3.65)2.07 (0.88–4.84)
Median age at diagnosis (IQR)32 (14.5–45)13 (9.5–15.5)41.5 (33–51)
No. of patients undergoing surgery22913
No. of patients undergoing radiotherapy1569
No. of patients BMI 20–29 kg/m2 (%)10 (38.5)4 (40)6 (37.5)
No. of patients BMI 30–39 kg/m2 (%)13 (50)4 (40)9 (56.3)
No. of patients BMI > 40 kg/m2 (%)3 (11.5)2 (20)1 (6.25)
No. of patients with pituitary hormone deficiencies (%)25 (96.2)10 (100)15 (93.8)
Regrowth/recurrence (out of 23)° (%)6 (26.09)2 (8.70)4 (17.39)
Incidence estimates(2008–2019)
Number of patients (n)1349
Overall SIR (/100,000/year) (95% CI)0.3 (0.18–0.5)0.72 (0.35–1.48)0.19 (0.09–0.39)
Male (n)835
Female (n)514
SIR male (/100,000/year) (95% CI)0.4 (0.21–0.74)0.10 (0.44–2.38)0.22 (0.09–0.56)
SIR female (/100,000/year) (95% CI)0.2 (0.08–0.47)0.37 (0.1–1.46)0.15 (0.05–0.45)
Median longest diameter at diagnosis (IQR)32.1 (23.25–41.5)43 (42.5–47.25)27 (20.55–31.55)
Median age at diagnosis (IQR)41 (17–53)12 (9.75–14)51 (41–58)

*out of a cohort of 29 patients, 3 patients passed away by 30 June 2019, and hence could not be included in this study’s prevalence estimates.

°In 3 patients, regrowth could not be established (follow up scans or comparative imaging was unavailable).

‡All the consecutive patients (n = 13) diagnosed between beginning of 2008 and end of 2018 were included for SIR estimation.

Tumour characteristics

We described tumour characteristics in a sub-cohort of 14 consecutive patients for whom all pre- and post-operative imaging were available, allowing for assessment of variations during follow-up. Tumour characteristics varied significantly between patients with childhood-onset and those with adult-onset disease (Table 2). As seen in Fig. 1, the commonest tumour grading was grade 3A (5 patients, 35.7%) followed by grade 3B (4 patients, 28.6%). One patient had a grade 2 tumour (7.1%). No tumours were solely intrasellar (grade 1). The presence of hydrocephalus as well as hypothalamic and/or 3rd ventricle involvement was commoner in patients with childhood-onset disease (5 out of 5 patients, 100% vs 3 out of 9 patients, 33.3%). In 10 patients (71.4%), the optic chiasm was compressed from its posterior aspect, with resultant splaying of the chiasm. Most tumours were multicystic (6 out of 14 patients, 42.9%). Such tumours were present in significantly higher numbers in childhood- compared with adult-onset disease (5 patients, 83.3% vs 1 patient, 16.7%). For the cohort of 14 patients, the median longest tumour diameter was 31.0 mm (IQR 21–41). There was a statistically significant difference between the longest tumour diameter in patients with childhood-onset and those with adult-onset disease (P = 0.011) (Table 2).

Table 2

Tumour characteristics at presentation.

All craniopharyngiomas (%)Childhood-onset (%)Adult-onset (%)
Number of patients14*59
LocalisationConfined to sella0 (0.0)0 (0.0)0 (0.0)
Suprasellar7 (50.0)5 (100)2 (22.2)
Intra-/supra-sellar extension6 (42.9)5 (100)1 (11.1)
ConsistencySolid1 (7.1)0 (0.0)1 (11.1)
Cystic with solid components4 (28.6)2 (40.0)2 (22.2)
Multicystic6 (42.9)2 (40.0)4 (44.4)
Unicystic3 (21.4)2 (40.0)1 (11.1)
Hydrocephalus5 (35.7)5 (100)0 (0.0)
Hypothalamic/3rd ventricle involvement8 (57.1)5 (100)3 (33.3)
Brainstem compression4 (28.6)4 (80.0)0 (0.0)
Chiasmal compressionInfra-chiasmatic3 (21.4)0 (0.0)3 (33.3)
Supra-chiasmatic0 (0.0)0 (0.0)0 (0.0)
Posteriorly + splaying10 (71.4)4 (80.0)6 (66.7)
Nil1 (7.1)1 (20.0)0 (0.0)
Median longest diameter (mm) (IQR)31.0 (21–41)43.0 (42.5–47.25)24.0 (20–31)

*sub-cohort of 14 consecutive patients for whom all pre- and post-operative imaging were available.

Presentation, treatment and follow up

Visual disturbances and symptoms secondary to raised intracranial pressure (ICP) such as headaches, nausea and vomiting were the commonest presenting features, affecting 11 (37.9%) and 10 (34.5%) patients out of 29 patients respectively, followed by endocrine disturbances mainly resultant of hypopituitarism (presenting as menstrual irregularities, decreased libido and erectile dysfunction, lethargy and delayed puberty) as well as abnormal thirst and cognitive impairment, affecting a total of 8 patients (27.6%).

Twenty-four patients (out of the total cohort of 29 patients) required neurosurgical intervention (82.8%). Eleven patients (45.8%) underwent trans-frontal/transcranial surgery whilst 14 patients (58.3%) underwent a trans-sphenoidal approach. Seven patients with childhood-onset disease underwent trans-frontal/transcranial surgery compared to 4 patients with adult-onset disease. All patients had adamantinomatous craniopharyngioma on histology. Fifteen patients out of the total cohort underwent surgery through the trans-frontal/transcranial route whilst 11 patients underwent trans-sphenoidal surgery, with two patients requiring both surgical approaches due to residual tumour or recurrence post-operatively. Sixty percent had been administered radiotherapy post-operatively.

The median follow-up period since the time of diagnosis for the whole cohort of 29 patients was 13.0 years (IQR 5–25); 14.0 years (IQR 4.5–28) for childhood-onset craniopharyngioma and 9.5 years (IQR 5.21–21) for the adult-onset group, with no statistically significant difference in length of follow-up between both groups (P = 0.674). The commonest long-term sequalae were pituitary hormone deficiencies (defined as deficiency of one or more of ACTH, GH, TSH, LH, FSH, ADH), present in 27 patients (93.1%). The second most common long-term complication was obesity (BMI > 30 kg/m2) present in 18 patients (62.1%), followed by cognitive deficits (affecting 5 patients, 17.2%).

Seven patients (out of 23, 30.4%) had evidence of tumour regrowth or recurrence during follow-up, with 2 patients having childhood-onset disease and 4 individuals with adult-onset disease. Out of these patients, 3 (42.9%) had undergone sub-total resection through a trans-cranial approach followed by immediate post-operative radiotherapy and 4 individuals (57.1%) were given radiotherapy months after surgical removal in view of recurrence or regrowth. Kaplan–Meier estimates showed no statistically significant difference regarding regrowth and recurrence (Supplementary Fig. 1, see section on Supplementary materials given at the end of this article). Three patients passed away during follow-up. Survival rates were comparable for patients undergoing surgery or surgery with adjuvant radiotherapy.

Discussion

To date, very few epidemiological studies relating to craniopharyngiomas have been published and precise population-based data for craniopharyngiomas are limited (Bunin et al. 1998, Haupt et al. 2006, Nielsen et al. 2011, Zacharia et al. 2012). Such data are important to accurately estimate the disease burden and eventual need and utilisation of health care resources. In previous studies, the quoted tumour’s epidemiology was obtained from cancer registries or tertiary referral centre patients (Bunin et al. 1998, Nielsen et al. 2011). Since data obtained from registries and non-population-based studies are pre-collected, necessary information might be subject to under-reporting, inaccessible or miscategorized (Thygesen & Ersbøll 2014). It is therefore possible that the quoted incidence rates are lower than the actual figures. Calculating accurate incidence rates was possible in our study since we had precise and sensitive identification of patients during a well-defined period of time, as well as reliable information about the size and characteristics of the background population. Furthermore, data selection was controlled by the researcher and was not limited by variables recorded in the registers. To our knowledge, this is the first population-based study on craniopharyngiomas quoting both prevalence and incidence rates.

Our estimated prevalence rate amounts to 5.27/100,000 people. To our knowledge, this is a novel finding, since prevalence rates have not been reported in other studies. As shown in Table 3, our SIR of 0.3/100,000/year is higher (approximately double) than rates quoted in the literature. Nielsen et al. (2011) carried out a study through which 189 patients with probable craniopharyngioma were identified from different patient registries in Denmark between 1985 and 2004. The standardised incidence rate for craniopharyngiomas during the 20-year study period was 0.19/100,000/year (95% CI 0.16–0.21), with similar rates for males and females (0.17 vs 0.15). They also estimated the worldwide incidence rate by searching literature that contained the terms ‘craniopharyngioma’, ‘incidence’ and ‘epidemiology’, identifying 1232 patients diagnosed with craniopharyngioma between 1935 and 1999. Absolute incidence rates varied between 0.09 and 0.57/100,000/year with no geographical pattern and the weighted summary incidence rate was of 0.13/100,000/year (95% CI 0.12–0.15) for the whole population. The CBTRUS report for 2005–2009 obtained incidence rates from 49 cancer registries (44 NPCR (National Programme of Cancer Registries), and 5 SEER (surveillance, epidemiology and end results), including both malignant and non-malignant brain and CNS tumours. From this registry-based study, Dolecek et al. (2012) identified 2680 patients with craniopharyngioma, with an annual incidence rate of 0.18/100,000/year (95% CI 0.18–0.19). Zacharia et al. (2012) quote an incidence rate of 0.17 cases per 100,000 person-years from a SEER database over a period of 5 years (2004–2009) which included 644 patients diagnosed with craniopharyngioma. As was noted in the CBTRUS study (Dolecek et al. 2012), where only 28.7% (766 patients) from a total cohort of 2680 had childhood-onset craniopharyngioma, in our cohort, adult-onset disease was more frequently prevalent (16 out of 26 patients, 61.5%). In our study, peak incidence rates were observed in the 2nd and 5th decade, similarly to what is quoted in the literature. Dolecek et al. (2012) quote peaks in incidence rates between 65–74 years (0.25/100,000/year, 95% CI 0.22–0.28) for the adult population and 5–9 age group (0.26/100,000/year, 95% CI 0.23–0.29). Nielsen et al. (2011) quote a peak at ages 5–9 and 40–44 years, and Bunin et al. (1998) noted a bimodal age distribution with a peak incidence in children aged 5–14 years and adults aged 65–74 years. Similar to what was observed in the study by Warmuth-Metz et al. (2004) in our population, males were more commonly affected. However, in contrast to our rates, in various other studies, there was no statistically significant difference in incidence rates between genders (De Vries et al. 2003, Haupt et al. 2006, Jane & Laws 2006, Dolecek et al. 2012, Cohen et al. 2013, Karavitaki 2014, Wijnen et al. 2017, Andereggen et al. 2018).

Table 3

Standardised incidence rates for present study compared to other literature.

Present study, 2021Dolecek et al. 2012Nielsen et al. 2011Bunin et al. 1998
Overall SIR (/100,000/year) (95% CI)0.30 (0.18–0.50)0.18 (0.18–0.19)0.16 (0.13–0.18)0.13
SIR male (/100,000/year) (95% CI)0.40 (0.21–0.74)0.18 (0.17–0.19)0.17 (0.13–0.20)0.13
SIR female (/100,000/year) (95% CI)0.20 (0.08–0.47)0.18 (0.17–0.19)0.15 (0.12–0.18)0.12

In our local population, multicystic tumours were present in significantly higher numbers in childhood- compared with adult-onset disease, as was also observed in a Bonferroni-corrected post hoc analysis carried out by Wijnen et al. (2017). In agreement with our results, these researchers concluded that multi-cystic craniopharyngiomas were more commonly seen in patients with childhood-onset when compared to adult-onset disease patients (45% vs 21%, P = 0.02). Locally, similarly to what was observed in other studies, hydrocephalus and 3rd ventricle involvement were more frequently observed in patients with childhood-onset disease. In our study, 62.5% of patients with childhood-onset (vs 35.5% of adult-onset) had hydrocephalus, compared to 44% of patients in the study carried out by Karavitaki et al. (2014) and 40% of patients with childhood-onset disease in the study by Wijnen et al. (2017).

Craniopharyngiomas can arise anywhere along the craniopharyngeal canal but are most commonly seen in the sellar and para-sellar areas (Karavitaki et al. 2006). Both locally and in other studies, the majority were intrasellar tumours with suprasellar extension (Table 2) (Petito et al. 1976, Hoffman et al. 1992, Karavitaki et al. 2006). No patients from our cohort had tumours restricted to the intrasellar region. In concordance, in the literature, this was the least common location (5–6%) (Petito et al. 1976, Karavitaki et al. 2006). By assessing the epidemiology and tumour characteristics of a cohort of 14 patients who were consecutively diagnosed over a 10-year period, we think we obtained a clearer overview of the variations in craniopharyngiomas in a definite population.

Both in our cohort and in other studies, the presentation was usually dominated by non-specific symptoms related to raised intracranial pressure such as headache and visual disturbances and pituitary hormone deficiencies (De Vries et al. 2003, Halac & Zimmerman 2005, Karavitaki et al. 2005, Wijnen et al. 2017, Andereggen et al. 2018). Overall survival rates in our cohort were comparable to other studies. Wijnen et al. (2017) quote an overall survival rate of 85%, and other recent studies report 10-year survival rates between 40 and 95% (Sherlock et al. 2010, Lee et al. 2014, Hoffmann et al. 2015, Olsson et al. 2015, Hoffmann et al. 2016, Pan et al. 2016). In our cohort, the commonest surgical modality was the trans-sphenoidal approach, targeted at tumour resection whilst preserving hypothalamic function.

Limitations to this study include the retrospective nature of data gathering with certain details being unavailable in some patients. However, due to the scarcity of the condition and the necessity for lengthy follow-up, prospective observational studies would be very challenging and time-consuming. Another limitation was the small sample size, which we tried to mitigate by including all patients diagnosed with craniopharyngioma from a well-defined population. Since there were various health care professionals taking care of these patients, this could result in possible differences when interpreting imaging and investigations. To minimize this, radiological investigations as well as tumour characteristics were all objectively assessed by a single specialist.

Conclusion

This study provides up-to-date and in-depth epidemiological data of craniopharyngiomas in a population. Being the first population-based study in this respect, the resultant rates were about twice the previously quoted figures. We think that these figures and tumour characteristic analysis better represent the health burden of craniopharyngiomas.

Supplementary materials

This is linked to the online version of the paper at https://doi.org/10.1530/EO-21-0006.

Declaration of interest

The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.

Funding

This work did not receive any specific grant from any funding agency in the public, commercial, or not-for-profit sector.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Acknowledgements

The authors would like to thank Professor J Vassallo, Dr MJ Cachia and Dr M Giordano Imbroll, for their assistance and support in this study and acknowledge all the medical staff and healthcare professionals at Mater Dei Hospital who gave invaluable input for this study to be completed.

References

  • Ahmad OB, Boschi-Pinto C, Lopez AD, Murray CJ, Lozano R & Inoue M 2001 Age Standardization of Rates: A New WHO Standard, vol 9. Geneva, Switzerland: World Health Organization. (available at: https://www.who.int/healthinfo/paper31.pdf)

    • Search Google Scholar
    • Export Citation
  • Andereggen L, Hess B, Andres R, El-Koussy M, Mariani L, Raabe A, Seiler RW & Christ E 2018 A ten-year follow-up study of treatment outcome of craniopharyngiomas. Swiss Medical Weekly 148 w14521. (https://doi.org/10.4414/smw.2018.14521)

    • Search Google Scholar
    • Export Citation
  • Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F & Bruner JM 1998 The descriptive epidemiology of craniopharyngioma. Journal of Neurosurgery 89 547551. (https://doi.org/10.3171/jns.1998.89.4.0547)

    • Search Google Scholar
    • Export Citation
  • Cohen M, Syme C, McCrindle BW & Hamilton J 2013 Autonomic nervous system balance in children and adolescents with craniopharyngioma and hypothalamic obesity. European Journal of Endocrinology 168 845852. (https://doi.org/10.1530/EJE-12-1082)

    • Search Google Scholar
    • Export Citation
  • De Vries L, Lazar L & Phillip M 2003 Craniopharyngioma: presentation and endocrine sequelae in 36 children. Journal of Pediatric Endocrinology and Metabolism 16 703710. (https://doi.org/10.1515/jpem.2003.16.5.703)

    • Search Google Scholar
    • Export Citation
  • Dolecek TA, Propp JM, Stroup NE & Kruchko C 2012 CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2005–2009. Neuro-Oncology 14(Supplement 5) v1v49. (https://doi.org/10.1093/neuonc/nos218)

    • Search Google Scholar
    • Export Citation
  • Flitsch J, Müller HL & Burkhardt T 2011 Surgical strategies in childhood craniopharyngioma. Frontiers in Endocrinology 2 96. (https://doi.org/10.3389/fendo.2011.00096)

    • Search Google Scholar
    • Export Citation
  • Friedman MA, Meyers CA & Sawaya R 2003 Neuropsychological effects of third ventricle tumor surgery. Neurosurgery 52 7918; discussion 798. (https://doi.org/10.1227/01.neu.0000053367.94965.6b)

    • Search Google Scholar
    • Export Citation
  • Gruppetta M, Mercieca C & Vassallo J 2013 Prevalence and incidence of pituitary adenomas: a population based study in Malta. Pituitary 16 545553. (https://doi.org/10.1007/s11102-012-0454-0)

    • Search Google Scholar
    • Export Citation
  • Halac I & Zimmerman D 2005 Endocrine manifestations of craniopharyngioma. Child’s Nervous System 21 640648. (https://doi.org/10.1007/s00381-005-1246-x)

    • Search Google Scholar
    • Export Citation
  • Haupt R, Magnani C, Pavanello M, Caruso S, Dama E & Garrè ML 2006 Epidemiological aspects of craniopharyngioma. Journal of Pediatric Endocrinology and Metabolism 19 (Supplement 1) 289293.

    • Search Google Scholar
    • Export Citation
  • Hoffman HJ, De Silva M, Humphreys RP, Drake JM, Smith ML & Blaser SI 1992 Aggressive surgical management of craniopharyngiomas in children. Journal of Neurosurgery 76 4752. (https://doi.org/10.3171/jns.1992.76.1.0047)

    • Search Google Scholar
    • Export Citation
  • Hoffmann A, Boekhoff S, Gebhardt U, Sterkenburg AS, Daubenbüchel AM, Eveslage M & Mueller HL 2015 History before diagnosis in childhood craniopharyngioma: associations with initial presentation and long-term prognosis. European Journal of Endocrinology 173 853862. (https://doi.org/10.1530/EJE-15-0709)

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  • Hoffmann A, Warmuth-Metz M, Lohle K, Reichel J, Daubenbüchel AM, Sterkenburg AS & Müller HL 2016 Fusiform dilatation of the internal carotid artery in childhood-onset craniopharyngioma: multicenter study on incidence and long-term outcome. Pituitary 19 422428. (https://doi.org/10.1007/s11102-016-0722-5)

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  • Jane JA & Laws ER 2006 Craniopharyngioma. Pituitary 9 323326. (https://doi.org/10.1007/s11102-006-0413-8)

  • Karavitaki N 2014 Management of craniopharyngiomas. Journal of Endocrinological Investigation 37 219228. (https://doi.org/10.1007/s40618-013-0050-9)

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  • Karavitaki N, Brufani C, Warner JT, Adams CBT, Richards P, Ansorge O, Shine B, Turner HE & Wass JAH 2005 Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clinical Endocrinology 62 397409. (https://doi.org/10.1111/j.1365-2265.2005.02231.x)

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  • Karavitaki N, Cudlip S, Adams CB & Wass JA 2006 Craniopharyngiomas. Endocrine Reviews 27 371397. (https://doi.org/10.1210/er.2006-0002)

  • Lee CC, Yang HC, Chen CJ, Hung YC, Wu HM, Shiau CY, Guo WY, Pan DH, Chung WY & Liu KD 2014 Gamma Knife surgery for craniopharyngioma: report on a 20-year experience. Journal of Neurosurgery 121 (Supplement) 167178. (https://doi.org/10.3171/2014.8.GKS141411)

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    • Export Citation
  • Müller HL 2014 Craniopharyngioma. Endocrine Reviews 35 513543. (https://doi.org/10.1210/er.2013-1115)

  • Nielsen EH, Feldt-Rasmussen U, Poulsgaard L, Kristensen LO, Astrup J, Jørgensen JO, Bjerre P, Andersen M, Andersen C, Jørgensen J, et al. 2011 Incidence of craniopharyngioma in Denmark (n=189) and estimated world incidence of craniopharyngioma in children and adults. Journal of Neuro-Oncology 104 755763. (https://doi.org/10.1007/s11060-011-0540-6)

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  • Olsson DS, Andersson E, Bryngelsson IL, Nilsson AG & Johannsson G 2015 Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood onset: a population-based study in Sweden. Journal of Clinical Endocrinology and Metabolism 100 467474. (https://doi.org/10.1210/jc.2014-3525)

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  • Pan J, Qi S, Liu Y, Lu Y, Peng J, Zhang X, Xu Y, Huang GL & Fan J 2016 Growth patterns of craniopharyngiomas: clinical analysis of 226 patients. Journal of Neurosurgery: Pediatrics 17 418433. (https://doi.org/10.3171/2015.7.PEDS14449)

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  • Petito CK, Degirolami U & Earle KM 1976 Craniopharyngiomas. A clinical and pathological review. Cancer 37 19441952 (https://doi.org/10.1002/1097-0142(197604)37:4<1944::aid-cncr2820370446>3.0.co;2-#)

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  • Sherlock M, Ayuk J, Tomlinson JW, Toogood AA, Aragon-Alonso A, Sheppard MC, Bates AS & Stewart PM 2010 Mortality in patients with pituitary disease. Endocrine Reviews 31 301342. (https://doi.org/10.1210/er.2009-0033)

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    • Export Citation
  • Thygesen LC & Ersbøll AK 2014 When the entire population is the sample: strengths and limitations in register-based epidemiology. European Journal of Epidemiology 29 551558. (https://doi.org/10.1007/s10654-013-9873-0)

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    • Export Citation
  • Warmuth-Metz M, Gnekow AK, Müller H & Solymosi L 2004 Differential diagnosis of suprasellar tumors in children. Klinische Padiatrie 216 323330. (https://doi.org/10.1055/s-2004-832358)

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  • Wijnen M, van den Heuvel-Eibrink MM, Janssen JAMJL, Catsman-Berrevoets CE, Michiels EMC, Ivan Veelen-Vincent MC, Dallenga AHG, Van den Berge JH, Van Rij CM, Van der Lely AJ, et al. 2017 Very long-term sequelae of craniopharyngioma. European Journal of Endocrinology 176 755767. (doi:10.1530/EJE-17-0044

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  • Zacharia BE, Bruce SS, Goldstein H, Malone HR, Neugut AI & Bruce JN 2012 Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program. Neuro-Oncology 14 10701078. (https://doi.org/10.1093/neuonc/nos142)

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    • Export Citation
  • Zoicas F & Schöfl C 2012 Craniopharyngioma in adults. Frontiers in Endocrinology 3 46. (https://doi.org/10.3389/fendo.2012.00046)

 

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    Midline sagittal MR contrast enhanced T1 weighted images. (A) Normal view, (B) classification system of craniopharyngioma. Intra and suprasellar region is divided into three sections. Region 1 is limited intrasellarly, region 2 is beneath the optic chiasm and mamillary bodies, region 3 is above the optic chiasm and mamillary bodies. Region 3 is divided in two: anterior (3A) and posterior (3B) to the mammillary bodies. (C–P) Tumours in our study were classified as explained in (B). (C) Grade 2; (D–H) Grade 3A; (I–L) Grade 3B; (M–P) Grade 3B + brainstem compression.

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    Standardised incidence rates according to 10-year age groups (2008–2018).

  • Ahmad OB, Boschi-Pinto C, Lopez AD, Murray CJ, Lozano R & Inoue M 2001 Age Standardization of Rates: A New WHO Standard, vol 9. Geneva, Switzerland: World Health Organization. (available at: https://www.who.int/healthinfo/paper31.pdf)

    • Search Google Scholar
    • Export Citation
  • Andereggen L, Hess B, Andres R, El-Koussy M, Mariani L, Raabe A, Seiler RW & Christ E 2018 A ten-year follow-up study of treatment outcome of craniopharyngiomas. Swiss Medical Weekly 148 w14521. (https://doi.org/10.4414/smw.2018.14521)

    • Search Google Scholar
    • Export Citation
  • Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F & Bruner JM 1998 The descriptive epidemiology of craniopharyngioma. Journal of Neurosurgery 89 547551. (https://doi.org/10.3171/jns.1998.89.4.0547)

    • Search Google Scholar
    • Export Citation
  • Cohen M, Syme C, McCrindle BW & Hamilton J 2013 Autonomic nervous system balance in children and adolescents with craniopharyngioma and hypothalamic obesity. European Journal of Endocrinology 168 845852. (https://doi.org/10.1530/EJE-12-1082)

    • Search Google Scholar
    • Export Citation
  • De Vries L, Lazar L & Phillip M 2003 Craniopharyngioma: presentation and endocrine sequelae in 36 children. Journal of Pediatric Endocrinology and Metabolism 16 703710. (https://doi.org/10.1515/jpem.2003.16.5.703)

    • Search Google Scholar
    • Export Citation
  • Dolecek TA, Propp JM, Stroup NE & Kruchko C 2012 CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2005–2009. Neuro-Oncology 14(Supplement 5) v1v49. (https://doi.org/10.1093/neuonc/nos218)

    • Search Google Scholar
    • Export Citation
  • Flitsch J, Müller HL & Burkhardt T 2011 Surgical strategies in childhood craniopharyngioma. Frontiers in Endocrinology 2 96. (https://doi.org/10.3389/fendo.2011.00096)

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    • Export Citation
  • Friedman MA, Meyers CA & Sawaya R 2003 Neuropsychological effects of third ventricle tumor surgery. Neurosurgery 52 7918; discussion 798. (https://doi.org/10.1227/01.neu.0000053367.94965.6b)

    • Search Google Scholar
    • Export Citation
  • Gruppetta M, Mercieca C & Vassallo J 2013 Prevalence and incidence of pituitary adenomas: a population based study in Malta. Pituitary 16 545553. (https://doi.org/10.1007/s11102-012-0454-0)

    • Search Google Scholar
    • Export Citation
  • Halac I & Zimmerman D 2005 Endocrine manifestations of craniopharyngioma. Child’s Nervous System 21 640648. (https://doi.org/10.1007/s00381-005-1246-x)

    • Search Google Scholar
    • Export Citation
  • Haupt R, Magnani C, Pavanello M, Caruso S, Dama E & Garrè ML 2006 Epidemiological aspects of craniopharyngioma. Journal of Pediatric Endocrinology and Metabolism 19 (Supplement 1) 289293.

    • Search Google Scholar
    • Export Citation
  • Hoffman HJ, De Silva M, Humphreys RP, Drake JM, Smith ML & Blaser SI 1992 Aggressive surgical management of craniopharyngiomas in children. Journal of Neurosurgery 76 4752. (https://doi.org/10.3171/jns.1992.76.1.0047)

    • Search Google Scholar
    • Export Citation
  • Hoffmann A, Boekhoff S, Gebhardt U, Sterkenburg AS, Daubenbüchel AM, Eveslage M & Mueller HL 2015 History before diagnosis in childhood craniopharyngioma: associations with initial presentation and long-term prognosis. European Journal of Endocrinology 173 853862. (https://doi.org/10.1530/EJE-15-0709)

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  • Hoffmann A, Warmuth-Metz M, Lohle K, Reichel J, Daubenbüchel AM, Sterkenburg AS & Müller HL 2016 Fusiform dilatation of the internal carotid artery in childhood-onset craniopharyngioma: multicenter study on incidence and long-term outcome. Pituitary 19 422428. (https://doi.org/10.1007/s11102-016-0722-5)

    • Search Google Scholar
    • Export Citation
  • Jane JA & Laws ER 2006 Craniopharyngioma. Pituitary 9 323326. (https://doi.org/10.1007/s11102-006-0413-8)

  • Karavitaki N 2014 Management of craniopharyngiomas. Journal of Endocrinological Investigation 37 219228. (https://doi.org/10.1007/s40618-013-0050-9)

    • Search Google Scholar
    • Export Citation
  • Karavitaki N, Brufani C, Warner JT, Adams CBT, Richards P, Ansorge O, Shine B, Turner HE & Wass JAH 2005 Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clinical Endocrinology 62 397409. (https://doi.org/10.1111/j.1365-2265.2005.02231.x)

    • Search Google Scholar
    • Export Citation
  • Karavitaki N, Cudlip S, Adams CB & Wass JA 2006 Craniopharyngiomas. Endocrine Reviews 27 371397. (https://doi.org/10.1210/er.2006-0002)

  • Lee CC, Yang HC, Chen CJ, Hung YC, Wu HM, Shiau CY, Guo WY, Pan DH, Chung WY & Liu KD 2014 Gamma Knife surgery for craniopharyngioma: report on a 20-year experience. Journal of Neurosurgery 121 (Supplement) 167178. (https://doi.org/10.3171/2014.8.GKS141411)

    • Search Google Scholar
    • Export Citation
  • Müller HL 2014 Craniopharyngioma. Endocrine Reviews 35 513543. (https://doi.org/10.1210/er.2013-1115)

  • Nielsen EH, Feldt-Rasmussen U, Poulsgaard L, Kristensen LO, Astrup J, Jørgensen JO, Bjerre P, Andersen M, Andersen C, Jørgensen J, et al. 2011 Incidence of craniopharyngioma in Denmark (n=189) and estimated world incidence of craniopharyngioma in children and adults. Journal of Neuro-Oncology 104 755763. (https://doi.org/10.1007/s11060-011-0540-6)

    • Search Google Scholar
    • Export Citation
  • Olsson DS, Andersson E, Bryngelsson IL, Nilsson AG & Johannsson G 2015 Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood onset: a population-based study in Sweden. Journal of Clinical Endocrinology and Metabolism 100 467474. (https://doi.org/10.1210/jc.2014-3525)

    • Search Google Scholar
    • Export Citation
  • Pan J, Qi S, Liu Y, Lu Y, Peng J, Zhang X, Xu Y, Huang GL & Fan J 2016 Growth patterns of craniopharyngiomas: clinical analysis of 226 patients. Journal of Neurosurgery: Pediatrics 17 418433. (https://doi.org/10.3171/2015.7.PEDS14449)

    • Search Google Scholar
    • Export Citation
  • Petito CK, Degirolami U & Earle KM 1976 Craniopharyngiomas. A clinical and pathological review. Cancer 37 19441952 (https://doi.org/10.1002/1097-0142(197604)37:4<1944::aid-cncr2820370446>3.0.co;2-#)

    • Search Google Scholar
    • Export Citation
  • Sherlock M, Ayuk J, Tomlinson JW, Toogood AA, Aragon-Alonso A, Sheppard MC, Bates AS & Stewart PM 2010 Mortality in patients with pituitary disease. Endocrine Reviews 31 301342. (https://doi.org/10.1210/er.2009-0033)

    • Search Google Scholar
    • Export Citation
  • Thygesen LC & Ersbøll AK 2014 When the entire population is the sample: strengths and limitations in register-based epidemiology. European Journal of Epidemiology 29 551558. (https://doi.org/10.1007/s10654-013-9873-0)

    • Search Google Scholar
    • Export Citation
  • Warmuth-Metz M, Gnekow AK, Müller H & Solymosi L 2004 Differential diagnosis of suprasellar tumors in children. Klinische Padiatrie 216 323330. (https://doi.org/10.1055/s-2004-832358)

    • Search Google Scholar
    • Export Citation
  • Wijnen M, van den Heuvel-Eibrink MM, Janssen JAMJL, Catsman-Berrevoets CE, Michiels EMC, Ivan Veelen-Vincent MC, Dallenga AHG, Van den Berge JH, Van Rij CM, Van der Lely AJ, et al. 2017 Very long-term sequelae of craniopharyngioma. European Journal of Endocrinology 176 755767. (doi:10.1530/EJE-17-0044

    • Search Google Scholar
    • Export Citation
  • Zacharia BE, Bruce SS, Goldstein H, Malone HR, Neugut AI & Bruce JN 2012 Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program. Neuro-Oncology 14 10701078. (https://doi.org/10.1093/neuonc/nos142)

    • Search Google Scholar
    • Export Citation
  • Zoicas F & Schöfl C 2012 Craniopharyngioma in adults. Frontiers in Endocrinology 3 46. (https://doi.org/10.3389/fendo.2012.00046)