Abstract
Craniopharyngiomas are rare malformational tumours of low histological malignancy arising along the craniopharyngeal duct. The two histological subtypes, adamantinomatous craniopharyngioma (ACP) and papillary craniopharyngioma (PCP), differ in genesis and age distribution. ACPs are diagnosed with a bimodal peak of incidence (5–15 years and 45–60 years), whereas PCPs are restricted to adults mainly in the fifth and sixth decades of life. ACPs are driven by somatic mutations in CTNNB1 (encoding β-catenin) that affect β-catenin stability and are predominantly cystic in appearance. PCPs frequently harbour somatic BRAFV600E mutations and are typically solid tumours. Clinical manifestations due to increased intracranial pressure, visual impairment and endocrine deficiencies should prompt imaging investigations, preferentially MRI. Treatment comprises neurosurgery and radiotherapy; intracystic chemotherapy is used in monocystic ACP. Although long-term survival is high, quality of life and neuropsychological function are frequently impaired due to the close anatomical proximity to the optic chiasm, hypothalamus and pituitary gland. Indeed, hypothalamic involvement and treatment-related hypothalamic lesions frequently result in hypothalamic obesity, physical fatigue and psychosocial deficits. Given the rarity of these tumours, efforts to optimize infrastructure and international collaboration should be research priorities.
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References
Muller, H. L., Merchant, T. E., Puget, S. & Martinez-Barbera, J. P. New outlook on the diagnosis, treatment and follow-up of childhood-onset craniopharyngioma. Nat. Rev. Endocrinol. 13, 299–312 (2017). A comprehensive review on the current state of the art in diagnostics, treatment and follow-up of CP.
Muller, H. L. Management of endocrine disease: childhood-onset craniopharyngioma: state of the art of care in 2018. Eur. J. Endocrinol. 180, R159–R174 (2019).
Bogusz, A. & Muller, H. L. Childhood-onset craniopharyngioma: latest insights into pathology, diagnostics, treatment, and follow-up. Expert Rev. Neurother. 18, 793–806 (2018).
Pascual, J. M. et al. Craniopharyngioma classification. J. Neurosurg. 109, 1180–1182 (2008).
Gabel, B. C. et al. Unusual and rare locations for craniopharyngiomas: clinical significance and review of the literature. World Neurosurg. 98, 381–387 (2017).
Hoffmann, A., Brentrup, A. & Muller, H. L. First report on spinal metastasis in childhood-onset craniopharyngioma. J. Neurooncol. 129, 193–194 (2016).
Buslei, R. et al. in WHO Classification of Tumours of the Central Nervous System (eds Louis, D. N. et al.) 324–328 (International Agency for Research on Cancer, 2016).
Apps, J. R. et al. Tumour compartment transcriptomics demonstrates the activation of inflammatory and odontogenic programmes in human adamantinomatous craniopharyngioma and identifies the MAPK/ERK pathway as a novel therapeutic target. Acta Neuropathol. 135, 757–777 (2018).
Sekine, S. et al. Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am. J. Pathol. 161, 1997–2001 (2002).
Buslei, R. et al. Common mutations of β-catenin in adamantinomatous craniopharyngiomas but not in other tumours originating from the sellar region. Acta Neuropathol. 109, 589–597 (2005).
Brastianos, P. K. et al. Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas. Nat. Genet. 46, 161–165 (2014). The first report on BRAF mutations as a typical molecular finding in PCP.
Goschzik, T. et al. Genomic alterations of adamantinomatous and papillary craniopharyngioma. J. Neuropathol. Exp. Neurol. 76, 126–134 (2017).
Haston, S. et al. MAPK pathway control of stem cell proliferation and differentiation in the embryonic pituitary provides insights into the pathogenesis of papillary craniopharyngioma. Development 144, 2141–2152 (2017).
Johnson, L. N., Hepler, R. S., Yee, R. D., Frazee, J. G. & Simons, K. B. Magnetic resonance imaging of craniopharyngioma. Am. J. Ophthalmol. 102, 242–244 (1986).
Warmuth-Metz, M. Imaging and Diagnosis in Pediatric Brain Tumor Studies (Springer Nature, 2017).
Forbes, J. A. et al. Endonasal endoscopic transsphenoidal resection of intrinsic third ventricular craniopharyngioma: surgical results. J. Neurosurg. 131, 1152–1162 (2019).
Hidalgo, E. T. et al. Quality of life, hypothalamic obesity, and sexual function in adulthood two decades after primary gross-total resection for childhood craniopharyngioma. Childs Nerv. Syst. https://doi.org/10.1007/s00381-019-04161-9 (2019).
Apra, C., Enachescu, C., Lapras, V., Raverot, G. & Jouanneau, E. Is gross total resection reasonable in adults with craniopharyngiomas with hypothalamic involvement? World Neurosurg. 129, e803–e811 (2019).
Madsen, P. J. et al. Endoscopic endonasal resection versus open surgery for pediatric craniopharyngioma: comparison of outcomes and complications. J. Neurosurg. Pediatr. 24, 236–245 (2019).
d’Avella, E. et al. The endoscopic endonasal approach for pediatric craniopharyngiomas: the key lessons learned. Childs Nerv. Syst. https://doi.org/10.1007/s00381-019-04168-2 (2019).
Ajithkumar, T. et al. Proton therapy for craniopharyngioma — an early report from a single European centre. Clin. Oncol. 30, 307–316 (2018).
Ordonez-Rubiano, E. G. et al. Preserve or sacrifice the stalk? Endocrinological outcomes, extent of resection, and recurrence rates following endoscopic endonasal resection of craniopharyngiomas. J. Neurosurg. 131, 1163–1171 (2019).
Bunin, G. R. et al. The descriptive epidemiology of craniopharyngioma. J. Neurosurg. 89, 547–551 (1998).
Olsson, D. S., Andersson, E., Bryngelsson, I. L., Nilsson, A. G. & Johannsson, G. Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood onset: a population-based study in Sweden. J. Clin. Endocrinol. Metab. 100, 467–474 (2015).
Zacharia, B. E. et al. Incidence, treatment and survival of patients with craniopharyngioma in the Surveillance, Epidemiology and End Results program. Neuro Oncol. 14, 1070–1078 (2012).
Makino, K., Nakamura, H., Yano, S., Kuratsu, J. & Kumamoto Brain Tumor Group. Population-based epidemiological study of primary intracranial tumors in childhood. Childs Nerv. Syst. 26, 1029–1034 (2010).
Rosemberg, S. & Fujiwara, D. Epidemiology of pediatric tumors of the nervous system according to the WHO 2000 classification: a report of 1,195 cases from a single institution. Childs Nerv. Syst. 21, 940–944 (2005).
Muller, H. L. Craniopharyngioma. Handb. Clin. Neurol. 124, 235–253 (2014).
Nielsen, E. H. et al. Incidence of craniopharyngioma in Denmark (n = 189) and estimated world incidence of craniopharyngioma in children and adults. J. Neurooncol. 104, 755–763 (2011).
Muller-Scholden, J. et al. Radical surgery in a neonate with craniopharyngioma. Report of a case. Pediatr. Neurosurg. 33, 265–269 (2000).
Larkin, S. J. & Ansorge, O. Pathology and pathogenesis of craniopharyngiomas. Pituitary 16, 9–17 (2013).
Bailey, W., Freidenberg, G. R., James, H. E., Hesselink, J. R. & Jones, K. L. Prenatal diagnosis of a craniopharyngioma using ultrasonography and magnetic resonance imaging. Prenat. Diagn. 10, 623–629 (1990).
Chentli, F., Belhimer, F., Kessaci, F. & Mansouri, B. Congenital craniopharyngioma: a case report and literature review. J. Pediatr. Endocrinol. Metab. 25, 1181–1183 (2012).
Crotty, T. B. et al. Papillary craniopharyngioma: a clinicopathological study of 48 cases. J. Neurosurg. 83, 206–214 (1995).
Sorva, R. & Heiskanen, O. Craniopharyngioma in Finland. A study of 123 cases. Acta Neurochir. 81, 85–89 (1986).
Boch, A. L., van Effenterre, R. & Kujas, M. Craniopharyngiomas in two consanguineous siblings: case report. Neurosurgery 41, 1185–1187 (1997).
Green, A. L., Yeh, J. S. & Dias, P. S. Craniopharyngioma in a mother and daughter. Acta Neurochir. 144, 403–404 (2002).
Pereira, A. M. et al. High prevalence of long-term cardiovascular, neurological and psychosocial morbidity after treatment for craniopharyngioma. Clin. Endocrinol. 62, 197–204 (2005).
Muller, H. L. et al. Obesity after childhood craniopharyngioma — German multicenter study on pre-operative risk factors and quality of life. Klin. Padiatr. 213, 244–249 (2001).
Poretti, A., Grotzer, M. A., Ribi, K., Schonle, E. & Boltshauser, E. Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev. Med. Child Neurol. 46, 220–229 (2004).
Visser, J. et al. Late mortality in pediatric patients with craniopharyngioma. J. Neurooncol. 100, 105–111 (2010).
Karavitaki, N. et al. Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clin. Endocrinol. 62, 397–409 (2005).
Pemberton, L. S., Dougal, M., Magee, B. & Gattamaneni, H. R. Experience of external beam radiotherapy given adjuvantly or at relapse following surgery for craniopharyngioma. Radiother. Oncol. 77, 99–104 (2005).
Fahlbusch, R., Honegger, J., Paulus, W., Huk, W. & Buchfelder, M. Surgical treatment of craniopharyngiomas: experience with 168 patients. J. Neurosurg. 90, 237–250 (1999).
Bulow, B. et al. Postoperative prognosis in craniopharyngioma with respect to cardiovascular mortality, survival, and tumor recurrence. J. Clin. Endocrinol. Metab. 83, 3897–3904 (1998).
Adamson, T. E., Wiestler, O. D., Kleihues, P. & Yasargil, M. G. Correlation of clinical and pathological features in surgically treated craniopharyngiomas. J. Neurosurg. 73, 12–17 (1990).
Tavangar, S. M. et al. Craniopharyngioma: a clinicopathological study of 141 cases. Endocr. Pathol. 15, 339–344 (2004).
Weiner, H. L. et al. Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery 35, 1001–1010 (1994).
Hoffmann, A. et al. Fusiform dilatation of the internal carotid artery in childhood-onset craniopharyngioma: multicenter study on incidence and long-term outcome. Pituitary 19, 422–428 (2016).
Wijnen, M. et al. Excess morbidity and mortality in patients with craniopharyngioma: a hospital-based retrospective cohort study. Eur. J. Endocrinol. 178, 93–102 (2018).
Wijnen, M. et al. The metabolic syndrome and its components in 178 patients treated for craniopharyngioma after 16 years of follow-up. Eur. J. Endocrinol. 178, 11–22 (2018).
Wijnen, M. et al. Very long-term sequelae of craniopharyngioma. Eur. J. Endocrinol. 176, 755–767 (2017).
Holmer, H. et al. Hypothalamic involvement and insufficient sex steroid supplementation are associated with low bone mineral density in women with childhood onset craniopharyngioma. Eur. J. Endocrinol. 165, 25–31 (2011).
Hoffmann, A. et al. Nonalcoholic fatty liver disease and fatigue in long-term survivors of childhood-onset craniopharyngioma. Eur. J. Endocrinol. 173, 389–397 (2015).
Heinks, K. et al. Periostin concentrations in childhood-onset craniopharyngioma patients. J. Endocrinol. Invest. 42, 815–824 (2019).
Erfurth, E. M., Holmer, H. & Fjalldal, S. B. Mortality and morbidity in adult craniopharyngioma. Pituitary 16, 46–55 (2013).
Holsken, A., Buchfelder, M., Fahlbusch, R., Blumcke, I. & Buslei, R. Tumour cell migration in adamantinomatous craniopharyngiomas is promoted by activated Wnt-signalling. Acta Neuropathol. 119, 631–639 (2010).
Gaston-Massuet, C. et al. Increased Wingless (Wnt) signaling in pituitary progenitor/stem cells gives rise to pituitary tumors in mice and humans. Proc. Natl Acad. Sci. USA 108, 11482–11487 (2011).
Buslei, R. et al. Nuclear β-catenin accumulation associates with epithelial morphogenesis in craniopharyngiomas. Acta Neuropathol. 113, 585–590 (2007).
Hofmann, B. M. et al. Nuclear β-catenin accumulation as reliable marker for the differentiation between cystic craniopharyngiomas and rathke cleft cysts: a clinico-pathologic approach. Am. J. Surg. Pathol. 30, 1595–1603 (2006).
Burghaus, S. et al. A tumor-specific cellular environment at the brain invasion border of adamantinomatous craniopharyngiomas. Virchows Arch. 456, 287–300 (2010).
Apps, J. R. et al. Imaging invasion: micro-CT imaging of adamantinomatous craniopharyngioma highlights cell type specific spatial relationships of tissue invasion. Acta Neuropathol. Commun. 4, 57 (2016).
Andoniadou, C. L. et al. Sox2+ stem/progenitor cells in the adult mouse pituitary support organ homeostasis and have tumor-inducing potential. Cell Stem Cell 13, 433–445 (2013).
Thimsen, V. et al. Expression of SRY-related HMG box transcription factors (Sox) 2 and 9 in craniopharyngioma subtypes and surrounding brain tissue. Sci. Rep. 7, 15856 (2017).
Andoniadou, C. L. et al. Identification of novel pathways involved in the pathogenesis of human adamantinomatous craniopharyngioma. Acta Neuropathol. 124, 259–271 (2012).
Martinez-Barbera, J. P. & Andoniadou, C. L. Concise review: paracrine role of stem cells in pituitary tumors: a focus on adamantinomatous craniopharyngioma. Stem Cells 34, 268–276 (2016).
Uhrbom, L., Hesselager, G., Nister, M. & Westermark, B. Induction of brain tumors in mice using a recombinant platelet-derived growth factor B-chain retrovirus. Cancer Res. 58, 5275–5279 (1998).
Fomchenko, E. I. et al. Recruited cells can become transformed and overtake PDGF-induced murine gliomas in vivo during tumor progression. PLOS ONE 6, e20605 (2011).
Memarzadeh, S. et al. Enhanced paracrine FGF10 expression promotes formation of multifocal prostate adenocarcinoma and an increase in epithelial androgen receptor. Cancer Cell 12, 572–585 (2007).
Chen, Y. et al. Glioma initiating cells contribute to malignant transformation of host glial cells during tumor tissue remodeling via PDGF signaling. Cancer Lett. 365, 174–181 (2015).
Kode, A. et al. Leukaemogenesis induced by an activating β-catenin mutation in osteoblasts. Nature 506, 240–244 (2014).
Lujambio, A. et al. Non-cell-autonomous tumor suppression by p53. Cell 153, 449–460 (2013).
McCarthy, N. As directed. Nat. Rev. Cancer 13, 824–825 (2013).
Visvader, J. E. & Lindeman, G. J. Cancer stem cells: current status and evolving complexities. Cell Stem Cell 10, 717–728 (2012).
Carreno, G. et al. SHH pathway inhibition is protumourigenic in adamantinomatous craniopharyngioma. Endocr. Relat. Cancer 26, 355–366 (2019).
Donson, A. M. et al. Molecular analyses reveal inflammatory mediators in the solid component and cyst fluid of human adamantinomatous craniopharyngioma. J. Neuropathol. Exp. Neurol. 76, 779–788 (2017).
Gump, J. M. et al. Identification of targets for rational pharmacological therapy in childhood craniopharyngioma. Acta Neuropathol. Commun. 3, 30 (2015).
Zhou, J., Zhang, C., Pan, J., Chen, L. & Qi, S. T. Interleukin-6 induces an epithelialmesenchymal transition phenotype in human adamantinomatous craniopharyngioma cells and promotes tumor cell migration. Mol. Med. Rep. 15, 4123–4131 (2017).
Gomes, D. C. et al. Sonic hedgehog pathway is upregulated in adamantinomatous craniopharyngiomas. Eur. J. Endocrinol. 172, 603–608 (2015).
Holsken, A. et al. Adamantinomatous and papillary craniopharyngiomas are characterized by distinct epigenomic as well as mutational and transcriptomic profiles. Acta Neuropathol. Commun. 4, 20 (2016).
Sekine, S. et al. Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma: evidence for its odontogenic epithelial differentiation. Histopathology 45, 573–579 (2004).
Seemayer, T. A., Blundell, J. S. & Wiglesworth, F. W. Pituitary craniopharyngioma with tooth formation. Cancer 29, 423–430 (1972).
Pascual, J. M. et al. Jakob Erdheim (1874–1937): father of hypophyseal-duct tumors (craniopharyngiomas). Virchows Arch. 467, 459–469 (2015).
Gonzalez-Meljem, J. M. et al. Stem cell senescence drives age-attenuated induction of pituitary tumours in mouse models of paediatric craniopharyngioma. Nat. Commun. 8, 1819 (2017).
Gonzalez-Meljem, J. M. & Martinez-Barbera, J. P. Senescence drives non-cell autonomous tumorigenesis in the pituitary gland. Mol. Cell Oncol. 5, e1435180 (2018).
Kirkland, J. L. & Tchkonia, T. Cellular senescence: a translational perspective. EBioMedicine 21, 21–28 (2017).
Zhu, Y. et al. The Achilles' heel of senescent cells: from transcriptome to senolytic drugs. Aging Cell 14, 644–658 (2015).
Mori, M., Takeshima, H. & Kuratsu, J. Expression of interleukin-6 in human craniopharyngiomas: a possible inducer of tumor-associated inflammation. Int. J. Mol. Med. 14, 505–509 (2004).
Pettorini, B. L. et al. The role of inflammation in the genesis of the cystic component of craniopharyngiomas. Childs Nerv. Syst. 26, 1779–1784 (2010).
Massimi, L., Martelli, C., Caldarelli, M., Castagnola, M. & Desiderio, C. Proteomics in pediatric cystic craniopharyngioma. Brain Pathol. 27, 370–376 (2017).
Coy, S. et al. Multiplexed immunofluorescence reveals potential PD-1/PD-L1 pathway vulnerabilities in craniopharyngioma. Neuro Oncol. 20, 1101–1112 (2018).
Dhomen, N. et al. Oncogenic Braf induces melanocyte senescence and melanoma in mice. Cancer Cell 15, 294–303 (2009).
Hoffmann, A. et al. History before diagnosis in childhood craniopharyngioma: associations with initial presentation and long-term prognosis. Eur. J. Endocrinol. 173, 853–862 (2015).
Muller, H. L. et al. Xanthogranuloma, Rathke's cyst, and childhood craniopharyngioma: results of prospective multinational studies of children and adolescents with rare sellar malformations. J. Clin. Endocrinol. Metab. 97, 3935–3943 (2012).
Walz, P. C. et al. Pediatric pituitary adenomas. Childs Nerv. Syst. https://doi.org/10.1007/s00381-019-04293-y (2019).
Pal, A., Leaver, L. & Wass, J. Pituitary adenomas. BMJ 365, l2091 (2019).
Boekhoff, S., Bison, B., Eveslage, M., Sowithayasakul, P. & Muller, H. L. Craniopharyngiomas presenting as incidentalomas: results of KRANIOPHARYNGEOM 2007. Pituitary 22, 532–541 (2019).
Muller, H. L. et al. Longitudinal study on growth and body mass index before and after diagnosis of childhood craniopharyngioma. J. Clin. Endocrinol. Metab. 89, 3298–3305 (2004). This study demonstrates the effect of hypothalamic involvement on growth and the development of obesity before diagnosis and during long-term follow-up.
Prieto, R., Pascual, J. M. & Barrios, L. Optic chiasm distortions caused by craniopharyngiomas: clinical and magnetic resonance imaging correlation and influence on visual outcome. World Neurosurg. 83, 500–529 (2015).
Elliott, R. E., Jane, J. A. Jr & Wisoff, J. H. Surgical management of craniopharyngiomas in children: meta-analysis and comparison of transcranial and transsphenoidal approaches. Neurosurgery 69, 630–643 (2011).
Muller, H. L. Childhood craniopharyngioma. Recent advances in diagnosis, treatment and follow-up. Horm. Res. 69, 193–202 (2008).
Hoffman, H. J. et al. Aggressive surgical management of craniopharyngiomas in children. J. Neurosurg. 76, 47–52 (1992).
Honegger, J., Buchfelder, M. & Fahlbusch, R. Surgical treatment of craniopharyngiomas: endocrinological results. J. Neurosurg. 90, 251–257 (1999).
Feng, Y., Ni, M., Wang, Y. G. & Zhong, L. Y. Comparison of neuroendocrine dysfunction in patients with adamantinomatous and papillary craniopharyngiomas. Exp. Ther. Med. 17, 51–56 (2019).
Pascual, J. M. et al. Craniopharyngiomas primarily involving the hypothalamus: a model of neurosurgical lesions to elucidate the neurobiological basis of psychiatric disorders. World Neurosurg. 120, e1245–e1278 (2018).
Roth, C. L., Gebhardt, U. & Muller, H. L. Appetite-regulating hormone changes in patients with craniopharyngioma. Obesity 19, 36–42 (2011).
Kilday, J. P. et al. Favorable survival and metabolic outcome for children with diencephalic syndrome using a radiation-sparing approach. J. Neurooncol. 116, 195–204 (2014).
Hoffmann, A., Gebhardt, U., Sterkenburg, A. S., Warmuth-Metz, M. & Muller, H. L. Diencephalic syndrome in childhood craniopharyngioma-results of german multicenter studies on 485 long-term survivors of childhood craniopharyngioma. J. Clin. Endocrinol. Metab. 99, 3972–3977 (2014).
Rossi, A. et al. Neuroimaging of pediatric craniopharyngiomas: a pictorial essay. J. Pediatr. Endocrinol. Metab. 19, 299–319 (2006).
Hoffmann, A. et al. Childhood craniopharyngioma — changes of treatment strategies in the trials KRANIOPHARYNGEOM 2000/2007. Klin. Padiatr. 226, 161–168 (2014).
Elliott, R. E., Moshel, Y. A. & Wisoff, J. H. Minimal residual calcification and recurrence after gross-total resection of craniopharyngioma in children. J. Neurosurg. Pediatr. 3, 276–283 (2009).
Sartoretti-Schefer, S., Wichmann, W., Aguzzi, A. & Valavanis, A. MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. AJNR Am. J. Neuroradiol. 18, 77–87 (1997).
Steno, J., Malacek, M. & Bizik, I. Tumor-third ventricular relationships in supradiaphragmatic craniopharyngiomas: correlation of morphological, magnetic resonance imaging, and operative findings. Neurosurgery 54, 1051–1058 (2004).
Omay, S. B. et al. Do craniopharyngioma molecular signatures correlate with clinical characteristics? J. Neurosurg. 128, 1473–1478 (2018).
Fujio, S. et al. A clinical rule for preoperative prediction of braf mutation status in craniopharyngiomas. Neurosurgery 85, 204–210 (2019).
Kordes, U. et al. Ectopic craniopharyngioma. Klin. Padiatr. 223, 176–177 (2011).
Prieto, R., Pascual, J. M. & Barrios, L. Topographic diagnosis of craniopharyngiomas: the accuracy of MRI findings observed on conventional T1 and T2 images. AJNR Am. J. Neuroradiol. 38, 2073–2080 (2017).
Prieto, R., Pascual, J. M., Rosdolsky, M. & Barrios, L. Preoperative assessment of craniopharyngioma adherence: magnetic resonance imaging findings correlated with the severity of tumor attachment to the hypothalamus. World Neurosurg. 110, e404–e426 (2018).
Fouladi, M. et al. Survival and functional outcome of children with hypothalamic/chiasmatic tumors. Cancer 97, 1084–1092 (2003).
Surawicz, T. S. et al. Descriptive epidemiology of primary brain and CNS tumors: results from the central brain tumor registry of the United States, 1990–1994. Neuro Oncol. 1, 14–25 (1999).
Ogashiwa, M. et al. [Clinicopathological study on low grade glioma. In relation to malignant transformation]. Neurol. Med. Chir. 30, 820–826 (1990).
Barkovich, J. A. in Pediatric Neuroimaging (ed. Barkovich, J. A.) 445–446 (Williams & Wilkins, 2000).
Martinez, R., Honegger, J., Fahlbusch, R. & Buchfelder, M. Endocrine findings in patients with optico-hypothalamic gliomas. Exp. Clin. Endocrinol. Diabetes 111, 162–167 (2003).
Salzman, K. L. et al. Primary intracranial germ cell tumors: clinicopathologic review of 32 cases. Pediatr. Pathol. Lab. Med. 17, 713–727 (1997).
Fujimaki, T. et al. CT and MRI features of intracranial germ cell tumors. J. Neurooncol. 19, 217–226 (1994).
Kanagaki, M. et al. MRI and CT findings of neurohypophyseal germinoma. Eur. J. Radiol. 49, 204–211 (2004).
Kamoshima, Y., Sawamura, Y., Motegi, H., Kubota, K. & Houkin, K. Xanthogranuloma of the sellar region of children: series of five cases and literature review. Neurol. Med. Chir. 51, 689–693 (2011).
Bonneville, J. F., Bonneville, F. & Cattin, F. Magnetic resonance imaging of pituitary adenomas. Eur. Radiol. 15, 543–548 (2005).
Steiner, E., Imhof, H. & Knosp, E. Gd-DTPA enhanced high resolution MR imaging of pituitary adenomas. Radiographics 9, 587–598 (1989).
Pascual, J. M., Prieto, R., Carrasco, R. & Barrios, L. Displacement of mammillary bodies by craniopharyngiomas involving the third ventricle: surgical-MRI correlation and use in topographical diagnosis. J. Neurosurg. 119, 381–405 (2013).
Puget, S. et al. Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J. Neurosurg. 106, 3–12 (2007).
Van Gompel, J. J., Nippoldt, T. B., Higgins, D. M. & Meyer, F. B. Magnetic resonance imaging-graded hypothalamic compression in surgically treated adult craniopharyngiomas determining postoperative obesity. Neurosurg. Focus 28, E3 (2010).
Muller, H. L. et al. Post-operative hypothalamic lesions and obesity in childhood craniopharyngioma: results of the multinational prospective trial KRANIOPHARYNGEOM 2000 after 3-year follow-up. Eur. J. Endocrinol. 165, 17–24 (2011).
Prieto, R. et al. Craniopharyngioma adherence: a reappraisal of the evidence. Neurosurg. Rev. https://doi.org/10.1007/s10143-018-1010-9 (2018).
Muller, H. L. et al. Prognosis and sequela in patients with childhood craniopharyngioma — results of HIT-ENDO and update on KRANIOPHARYNGEOM 2000. Klin. Padiatr. 216, 343–348 (2004).
Cheng, J., Shao, Q., Pan, Z. & You, J. Analysis and long-term follow-up of the surgical treatment of children with craniopharyngioma. J. Craniofac. Surg. 27, e763–e766 (2016).
Daubenbuchel, A. M. et al. Hydrocephalus and hypothalamic involvement in pediatric patients with craniopharyngioma or cysts of Rathke's pouch: impact on long-term prognosis. Eur. J. Endocrinol. 172, 561–569 (2015).
Elowe-Gruau, E. et al. Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity. J. Clin. Endocrinol. Metab. 98, 2376–2382 (2013). A unique single-centre comparison of different treatment strategies (GTR versus hypothalamus-sparing surgery plus radiotherapy) that shows that hypothalamus-sparing strategies were superior in terms of long-term obesity and QOL and with comparable relapse and progression rates.
Sterkenburg, A. S. et al. Survival, hypothalamic obesity, and neuropsychological/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes. Neuro Oncol. 17, 1029–1038 (2015). This 20-year follow-up analysis of 485 patients with childhood-onset CP shows impaired overall survival in patients with hypothalamic involvement. Relapse and progression rates were similar with regards to different degrees of resection (GTR versus incomplete resection).
Fjalldal, S. et al. Detailed assessment of hypothalamic damage in craniopharyngioma patients with obesity. Int. J. Obes. 43, 533–544 (2019). This study demonstrates that hypothalamus volume on MRI is a predictor for severity of hypothalamic syndrome after CP.
Park, S. W. et al. Tumor origin and growth pattern at diagnosis and surgical hypothalamic damage predict obesity in pediatric craniopharyngioma. J. Neurooncol. 113, 417–424 (2013).
Roth, C. L. et al. Semiquantitative analysis of hypothalamic damage on MRI predicts risk for hypothalamic obesity. Obesity 23, 1226–1233 (2015). A grading system for imaging of hypothalamic lesions after initial surgery in CP, showing high predictive sensitivity for the devlopment of hypothalamic obesity.
Kilday, J. P. et al. Intracystic interferon-alpha in pediatric craniopharyngioma patients: an international multicenter assessment on behalf of SIOPE and ISPN. Neuro Oncol. 19, 1398–1407 (2017).
Zhang, S., Fang, Y., Cai, B. W., Xu, J. G. & You, C. Intracystic bleomycin for cystic craniopharyngiomas in children. Cochrane Database Syst. Rev. 7, CD008890 (2016).
Zhu, W. et al. A reformed surgical treatment modality for children with giant cystic craniopharyngioma. Childs Nerv. Syst. 33, 1491–1500 (2017).
Pascual, J. M., Prieto, R. & Carrasco, R. Infundibulo-tuberal or not strictly intraventricular craniopharyngioma: evidence for a major topographical category. Acta Neurochir. 153, 2403–2425 (2011).
Muller, H. L. Hypothalamic involvement in craniopharyngioma — implications for surgical, radiooncological, and molecularly targeted treatment strategies. Pediatr. Blood Cancer 65, e26936 (2018).
Mortini, P. et al. Magnetic resonance imaging as predictor of functional outcome in craniopharyngiomas. Endocrine 51, 148–162 (2016). A study analysing and comparing the predictive value of different neuroradiological grading systems of hypothalamic involvement or lesions for outcome after CP.
Pascual, J. M. et al. [The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part I: historical review of the topographical diagnosis and classification schemes of craniopharyngiomas]. Neurocirugia 25, 154–169 (2014).
Pascual, J. M. et al. [The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part II: anatomical and neuroradiological evidence to define triple-axis topography and its usefulness in predicting individual surgical risk]. Neurocirugia 25, 211–239 (2014).
Prieto, R. et al. Craniopharyngioma adherence: a comprehensive topographical categorization and outcome-related risk stratification model based on the methodical examination of 500 tumors. Neurosurg. Focus 41, E13 (2016).
Koutourousiou, M., Fernandez-Miranda, J. C., Wang, E. W., Snyderman, C. H. & Gardner, P. A. The limits of transsellar/transtuberculum surgery for craniopharyngioma. J. Neurosurg. Sci. 62, 301–309 (2018).
Locatelli, D. et al. Endoscopic endonasal approaches to anterior skull base defects in pediatric patients. Childs Nerv. Syst. 22, 1411–1418 (2006).
Giovannetti, F. et al. Minimally-invasive endoscopic-assisted sinus augmentation. J. Craniofac. Surg. 30, e359–e362 (2019).
Tomita, T. & Bowman, R. M. Craniopharyngiomas in children: surgical experience at Children's Memorial hospital. Childs Nerv. Syst. 21, 729–746 (2005).
Wang, K. C., Hong, S. H., Kim, S. K. & Cho, B. K. Origin of craniopharyngiomas: implication on the growth pattern. Childs Nerv. Syst. 21, 628–634 (2005).
Tosta-Hernandez, P. D. C. et al. Childhood craniopharyngioma: a 22-year challenging follow-up in a single center. Horm. Metab. Res. 50, 675–682 (2018).
Feng, S. Y. et al. Microsurgical treatment of craniopharyngioma: experiences on 183 consecutive patients. Medicine 97, e11746 (2018).
Liu, J. K., Sevak, I. A., Carmel, P. W. & Eloy, J. A. Microscopic versus endoscopic approaches for craniopharyngiomas: choosing the optimal surgical corridor for maximizing extent of resection and complication avoidance using a personalized, tailored approach. Neurosurg. Focus 41, E5 (2016).
Chamoun, R. & Couldwell, W. T. Transcortical-transforaminal microscopic approach for purely intraventricular craniopharyngioma. Neurosurg. Focus 34, Video 4 (2013).
Hardesty, D. A., Montaser, A. S., Beer-Furlan, A., Carrau, R. L. & Prevedello, D. M. Limits of endoscopic endonasal surgery for III ventricle craniopharyngiomas. J. Neurosurg. Sci. 62, 310–321 (2018).
de Vile, C. J. et al. Obesity in childhood craniopharyngioma: relation to post-operative hypothalamic damage shown by magnetic resonance imaging. J. Clin. Endocrinol. Metab. 81, 2734–2737 (1996).
Garre, M. L. & Cama, A. Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr. Opin. Pediatr. 19, 471–479 (2007).
Flitsch, J., Muller, H. L. & Burkhardt, T. Surgical strategies in childhood craniopharyngioma. Front. Endocrinol. 2, 96 (2011).
Mallucci, C. et al. Management of craniopharyngioma: the Liverpool experience following the introduction of the CCLG guidelines. Introducing a new risk assessment grading system. Childs Nerv. Syst. 28, 1181–1192 (2012).
Fjalldal, S. et al. Hypothalamic involvement predicts cognitive performance and psychosocial health in long-term survivors of childhood craniopharyngioma. J. Clin. Endocrinol. Metab. 98, 3253–3262 (2013). This study of neuropsychological sequelae after CP reveals an association with different grades of hypothalamic involvement.
Wang, X. Y., Xu, S. J. & Li, X. G. Post-operative implantation metastasis of craniopharyngioma: a case report. J. Int. Med. Res. 38, 1876–1882 (2010).
Adeberg, S. et al. Dosimetric comparison of proton radiation therapy, volumetric modulated arc therapy, and three-dimensional conformal radiotherapy based on intracranial tumor location. Cancers 10, E401 (2018).
Hill, T. K. et al. Patterns of care in pediatric craniopharyngioma: outcomes following definitive radiotherapy. Anticancer Res. 39, 803–807 (2019).
Merchant, T. E. et al. Necrosis, vasculopathy, and neurological complications after proton therapy for childhood craniopharyngioma: results from a prospective trial and a photon cohort comparison. Int. J. Radiat. Oncol. Biol. Phys. 96, S120–S121 (2016).
Merchant, T., Indelicato, D., Hua, C., Wu, S. & Conklin, H. Comparison of academic scores after proton and photon therapy in children and young adults with craniopharyngioma. Pediatr. Blood Cancer 64, e26772 (2017).
Borrill, R. et al. Papillary craniopharyngioma in a 4-year-old girl with BRAF V600E mutation: a case report and review of the literature. Childs Nerv. Syst. 35, 169–173 (2019).
Dandurand, C., Sepehry, A. A., Asadi Lari, M. H., Akagami, R. & Gooderham, P. Adult craniopharyngioma: case series, systematic review, and meta-analysis. Neurosurgery 83, 631–641 (2018).
Brastianos, P. K. et al. Dramatic response of BRAF V600E mutant papillary craniopharyngioma to targeted therapy. J. Natl Cancer Inst. 108, djv310 (2016). This report describes an impressive treatment response to targeted therapy in BRAF V600E -mutant PCP.
Eveslage, M. et al. The postoperative quality of life in children and adolescents with craniopharyngioma. Dtsch Arztebl. Int. 116, 321–328 (2019).
Fournier-Goodnight, A. S. et al. Neurocognitive functioning in pediatric craniopharyngioma: performance before treatment with proton therapy. J. Neurooncol. 134, 97–105 (2017).
DeVile, C. J., Grant, D. B., Hayward, R. D. & Stanhope, R. Growth and endocrine sequelae of craniopharyngioma. Arch. Dis. Child 75, 108–114 (1996).
Karavitaki, N., Cudlip, S., Adams, C. B. & Wass, J. A. Craniopharyngiomas. Endocr. Rev. 27, 371–397 (2006).
Smith, T. R., Cote, D. J., Jane, J. A. Jr & Laws, E. R. Jr. Physiological growth hormone replacement and rate of recurrence of craniopharyngioma: the Genentech National Cooperative Growth Study. J. Neurosurg. Pediatr. 18, 408–412 (2016).
Heinks, K. et al. Quality of life and growth after childhood craniopharyngioma: results of the multinational trial KRANIOPHARYNGEOM 2007. Endocrine 59, 364–372 (2018).
Boekhoff, S., Bogusz, A., Sterkenburg, A. S., Eveslage, M. & Muller, H. L. Long-term effects of growth hormone replacement therapy in childhood-onset craniopharyngioma: results of the German craniopharyngioma registry (HIT-Endo). Eur. J. Endocrinol. 179, 331–341 (2018).
Karavitaki, N. et al. GH replacement does not increase the risk of recurrence in patients with craniopharyngioma. Clin. Endocrinol. 64, 556–560 (2006).
Lustig, R. H. Hypothalamic obesity after craniopharyngioma: mechanisms, diagnosis, and treatment. Front. Endocrinol. 2, 60 (2011).
Muller, H. L. Craniopharyngioma and hypothalamic injury: latest insights into consequent eating disorders and obesity. Curr. Opin. Endocrinol. Diabetes Obes. 23, 81–89 (2016).
Daubenbuchel, A. M. & Muller, H. L. Neuroendocrine disorders in pediatric craniopharyngioma patients. J. Clin. Med. 4, 389–413 (2015).
Daubenbuchel, A. M. et al. Oxytocin in survivors of childhood-onset craniopharyngioma. Endocrine 54, 524–531 (2016). The first report in patients with CP showing that oxytocin concentrations are decreased in the saliva of patients with anterior hypothalamic lesions.
Hoffmann, A. et al. First experiences with neuropsychological effects of oxytocin administration in childhood-onset craniopharyngioma. Endocrine 56, 175–185 (2017).
Daubenbuchel, A. M. et al. Eating behaviour and oxytocin in patients with childhood-onset craniopharyngioma and different grades of hypothalamic involvement. Pediatr. Obes. 14, e12527 (2019).
Cook, N., Miller, J. & Hart, J. Parent observed neuro-behavioral and pro-social improvements with oxytocin following surgical resection of craniopharyngioma. J. Pediatr. Endocrinol. Metab. 29, 995–1000 (2016).
Hsu, E. A., Miller, J. L., Perez, F. A. & Roth, C. L. Oxytocin and naltrexone successfully treat hypothalamic obesity in a boy post-craniopharyngioma resection. J. Clin. Endocrinol. Metab. 103, 370–375 (2018).
van Iersel, L. et al. Pathophysiology and individualized treatment of hypothalamic obesity following craniopharyngioma and other suprasellar tumors: a systematic review. Endocr. Rev. 40, 193–235 (2019). A comprehensive review on treatment modalities for hypothalamic syndrome in CP.
Muller, H. L. et al. First experiences with laparoscopic adjustable gastric banding (LAGB) in the treatment of patients with childhood craniopharyngioma and morbid obesity. Klin. Padiatr. 219, 323–325 (2007).
Inge, T. H. et al. Gastric bypass surgery for treatment of hypothalamic obesity after craniopharyngioma therapy. Nat. Clin. Pract. Endocrinol. Metab. 3, 606–609 (2007).
Muller, H. L., Gebhardt, U., Maroske, J. & Hanisch, E. Long-term follow-up of morbidly obese patients with childhood craniopharyngioma after laparoscopic adjustable gastric banding (LAGB). Klin. Padiatr. 223, 372–373 (2011).
Bretault, M. et al. Bariatric surgery following treatment for craniopharyngioma: a systematic review and individual-level data meta-analysis. J. Clin. Endocrinol. Metab. 98, 2239–2246 (2013).
Wijnen, M. et al. Efficacy and safety of bariatric surgery for craniopharyngioma-related hypothalamic obesity: a matched case-control study with 2 years of follow-up. Int. J. Obes. 41, 210–216 (2017).
Muller, H. L. et al. Functional capacity, obesity and hypothalamic involvement: cross-sectional study on 212 patients with childhood craniopharyngioma. Klin. Padiatr. 215, 310–314 (2003).
Muller, H. L. et al. Longitudinal study on quality of life in 102 survivors of childhood craniopharyngioma. Childs Nerv. Syst. 21, 975–980 (2005).
Muller, H. L. et al. Functional capacity and body mass index in patients with sellar masses — cross-sectional study on 403 patients diagnosed during childhood and adolescence. Childs Nerv. Syst. 21, 539–545 (2005).
Mehren, A. et al. Self- and informant-rated apathy in patients with childhood-onset craniopharyngioma. J. Neurooncol. 140, 27–35 (2018).
Ondruch, A., Maryniak, A., Kropiwnicki, T., Roszkowski, M. & Daszkiewicz, P. Cognitive and social functioning in children and adolescents after the removal of craniopharyngioma. Childs Nerv. Syst. 27, 391–397 (2011).
Crom, D. et al. Health status in long-term survivors of pediatric craniopharyngiomas. J. Neurosci. Nurs. 42, 323–328 (2010).
Bogusz, A. et al. Posterior hypothalamus-sparing surgery improves outcome after childhood craniopharyngioma. Endocr. Connect 8, 481–492 (2019).
Carpentieri, S. C. et al. Memory deficits among children with craniopharyngiomas. Neurosurgery 49, 1053–1057 (2001).
Ozyurt, J., Muller, H. L. & Thiel, C. M. A systematic review of cognitive performance in patients with childhood craniopharyngioma. J. Neurooncol. 125, 9–21 (2015). A comprehensive review on the neuropsychological sequelae in childhood-onset CP.
Ozyurt, J. et al. Neuropsychological outcome in patients with childhood craniopharyngioma and hypothalamic involvement. J. Pediatr. 164, 876–881.e4 (2014).
Carpentieri, S. et al. Memory deficits among children with craniopharyngioma. Neurosurgery 49, 1053–1058 (2001).
Cohen, M., Guger, S. & Hamilton, J. Long term sequelae of pediatric craniopharyngioma — literature review and 20 years of experience. Front. Endocrinol. 2, 81 (2011).
Hankinson, T. C. et al. Patterns of care for craniopharyngioma: survey of members of the American Association of Neurological Surgeons. Pediatr. Neurosurg. 49, 131–136 (2013).
Schwartz, T. H. A role for centers of excellence in transsphenoidal surgery. World Neurosurg. 80, 270–271 (2013).
Casanueva, F. F. et al. Criteria for the definition of Pituitary Tumor Centers of Excellence (PTCOE): a Pituitary Society statement. Pituitary 20, 489–498 (2017).
Tallen, G. et al. Strategies to improve the quality of survival for childhood brain tumour survivors. Eur. J. Paediatr. Neurol. 19, 619–639 (2015).
Muller, H. L. et al. Low concordance between surgical and radiological assessment of degree of resection and treatment-related hypothalamic damage: results of KRANIOPHARYNGEOM 2007. Pituitary 21, 371–378 (2018).
Rimkus, T. K., Carpenter, R. L., Qasem, S., Chan, M. & Lo, H. W. Targeting the sonic hedgehog signaling pathway: review of Smoothened and GLI inhibitors. Cancers 8, E22 (2016).
Sekulic, A. et al. Long-term safety and efficacy of vismodegib in patients with advanced basal cell carcinoma: final update of the pivotal ERIVANCE BCC study. BMC Cancer 17, 332 (2017).
Miller, C., Guillaume, D., Dusenbery, K., Clark, H. B. & Moertel, C. Report of effective trametinib therapy in 2 children with progressive hypothalamic optic pathway pilocytic astrocytoma: documentation of volumetric response. J. Neurosurg. Pediatr. 19, 319–324 (2017).
Robert, C. et al. METRIC phase III study: efficacy of trametinib (T), a potent and selective mek inhibitor (MEKi), in progression-free survival (PFS) and overall survival (OS), compared with chemotherapy (C) in patients (pts) with BRAFV600/k mutant advanced or metastatic melanoma (MM). J. Clin. Oncol. 30, LBA8509 (2012).
Grob, S. et al. Targeting IL-6 is a potential treatment for primary cystic craniopharyngioma. Front. Oncol. 9, 791 (2019).
Roque, A. & Odia, Y. BRAF-V600E mutant papillary craniopharyngioma dramatically responds to combination BRAF and MEK inhibitors. CNS Oncol. 6, 95–99 (2017).
Himes, B. T. et al. Recurrent papillary craniopharyngioma with BRAF V600E mutation treated with dabrafenib: case report. J. Neurosurg. 130, 1299–1303 (2019).
Juratli, T. A. et al. Targeted treatment of papillary craniopharyngiomas harboring BRAF V600E mutations. Cancer 125, 2910–2914 (2019).
Peng, J. & Müller, H. L. Personalized therapy in craniopharyngioma — novel perspectives and limitations. J. Xiangya Med. 2, 71–76 (2017).
Gupta, D. K. et al. Recurrence in pediatric craniopharyngiomas: analysis of clinical and histological features. Childs Nerv. Syst. 22, 50–55 (2006).
Yasargil, M. G. et al. Total removal of craniopharyngiomas. Approaches and long-term results in 144 patients. J. Neurosurg. 73, 3–11 (1990).
Larijani, B. et al. Presentation and outcome of 93 cases of craniopharyngioma. Eur. J. Cancer Care 13, 11–15 (2004).
Hafez, M. A., ElMekkawy, S., AbdelBadie, H., Mohy, M. & Omar, M. Pediatric craniopharyngioma — rationale for multimodal management: the Egyptian experience. J. Pediatr. Endocrinol. Metab. 19, 371–380 (2006).
Zhang, Y. Q., Wang, C. C. & Ma, Z. Y. Pediatric craniopharyngiomas: clinicomorphological study of 189 cases. Pediatr. Neurosurg. 36, 80–84 (2002).
Adeloye, A., Nottidge, V. A. & Udi, J. Craniopharyngioma in Nigerian children. Childs Nerv. Syst. 4, 128–134 (1988).
Ersahin, Y., Yurtseven, T., Ozgiray, E. & Mutluer, S. Craniopharyngiomas in children: Turkey experience. Childs Nerv. Syst. 21, 766–772 (2005).
Amayiri, N. et al. Review of management and morbidity of pediatric craniopharyngioma patients in a low-middle-income country: a 12-year experience. Childs Nerv. Syst. 33, 941–950 (2017).
Rolland-Cachera, M. F. et al. Body mass index variations: centiles from birth to 87 years. Eur. J. Clin. Nutr. 45, 13–21 (1991).
Mason, P. W., Krawiecki, N. & Meacham, L. R. The use of dextroamphetamine to treat obesity and hyperphagia in children treated for craniopharyngioma. Arch. Pediatr. Adolesc. Med. 156, 887–892 (2002).
Ismail, D., O'Connell, M. A. & Zacharin, M. R. Dexamphetamine use for management of obesity and hypersomnolence following hypothalamic injury. J. Pediatr. Endocrinol. Metab. 19, 129–134 (2006).
Denzer, C. et al. Treatment of hypothalamic obesity with dextroamphetamine: a case series. Obes. Facts 12, 91–102 (2019).
Elfers, C. T. & Roth, C. L. Effects of methylphenidate on weight gain and food intake in hypothalamic obesity. Front. Endocrinol. 2, 78 (2011).
Lustig, R. H. et al. Octreotide therapy of pediatric hypothalamic obesity: a double-blind, placebo-controlled trial. J. Clin. Endocrinol. Metab. 88, 2586–2592 (2003).
Hamilton, J. K. et al. Hypothalamic obesity following craniopharyngioma surgery: results of a pilot trial of combined diazoxide and metformin therapy. Int. J. Pediatr. Endocrinol. 2011, 417949 (2011).
Zoicas, F., Droste, M., Mayr, B., Buchfelder, M. & Schofl, C. GLP-1 analogues as a new treatment option for hypothalamic obesity in adults: report of nine cases. Eur. J. Endocrinol. 168, 699–706 (2013).
Kalina, M. A. et al. Carbohydrate-lipid profile and use of metformin with micronized fenofibrate in reducing metabolic consequences of craniopharyngioma treatment in children: single institution experience. J. Pediatr. Endocrinol. Metab. 28, 45–51 (2015).
Acknowledgements
H.L.M. is supported by the German Childhood Cancer Foundation, Bonn, Germany (grant DKS2014/13). The authors thank C. L. Andoniadou, Centre for Craniofacial and Regenerative Biology, King’s College London, London, UK, for help in preparing Fig. 2.
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Introduction (H.L.M.); Epidemiology (H.L.M.); Mechanisms/pathophysiology (J.-P.M.-B.); Diagnosis, screening and prevention (H.L.M. and M.W.-M.); Management (T.E.M. and S.P.); Quality of life (H.L.M., T.E.M. and S.P.); Outlook (H.L.M.); Overview of the Primer (H.L.M.).
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H.L.M. has received reimbursement of participation fees for scientific meetings and continuing medical education events from the following companies: Ferring, Lilly, Pfizer, Sandoz/Hexal, Novo Nordisk, Ipsen and Merck Serono. He has received reimbursement of travel expenses from Ipsen and lecture honoraria from Pfizer. The remaining authors declare no competing interests.
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Nature Reviews Disease Primers thanks E. T. Hidalgo, J. M. Pascual, R. Prieto, T. Schwartz and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.
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Müller, H.L., Merchant, T.E., Warmuth-Metz, M. et al. Craniopharyngioma. Nat Rev Dis Primers 5, 75 (2019). https://doi.org/10.1038/s41572-019-0125-9
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DOI: https://doi.org/10.1038/s41572-019-0125-9
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