Incidence of craniopharyngioma in Denmark (n = 189) and estimated world incidence of craniopharyngioma in children and adults

Abstract

We studied the incidence of craniopharyngioma in Denmark during the period 1985-2004 and estimated worldwide incidence rates (IR) of craniopharyngioma based on a literature review. Craniopharyngioma patients diagnosed during the period 1985-2004 were identified from the Danish National Patient Registry, the Danish Cancer Registry and regional registries. Medical records were reviewed. Danish population data were obtained from Statistics Denmark. European and World population data were obtained from EU and WHO homepages. Prior studies providing data on craniopharyngioma IRs were identified via PubMed and, if appropriate, were included in a weighted analysis estimating overall and children's IRs of craniopharyngioma. IRs are given as new cases per million per year. We identified 189 patients with new verified (162) or probable craniopharyngioma. The overall WHO World-standardised incidence rate was 1.86 (1.60-2.14) for all ages and 2.14 (1.53-2.92) for children (age <15 years). Peak incidence rates were observed in age groups 5-9 and 40-44 years. Fifteen prior studies (including 1,232 craniopharyngioma cases) were identified. Seven and 11 studies, respectively, were eligible for weighted all-ages and childhood population IR analyses, yielding summary IRs of 1.34 (1.24-1.46) (all ages) and 1.44 (1.33-1.56) (children). We have provided a detailed survey of the incidence of craniopharyngioma in Denmark during a recent 20-year period. Overall IR of craniopharyngioma in Denmark was 1.86 (1.60-2.14) as compared to 2.14 (1.53-2.92) among children. Weighted estimates of craniopharyngioma world IRs were 1.34 (1.24-1.46) in all ages and 1.44 (1.33-1.56) among children.

Molecular oncogenesis of craniopharyngioma: current and future strategies for the development of targeted therapies

“…10,49,53,69 These tumors of the sellar region present in a bimodal distribution, with one peak occurring in the pediatric population between 5-14 years of age, and another peak in adults during the 5th decade of life. 64 Though classified by the World Health Organization as Grade I tumors, craniopharyngiomas can cause significant morbidity by intimate involvement and mass effect on surrounding critical structures, including the hypothalamus, pituitary gland, and optic chiasm. There have only been rare reports of de novo malignancy or malignant transformation, often as a sequela of radiation therapy.…”

60 YEARS OF NEUROENDOCRINOLOGY: Biology of human craniopharyngioma: lessons from mouse models

“…Although the overall incidence is low (1.60-2.14 new cases/million per year), they are the most common tumour of this area in children under 15 years of age (1.53-2.92) (Nielsen et al 2011, Muller 2014a). They are designated as grade I tumours according to the World Health Organization (WHO) classification (Louis et al 2014).…”

“…Adamantinomatous CPs (ACPs) represent the most common non-neuroepithelial intracranial lesions in children, and they contribute to 1.5-11.6% of all paediatric CNS tumours (Bunin et al 1998). There is a well-described bimodal age distribution in incidence, with peaks occurring between the ages of 5 and 14 (childhood-onset ACP) and between ages 65 and 74 (adult-onset ACP) (Bunin et al 1998, Nielsen et al 2011. ACPs are the archetypal suprasellar tumour for causing neuroendocrine disruption and dysfunction, not just from tumour pressure and invasive effects but also from treatments targeted to this area.…”

“…The papillary variant (papillary craniopharyngioma, PCP) is very rare and appears almost exclusively in adults, where peak incidence rates were observed in the 40-44-year-old age group (Nielsen et al 2011). Recently, overactivating mutations in BRAF p.Val600Glu have been described in association with PCP, which suggests that these mutations may drive tumorigenesis (Brastianos et al 2014).…”

Comparison Study of Clinical Presentation and Surgical Outcome between Children and Adults with Craniopharyngioma: A 22-Year Single-Center Experience in Southern Taiwan

“…For adult group, the Danish study found that the peak incidence occurred in individuals aged 40-44 years [12]. Although craniopharyngiomas could occur in both paediatric and adult groups, the clinical presentation and surgical outcome may differ.…”