• Lauren Ostling
  • Daphne A. Haas-Kogan
  • Robert H. Lustig
  • Nalin GuptaEmail author
Part of the Pediatric Oncology book series (PEDIATRICO)


Craniopharyngiomas are histologically benign neuroepithelial tumors that arise from rests of squamous cell epithelium that remain along the path of the primitive craniopharyngeal duct and adenohypophysis. Although considered benign (WHO grade I) tumors of the sellar region, they have a propensity to adhere to adjacent structures such as the hypothalamus and optic chiasm. This feature can prevent a complete resection from being achieved. Furthermore, even if a complete resection is achieved, there is the possibility of significant postoperative morbidity. Currently, there are reports in the literature favoring a variety of approaches such as gross total resection, subtotal resection with radiation, or intracystic therapy, including 32phosphorous, bleomycin, and interferon-alpha. In the pediatric population, the short-term and long-term adverse effects of radiation therapy must be carefully weighed against the potential surgical morbidity.


Diabetes Insipidus Optic Chiasm Subtotal Resection Gross Total Resection Proton Therapy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. Ali ZS, Lang SS, Kamat AR, Adappa ND, Palmer JN, Storm PB, Lee JY (2013) Suprasellar pediatric craniopharyngioma resection via endonasal endoscopic approach. Childs Nerv Syst 29(11):2065–2070CrossRefPubMedGoogle Scholar
  2. Anderson CA, Wilkening GN, Filley CM, Reardon MS, Kleinschmidt-DeMasters BK (1997) Neurobehavioral outcome in pediatric craniopharyngioma. Pediatr Neurosurg 26(5):255–260CrossRefPubMedGoogle Scholar
  3. Barloon TJ, Yuh WT, Sato Y, Sickels WJ (1988) Frontal lobe implantation of craniopharyngioma by repeated needle aspirations. AJNR Am J Neuroradiol 9(2):406–407PubMedGoogle Scholar
  4. Bishop AJ, Greenfield B, Mahajan A, Paulino AC, Okcu MF, Allen PK, Chintagumpala M, Kahalley LS, McAleer MF, McGovern SL, Whitehead WE, Grosshans DR (2014) Proton beam therapy versus conformal photon radiation therapy for childhood craniopharyngioma: multi-institutional analysis of outcomes, cyst dynamics, and toxicity. Int J Radiat Oncol Biol Phys 90(2):354–361CrossRefPubMedPubMedCentralGoogle Scholar
  5. Blackburn TP, Doughty D, Plowman PN (1999) Stereotactic intracavitary therapy of recurrent cystic craniopharyngioma by instillation of 90yttrium. British Journal of Neurosurgery 13:359–365Google Scholar
  6. Boehling NS, Grosshans DR, Bluett JB, Palmer MT, Song X, Amos RA, Sahoo N, Meyer JJ, Mahajan A, Woo SY (2012) Dosimetric comparison of three-dimensional conformal proton radiotherapy, intensity-modulated proton therapy, and intensity-modulated radiotherapy for treatment of pediatric craniopharyngiomas. Int J Radiat Oncol Biol Phys 82(2):643–652CrossRefPubMedGoogle Scholar
  7. Bojanowski K, Marchel A (2006) Long-term result of the surgical treatment of craniopharyngioma. Polish Journal of Neurology and Neurosurgery 40:478-482Google Scholar
  8. Bray GA, Gallagher TF (1975) Manifestations of hypothalamic obesity in man: a comprehensiveinvestigation of eight patients and a review of the literature. Medicine 54:301– 333Google Scholar
  9. Bretault M, Boillot A, Muzard L, Poitou C, Oppert JM, Barsamian C, Gatta B, Müller H, Weismann D, Rottembourg D, Inge T, Veyrie N, Carette C, Czernichow S (2013) Clinical review: bariatric surgery following treatment for craniopharyngioma: a systematic review and individual-level data meta-analysis. J Clin Endocrinol Metab 98(6):2239–2246CrossRefPubMedGoogle Scholar
  10. Bulow B, Attewell R, Hagmar L, Malmstrom P, Nordstrom CH, Erfurth EM (1998) Postoperative prognosis in craniopharyngioma with respect to cardiovascular mortality, survival, and tumor recurrence. J Clin Endocrinol Metab 83(11):3897–3904PubMedGoogle Scholar
  11. Buslei R, Nolde M, Hofmann B, Meissner S, Eyupoglu IY, Siebzehnrübl F, Hahnen E, Kreutzer J, Fahlbusch R (2005) Common mutations of beta-catenin in adamantinomatous craniopharyngiomas but not in other tumours originating from the sellar region. Acta Neuropathol 109(6):589–597CrossRefPubMedGoogle Scholar
  12. Caldarelli M, Massimi L, Tamburrini G, Cappa M, Di Rocco C (2005) Long-term results of the surgical treatment of craniopharyngioma: the experience at the Policlinico Gemelli, Catholic University, Rome. Childs Nerv Syst 21(8–9):747–757CrossRefPubMedGoogle Scholar
  13. Cao J, Lin JP, Yang LX, Chen K, Huang ZS (2010) Expression of aberrant beta-catenin and impaired p63 in craniopharyngiomas. Br J Neurosurg 24(3):249–256CrossRefPubMedGoogle Scholar
  14. Cavalheiro S, Di Rocco C, Valenzuela S, Dastoli PA, Tamburrini G, Massimi L, Nicacio JM, Faquini IV, Ierardi DF, Silva NS, Pettorini BL, Toledo SR (2010) Craniopharyngiomas: intratumoral chemotherapy with interferon-alpha: a multicenter preliminary study with 60 cases. Neurosurg Focus 28(4):E12CrossRefPubMedGoogle Scholar
  15. Chung WY, Pan DH, Shiau CY, Guo WY, Wang LW (2000) Gamma knife radiosurgery for craniopharyngiomas. J Neurosurg 93(Suppl 3):47–56PubMedGoogle Scholar
  16. Clark AJ, Cage TA, Aranda D, Parsa AT, Auguste KI, Gupta N (2012) Treatment related morbidity and the management of pediatric craniopharyngioma: a systematic review. J Neurosurg Pediatr 10(4):293–301CrossRefPubMedGoogle Scholar
  17. Clark AJ, Cage TA, Aranda D, Parsa AT, Sun PP, Auguste KI, Gupta N (2013) A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma. Childs Nerv Syst 29(2):231–238CrossRefPubMedGoogle Scholar
  18. Cohen M, Bartels U, Branson H, Kulkarni AV, Hamilton J (2013) Trends in treatment and outcomes of pediatric craniopharyngioma, 1975–2011. Neuro Oncol 15(6):767–774CrossRefPubMedPubMedCentralGoogle Scholar
  19. Combs SE, Thilmann C, Huber PE, Hoess A, Debus J, Schulz-Ertner D (2007) Achievement of long-term local control in patients with craniopharyngiomas using high precision stereotactic radiotherapy. Cancer 109(11):2308–2314CrossRefPubMedGoogle Scholar
  20. Coutant R, Maurey H, Rouleau S, Mathieu E, Mercier P, Limal JM, Le Bouil A (2003) Defect in epinephrine production in children with craniopharyngioma: functional or organic origin? Journal of Clincal Endocrinology and Metabolism 88:5969–5975Google Scholar
  21. Cushing H (1932) The craniopharyngiomas. In: Intracranial tumors- notes upon a series of two thousand verified cases with surgical mortality percentages thereto. Charles C Thomas, SpringfieldGoogle Scholar
  22. Daousi C, Dunn AJ, Foy PM, MacFarlane IA, Pinkney JH (2005) Endocrine and neuroanatomic features associated with weight gain and obesity in adult patients with hypothalamic damage. American Journal of Medicine 118:45–50Google Scholar
  23. de Vries L, Lazar L, Phillip M (2003) Craniopharyngioma: presentation and endocrine sequelae in 36 children. J Pediatr Endocrinol Metab 16(5):703–710CrossRefPubMedGoogle Scholar
  24. DeVile CJ, Grant DB, Hayward RD, Stanhope R (1996a) Growth and endocrine sequelae of craniopharyngioma. Arch Dis Child 75(2):108–114CrossRefPubMedPubMedCentralGoogle Scholar
  25. De Vile CJ, Grant DB, Kendall BE, Neville BG, Stanhope R, Watkins KE, Hayward RD (1996b) Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? Journal of Neurosurgery 85:73–81Google Scholar
  26. Donnet A, Schmitt A, Dufour H, Grisoli F (1999) Neuropsychological follow-up of twenty two adult patients after surgery for craniopharyngioma. Acta Neurochir (Wien) 141(10):1049–1054CrossRefGoogle Scholar
  27. Donovan JL, Nesbit GM (1996) Distinction of masses involving the sella and suprasellar space: specificity of imaging features. AJR Am J Roentgenol 167:597–603CrossRefPubMedGoogle Scholar
  28. Duff J, Meyer FB, Ilstrup DM, Laws ER Jr, Schleck CD, Scheithauer BW (2000) Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46(2):291–302CrossRefPubMedGoogle Scholar
  29. Einhaus SL, Sanford RA (1999) Craniopharyngiomas. In: Albright L, Pollack I, Adelson D (eds) Principles and practice of pediatric neurosurgery. Thieme, New YorkGoogle Scholar
  30. Elliott RE, Hsieh K, Hochm T, Belitskaya-Levy I, Wisoff J, Wisoff JH (2010) Efficacy and safety of radical resection of primary and recurrent craniopharyngiomas in 86 children. J Neurosurg Pediatr 5(1):30–48CrossRefPubMedGoogle Scholar
  31. Fahlbusch R, Honegger J, Paulus W, Huk W, Buchfelder M (1999) Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg 90(2):237–250CrossRefPubMedGoogle Scholar
  32. Fernandez-Miranda JC, Gardner PA, Snyderman CH, Devaney KO, Strojan P, Suárez C, Genden EM, Rinaldo A, Ferlito A (2012) Craniopharyngioma: a pathologic, clinical, and surgical review. Head Neck 34(7):1036–1044CrossRefPubMedGoogle Scholar
  33. Fischbein NJ, Dillon WP, Barkovich AJ (2000) Teaching atlas of brain imaging. Thieme, New YorkGoogle Scholar
  34. Gump JM, Donson AM, Birks DK, Amani VM, Rao KK, Griesinger AM, Kleinschmidt-De-Masters BK, Johnston JM, Anderson RCE, Rosenfeld A, Handler M, Gore L, Foreman N, Hankinson T (2015) Identification of targets for rational pharmacological therapy in childhood craniopharyngioma. Acta Neuropathol Commun 3:30CrossRefPubMedPubMedCentralGoogle Scholar
  35. Gunther JR, Sato M, Chintagumpala M, Ketonen L, Jones JY, Allen PK, Paulino AC, Okcu MF, Su JM, Weinberg J, Boehling NS, Khatua S, Adesina A, Dauser R, Whitehead WE, Mahajan A (2015) Imaging changes in pediatric intracranial ependymoma patients treated with proton beam radiation therapy compared to intensity modulated radiation therapy. Int J Radiat Oncol Biol Phys 93(1):54–63CrossRefPubMedGoogle Scholar
  36. Gupta K, Kuhn MJ, Shevlin DW, Wacaser LE (1999) Metastatic craniopharyngioma. AJNR Am J Neuroradiol 20(6):1059–1060PubMedGoogle Scholar
  37. Gurkaynak M, Ozyar E, Zorlu F, Akyol FH, Atahan IL (1994) Results of radiotherapy in craniopharyngiomas analyzed by the linear quadratic model. Acta Oncologica 33:941–943Google Scholar
  38. Habrand JL, Ganry O, Couanet D, Rouxel V, Levy-Piedbois C, Pierre-Kahn A, Kalifa C (1999) The role of radiation therapy in the management of craniopharyngioma: a 25-year experience and review of the literature. Int J Radiat Oncol Biol Phys 44(2):255–263CrossRefPubMedGoogle Scholar
  39. Harwood-Nash DC (1994) Neuroimaging of childhood craniopharyngioma. Pediatric Neurosurgery 21(Suppl 1):2–10Google Scholar
  40. Hetelekidis S, Barnes PD, Tao ML, Fischer EG, Schneider L, Scott RM, Tarbell NJ (1993) 20-year experience in childhood craniopharyngioma. Int J Radiat Oncol Biol Phys 27(2):189–195CrossRefPubMedGoogle Scholar
  41. Hoffman HJ (1994) Surgical management of craniopharyngioma. Pediatr Neurosurg 21(Suppl 1):44–49PubMedGoogle Scholar
  42. Hoffman HJ, Hendrick EB, Humphreys RP, Buncic JR, Armstrong DL, Jenkin RD (1977) Management of craniopharyngioma in children. J Neurosurg 47(2):218–227CrossRefPubMedGoogle Scholar
  43. Hoffman HJ, De Silva M, Humphreys RP, Drake JM, Smith ML, Blaser SI (1992) Aggressive surgical management of craniopharyngiomas in children. J Neurosurg 76(1):47–52CrossRefPubMedGoogle Scholar
  44. Hoffman HJ, Drake JM, Stapleton SR (1999) Craniopharyngiomas and pituitary tumors. In: Choux M, Di Rocco C, Hockley AD, Walker ML (eds) Pediatric neurosurgery. Churchill Livingstone, New YorkGoogle Scholar
  45. Hoffman BM, Kreutzer J, Saeger W, Buchfelder M, Blümcke I, Fahlbusch R, Buslei R (2006) Nuclear beta-catenin accumulation as reliable marker for the differentiation between cystic craniopharyngiomas and Rathke cleft cysts: a clinico-pathologic approach. American Journal of Surgical Pathology 30:1595–1603Google Scholar
  46. Holsken A, Gebhardt M, Buchfelder M, Fahlbusch R, Blumcke I, Buslei R (2011) EGFR signaling regulates tumor cell migration in craniopharyngiomas. Clin Cancer Res 17(13):4367–4377CrossRefPubMedGoogle Scholar
  47. Hölsken A, Kreutzer J, Hofmann BM, Hans V, Oppel F, Buchfelder M, Fahlbusch R, Blümcke I, Buslei R (2008) Target gene activation of the Wnt signaling pathway in nuclear beta-catenin accumulating cells of adamantinomatous craniopharyngiomas. Brain Pathology 19:357–364Google Scholar
  48. Honegger J, Barocka A, Sadri B, Fahlbusch R (1998) Neuropsychological results of craniopharyngiomasurgery in adults: a prospective study. Surgical Neurology 50:19–28; discussion 28–19Google Scholar
  49. Honegger J, Buchfelder M, Fahlbusch R (1999) Surgical treatment of craniopharyngiomas: endocrinological results. J Neurosurg 90(2):251–257Google Scholar
  50. Hussain I, Eloy JA, Carmel PW, Liu JK (2013) Molecular oncogenesis of craniopharyngioma: current and future strategies for the development of targeted therapies. J Neurosurg 119(1):106–112CrossRefPubMedGoogle Scholar
  51. Ierardi DF, Fernandes MJ, Silva IR, Thomazini-Gouveia J, Silva NS, Dastoli P, Toledo SR, Cavalheiro S (2007) Apoptosis in alpha interferon (IFN-alpha) intratumoral chemotherapy for cystic craniopharyngiomas. Childs Nerv Syst 23(9):1041–1046CrossRefPubMedGoogle Scholar
  52. Indelicato DJ, Flampouri S, Rotondo RL, Bradley JA, Morris CG, Aldana PR, Sandler E, Mendenhall NP (2014) Incidence and dosimetric parameters of pediatric brainstem toxicity following proton therapy. Acta Oncol 53(10):1298–1304CrossRefPubMedGoogle Scholar
  53. Inge TH, Pfluger P, Zeller M, Rose SR, Burget L, Sundararajan S, Daniels SR, Tschöp MH (2007) Gastric bypass surgery for treatment of hypothalamic obesity after craniopharyngioma therapy. Nature Clinical Practice. Endocrinology & Metabolism 3:606–609Google Scholar
  54. Israel ZH, Pomeranz S (1995) Intracranial craniopharyngioma seeding following radical resection. Pediatr Neurosurg 22(4):210–213CrossRefPubMedGoogle Scholar
  55. Ito M, Jamshidi J, Yamanaka K (2001) Does craniopharyngioma metastasize? Case report and review of the literature. Neurosurgery 48(4):933–935PubMedGoogle Scholar
  56. Jakacki RI, Cohen BH, Jamison C, Mathews VP, Arenson E, Longee DC, Hilden J, Cornelius A, Needle M, Heilman D, Boaz JC, Luerssen TG (2000) Phase II evaluation of interferon-alpha-2a for progressive or recurrent craniopharyngiomas. J Neurosurg 92:255–260CrossRefPubMedGoogle Scholar
  57. Kalapurakal JA, Goldman S, Hsieh YC, Tomita T, Marymont MH (2000) Clinical outcome in children with recurrent craniopharyngioma after primary surgery. Cancer J 6(6):388–393PubMedGoogle Scholar
  58. Kato K, Nakatani Y, Kanno H, Inayama Y, Ijiri R, Nagahara N, Miyake T, Tanaka M, Ito Y, Aida N, Tachibana K, Sekido K, Tanaka Y (2004) Possible linkage between specific histological structures and aberrant reactivation of the Wnt pathway in adamantinomatous craniopharyngioma. J Pathol 203(3):814–821CrossRefPubMedGoogle Scholar
  59. Khoo LT, Flagel J, Liker M, Levy M (2001) Craniopharyngiomas: surgical management. In: Petrovich P, Brady LW, Apuzzo ML, Bamberg M (eds) Combined modality therapy of central nervous system tumors. Springer, Berlin, pp 187–214Google Scholar
  60. Kobayashi T, Tanaka T, Kida Y (1994) Stereotactic gamma radiosurgery of craniopharyngiomas. Pediatr Neurosurg 21(Suppl 1):69–74PubMedGoogle Scholar
  61. Koutourousiou M, Gardner PA, Fernandez-Miranda JC, Tyler-Kabara EC, Wang EW, Snyderman CH (2013) Endoscopic endonasal surgery for craniopharyngiomas: surgical outcome in 64 patients. J Neurosurg 119(5):1194–1207CrossRefPubMedGoogle Scholar
  62. Kristopaitis T, Thomas C, Petruzzelli GJ, Lee JM (2000) Malignant craniopharyngioma. Arch Pathol Lab Med 124(9):1356–1360Google Scholar
  63. Laws ER (1994) Transsphenoidal removal of craniopharyngioma. Pediatr Neurosurg 21(Suppl 1):57–63PubMedGoogle Scholar
  64. Leber KA, Bergloff J, Pendl G (1998) Dose-response tolerance of the visual pathways and cranial nerves of the cavernous sinus to stereotactic radiosurgery. J Neurosurg 88(1):43–50CrossRefPubMedGoogle Scholar
  65. Lee HC, Curry DL, Stern JS (1989) Direct effect of CNS on insulin hypersecretion in obese Zucker rats: involvement of vagus nerve. American Journal of Physiology 256:E439–E444Google Scholar
  66. Lee JH, Kim CY, Kim DG, Jung HW (1999) Postoperative ectopic seeding of craniopharyngioma. Case illustration. J Neurosurg 90(4):796Google Scholar
  67. Leksell L, Lidén K (1952) A therapeutic trial with radioactive isotope in cystic brain tumor: radioisotope techniques I. Medical and Physiological Application 1:1–4Google Scholar
  68. Li K, Lu X, Yang N, Zheng J, Huang B, Li L (2015) Association of pituitary stalk management with endocrine outcomes and recurrence in microsurgery of craniopharyngiomas: a meta-analysis. Clin Neurol Neurosurg 136:20–24Google Scholar
  69. Lin LL, El Naqa I, Leonard JR, Park TS, Hollander AS, Michalski JM, Mansur DB (2008) Long-term outcome in children treated for craniopharyngioma with and without radiotherapy. Journal of Neurosurgery Pediatrics 1:126–130Google Scholar
  70. Linnert M, Gehl J (2009) Bleomycin treatment of brain tumors: an evaluation. Anticancer Drugs 20(3):157–164CrossRefPubMedGoogle Scholar
  71. Lunsford LD, Pollock BE, Kondziolka DS, Levine G, Flickinger JC (1994) Stereotactic options in the management of craniopharyngioma. Pediatr Neurosurg 21(Suppl 1):90–97PubMedGoogle Scholar
  72. Lustig RH (2002) Hypothalamic obesity: the sixth cranial endocrinopahty. The Endocrinologist 12:210–217Google Scholar
  73. Lustig RH (2007) The efferent arm of the energy balance regulatory pathway: neuroendocrinology and pathology. In: Donahoue PA (ed) Obesity and energy metabolism: research and clinical applications. Humana, Totowa, NJ, pp 69–86Google Scholar
  74. Lustig RH (2008) Hypothalamic obesity: causes, consequences, treatment. Pediatr Endocrinol Rev 6(2):220–227PubMedGoogle Scholar
  75. Lustig RH, Post SR, Srivannaboon K, Rose SR, Danish RK, Burghen GA, . . . Merchant TE (2003) Risk factors for the development of obesity in children surviving brain tumors. J Clin Endocrinol Metab 88(2):611–616Google Scholar
  76. Lustig RH, Rose SR, Burghen GA, Velasquez-Mieyer P, Broome DC, Smith K, Li H, Hudson MM,Heideman RL, Kun LE (1999) Hypothalamic obesity caused by cranial insult in children: altered glucose and insulin dynamics and reversal by a somatostatin agonist. Journal of Pediatrics 135:162–168Google Scholar
  77. Luu QT, Loredo LN, Archambeau JO, Yonemoto LT, Slater JM, Slater JD (2006) Fractionated proton radiation treatment for pediatric craniopharyngioma: preliminary report. Cancer J 12(2):155–159Google Scholar
  78. Malik JM, Cosgrove GR, VandenBerg SR (1992) Remote recurrence of craniopharyngioma in the epidural space. Case report. J Neurosurg 77(5):804–807Google Scholar
  79. Mason PW, Krawiecki N, Meacham LR (2002) The use of dextroamphetamine to treat obesity and hyperphagia in children treated for craniopharyngioma. Archives of Pediatric and Adolescent Medicine 156:887–892 Google Scholar
  80. Matsuo M, Yonemitsu N, Zaitsu M, Ishii K, Hamasaki Y, Fukutama K et al (2001) Expression of prostaglandin H synthetase-2 in human brain tumors. Acta Neuopathol, 102(2):181–187Google Scholar
  81. May JA, Krieger MD, Bowen I, Geffner ME (2006) Craniopharyngioma in childhood. Adv Pediatr 53:183–209Google Scholar
  82. Merchant TE, Kiehna EN, Sanford RA, Mulhern RK, Thompson SJ, Wilson MW, . . . Kun LE (2002) Craniopharyngioma: the St. Jude Children’s Research Hospital experience 1984–2001. Int J Radiat Oncol Biol Phys 53(3):533–542Google Scholar
  83. Merchant TE, Kun LE, Hua CH, Wu S, Xiong X, Sanford RA, Boop FA (2013) Disease control after reduced volume conformal and intensity modulated radiation therapy for childhood craniopharyngioma. Int J Radiat Oncol Biol Phys 85(4):e187–e192CrossRefPubMedGoogle Scholar
  84. Miller DC (1994) Pathology of craniopharyngiomas: clinical import of pathological findings. Pediatr Neurosurg 21(Suppl 1):11–17PubMedGoogle Scholar
  85. Minniti G, Saran F, Traish D, Soomal R, Sardell S, Gonsalves A, . . . Brada M (2007) Fractionated stereotactic conformal radiotherapy following conservative surgery in the control of craniopharyngiomas. Radiother Oncol 82(1):90–95Google Scholar
  86. Mokry M (1999) Craniopharyngiomas: a six year experience with Gamma Knife radiosurgery. Stereotact Funct Neurosurg 72(Suppl 1):140–149CrossRefPubMedGoogle Scholar
  87. Moore K, Couldwell WT (2000) Craniopharyngioma. In: Bernstein M, Berger MS (eds) Neuro-oncology: the essentials. Thieme, New YorkGoogle Scholar
  88. Muller HL (2014) Craniopharyngioma. Endocr Rev 35(3):513–543CrossRefPubMedGoogle Scholar
  89. Muller HL, Gebhardt U, Etavard-Gorris N, Korenke E, Warmuth-Metz M, Kolb R, . . . Calaminus G (2004) Prognosis and sequela in patients with childhood craniopharyngioma – results of HIT-ENDO and update on KRANIOPHARYNGEOM 2000. Klin Padiatr 216(6):43–348Google Scholar
  90. Norris JS, Pavaresh M, Afshar F (1998) Primary transsphenoidal microsurgery in the treatment of craniopharyngiomas. Br J Neurosurg 12(4):305–312CrossRefPubMedGoogle Scholar
  91. Nozaki M1, Tada M, Matsumoto R, Sawamura Y, Abe H, Iggo RD (1998) Rare occurrence of inactivating p53 gene mutations in primary non-astrocytic tumors of the central nervous system: reappraisal by yeast functional assay. Acta Neuropathol 95(3):291–6Google Scholar
  92. Ostrom QT, Gittleman H, Fulop J, Liu M, Blanda R, Kromer C, Wolinsky Y, Kruchko C, Barnholtz-Sloan JS (2015) CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2008–2012. Neuro Oncol 17(Suppl 4):iv1–iv62CrossRefPubMedGoogle Scholar
  93. Pekmezci M, Louie J, Gupta N, Bloomer MM, Tihan T (2010) Clinicopathological characteristics of adamantinomatous and papillary craniopharyngiomas: University of California, San Francisco experience 1985–2005. Neurosurgery 67(5):1341–1349CrossRefPubMedGoogle Scholar
  94. Pierre-Kahn A, Sainte-Rose C, Renier D (1994) Surgical approach to children with craniopharyngiomas and severely impaired vision: special considerations. Pediatr Neurosurg 21(Suppl 1):50–56PubMedGoogle Scholar
  95. Pollock BE, Lunsford LD, Kondziolka D, Levine G, Flickinger JC (1995) Phosphorus-32 intracavitary irradiation of cystic craniopharyngiomas: current technique and long-term results. Int J Radiat Oncol Biol Phys 33:437–446CrossRefPubMedGoogle Scholar
  96. Poretti A, Grotzer MA, Ribi K, Schonle E, Boltshauser E (2004) Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev Med Child Neurol 46(4):220–229CrossRefPubMedGoogle Scholar
  97. Prabhu VC, Brown HG (2005) The pathogenesis of craniopharyngiomas. Childs Nerv Syst 21(8–9):622–627CrossRefPubMedGoogle Scholar
  98. Preeyasombat C, Bacchetti P, Lazar AA, Lustig RH (2005) Racial and etiopathologic dichotomies in insulin secretion and resistance in obese children. Journal of Pediatrics 146:474–481Google Scholar
  99. Prieto R, Pascual JM (2013) Craniopharyngiomas with a mixed histological pattern: the missing link to the intriguing pathogenesis of adamantinomatous and squamous-papillary varieties? Neuropathology 33(6):682–686Google Scholar
  100. Puget S, Garnett M, Wray A, Grill J, Habrand JL, Bodaert N, Zerah M, Bezerra M, Renier D, Pierre-Kahn A, Sainte-Rose C (2007) Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. Journal of Neurosurgery 106:3–12Google Scholar
  101. Raghavan R, Dickey WT Jr, Margraf LR, White CL 3rd, Coimbra C, Hynan LS, Rushing EJ (2000) Proliferative activity in craniopharyngiomas: clinicopathological correlations in adults and children. Surg Neurol 54:241–247Google Scholar
  102. Ragoowansi AT, Piepgras DG (1991) Postoperative ectopic craniopharyngioma. Case report. J Neurosurg 74(4):653–655CrossRefPubMedGoogle Scholar
  103. Regine WF, Mohiuddin M, Kramer S (1993) Long-term results of pediatric and adult craniopharyngiomas treated with combined surgery and radiation. Radiother Oncol 27(1):13–21CrossRefPubMedGoogle Scholar
  104. Rienstein S, Adams EF, Pilzer D, Goldring AA, Goldman B, Friedman E (2003) Comparative genomic hybridization analysis of craniopharyngiomas. J Neurosurg 98(1):162–164CrossRefPubMedGoogle Scholar
  105. Rilliet B, de Paul Djientcheu V, Vernet O, Montes J, Farmer JP, Bertrand G (1999) Craniopharyngiomas, results in children and adolescents operated through a transsphenoidal approach compared with an intracranial approach. Front Radiat Ther Oncol 33:114–122CrossRefPubMedGoogle Scholar
  106. Riva D, Pantaleoni C, Devoti M, Saletti V, Nichelli F, Giorgi C (1998) Late neuropsychological and behavioural outcome of children surgically treated for craniopharyngioma. Childs Nerv Syst 14(4–5):179–184CrossRefPubMedGoogle Scholar
  107. Rose SR, Lustig RH, Pitukcheewanont P, Broome DC, Burghen GA, Li H, Hudson MM, Kun LE, Heideman RL (1999) Diagnosis of hidden central hypothyroidism in survivors of childhood cancer. J Clin Endocrinol Metab 84(12):4472–4479PubMedGoogle Scholar
  108. Rose SR, Lustig RH, Pitukcheewanont P, Broome DC, Burghen GA, Li H, Hudson MM, Kun LE, Heideman RL (1999b) Hidden central hypothyroidism in survivors of childhood cancer. Journal of Clincal Endocrinology and Metabolism 84:4472–4479Google Scholar
  109. Rose SR, Danish RK, Kearney NS, Schreiber RE, Lustig RH, Burghen GA, Hudson MM (2005) ACTH deficiency in childhood cancer survivors. Pediatr Blood Cancer 45(6):808–813CrossRefPubMedGoogle Scholar
  110. Samii M, Tatagiba M (1997) Surgical management of craniopharyngiomas: a review. Neurol Med Chir (Tokyo) 37(2):141–149CrossRefGoogle Scholar
  111. Sanford RA (1994) Craniopharyngioma: results of survey of the American Society of Pediatric Neurosurgery. Pediatr Neurosurg 21(Suppl 1):39–43PubMedGoogle Scholar
  112. Sanford RA, Muhlbauer MS (1991) Craniopharyngioma in children. Neurol Clin 9(2):453–465PubMedGoogle Scholar
  113. Sarubi JC, Bei H, Adams EF, Boson WL, Friedman E, Brandão K, Kalapothakis E, Miranda D, Valle FL, Sarquis MS, De Marco L (2001) Clonal composition of human adamantinomatous craniopharyngiomas and somatic mutation analyses of the patched (PTCH), Gsalpha and Gi2alpha genes. Neurosci Lett 310(1):5–8, Neurosci Lett. 310(1):5–8CrossRefPubMedGoogle Scholar
  114. Schofl C, Schleth A, Berger D, Terkamp C, Von Zur Muhlen A, Brabant G (2002) Sympathoadrenal counterregulation in patients with hypothalamic craniopharyngioma. Journal of Clincal Endocrinology and Metabolism 87: 624–629Google Scholar
  115. Shi XE, Wu B, Fan T, Zhou ZQ, Zhang YL (2008) Craniopharyngioma: surgical experience of 309 cases in China. Clin Neurol Neurosurg 110(2):151–159CrossRefPubMedGoogle Scholar
  116. Sidawy MK, Jannotta FS (1997) Intraoperative cytologic diagnosis of lesions of the central nervous system. Am J Clin Pathol 108:S56–S66PubMedGoogle Scholar
  117. Smith D, Finucane F, Phillips J, Baylis PH, Finucane J, Tormey W, Thompson CJ (2004) Abnormal regulation of thirst and vasopressin secretion following surgery for craniopharyngioma. Clinical Endocrinology 61:273–279Google Scholar
  118. Srinivasan S, Ogle GD, Garnett SP, Briody JN, Lee JW, Cowell CT (2004) Features of the metabolic syndrome after craniopharyngioma. Journal of Clinical Endocrinology and Metabolism 89:81–86Google Scholar
  119. Stafford SL, Pollock BE, Leavitt JA, Foote RL, Brown PD, Link MJ, Gorman DA, Schomberg PJ (2003) A study on the radiation tolerance of the optic nerves and chiasm after stereotactic radiosurgery. Int J Radiat Oncol Biol Phys 55(5):1177–1181Google Scholar
  120. Steinbok P, Hukin J (2010) Intracystic treatments for craniopharyngioma. Neurosurg Focus 28(4):E13CrossRefPubMedGoogle Scholar
  121. Sterkenberg AS, Hofmann A, Gebhardt U, Warmuth-Metz M, Daubenbüchel AM, Muller HL (2015) Survival, hypothalamic obesity, and neuropsychologic/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes. Neuro Oncol 17(7):1029–1038CrossRefGoogle Scholar
  122. Stripp DC, Maity A, Janss AJ, Belasco JB, Tochner ZA, Goldwein JW, Moshang T, Rorke LB, Phillips PC, Sutton LN, Shu HK (2004) Surgery with or without radiation therapy in the management of craniopharyngiomas in children and young adults. Int J Radiat Oncol Biol Phys 58(3):714–720CrossRefPubMedGoogle Scholar
  123. Suit HD, Goitein M, Tepper J, Koehler AM, Schmidt RA, Schneider R (1975) Exploratory study of proton radiation therapy using large field techniques and fractionated dose schedules. Cancer 35(6):1646–1657CrossRefPubMedGoogle Scholar
  124. Takahashi H, Nakazawa S, Shimura T (1985) Evaluation of postoperative intratumoral injection of bleomycin for craniopharyngioma in children. J Neurosurg 62(1):120–127CrossRefPubMedGoogle Scholar
  125. Takamaru KI, Ohmitsu M, Li FQ (2008) An oncogenic hub: beta-catenin as a molecular target for cancer therapeutics. Handbook of Experimental Pharmacology 186:261–284Google Scholar
  126. Thapar K, Stefaneanu L, Kovacs K, Scheithauer BW, Ricardo VL, Muller PJ, Laws ER (1994) Estrogen receptor gene expression in craniopharyngiomas: an in situ hybridization study. Neurosurgery 35:1012–1017CrossRefPubMedGoogle Scholar
  127. Tomita T, McLone DG (1993) Radical resections of childhood craniopharyngiomas. Pediatr Neurosurg 19(1):6–14CrossRefPubMedGoogle Scholar
  128. Van Effenterre R, Boch AL (2002) Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97(1):3–11CrossRefPubMedGoogle Scholar
  129. Veeravagu A, Lee M, Jiang B, Chang SD (2010) The role of radiosurgery in the treatment of craniopharyngiomas. Neurosurg Focus d28(4):E11CrossRefGoogle Scholar
  130. Vidal S, Kovacs K, Lloyd RV, Meyer FB, Scheithauer BW (2002) Angiogenesis in patients wtih craniopharyngiomas: correlation with treatment and outcome. Cancer 94(3):738–745Google Scholar
  131. Villani RM, Tomei G, Bello L, Sganzerla E, Ambrosi B, Re T, Giovanelli Barilari M (1997) Long-term results of treatment for craniopharyngioma in children. Childs Nerv Syst 13(7):397–405Google Scholar
  132. Voges J, Sturm V, Lehrke R, Treuer H, Gauss C, Berthold F (1997) Cystic craniopharyngioma: long-term results after intracavitary irradiation with stereotactically applied colloidal beta emitting radioactive sources. Neurosurgery 40:263–269CrossRefPubMedGoogle Scholar
  133. Weiner HL, Wisoff JH, Rosenberg ME, Kupersmith MJ, Cohen H, Zagzag D, Shiminski-Maher T, Flamm ES, Epstein FJ, Miller DC (1994) Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery 35(6):1001–1010CrossRefPubMedGoogle Scholar
  134. Weiss M, Sutton L, Marcial V, Fowble B, Packer R, Zimmerman R, Schut L, Bruce D, D’Angio G (1989) The role of radiation therapy in the management of childhood craniopharyngioma. Int J Radiat Oncol Biol Phys 17(6):1313–1321CrossRefPubMedGoogle Scholar
  135. Wilson JD, Foster DW, Kronenberg HM, Larsen PR (1998) Williams textbook of endocrinology. W.B. Saunders, PhiladelphiaGoogle Scholar
  136. Winkfield KM, Linsenmeier C, Yock TI, Grant PE, Yeap BY, Butler WE, Tarbell NJ (2009) Surveillance of craniopharyngioma cyst growth in children treated with proton radiotherapy. Int J Radiat Oncol Biol Phys 73(3):716–721CrossRefPubMedGoogle Scholar
  137. Wisoff J, Donahue B (2015) Craniopharyngiomas. In: Albright AL, Pollack IF, Adelson PD (eds) Principles and practice of pediatric neurosurgery, 3rd edn. Thieme, New YorkGoogle Scholar
  138. Yasargil MG, Curcic M, Kis M, Siegenthaler G, Teddy PJ, Roth P (1990) Total removal of craniopharyngiomas. Approaches and long-term results in 144 patients. J Neurosurg 73(1):3–11CrossRefPubMedGoogle Scholar
  139. Yeung JT, Pollack IF, Panigrahy A, Jakacki RI (2012) Pegylated interferon-α-2b for children with recurrent craniopharyngioma. J Neurosurg Pediatr 10:498–503CrossRefPubMedGoogle Scholar
  140. Yu X, Liu Z, Li S (2000) Combined treatment with stereotactic intracavitary irradiation and gamma knife surgery for craniopharyngiomas. Stereotact Funct Neurosurg 75(2–3):117–122PubMedGoogle Scholar
  141. Yuen KC, Koltowska-Häggström M, Cook DM, Fox JL, Jönsson PJ, Geffner ME, Abs R (2013) Clinical characteristics and effects of GH replacement therapy in adults with childhood-onset craniopharyngioma compared with those in adults with other causes of childhood-onset hypothalamic-pituitary dysfunction. Eur J Endocrinol 169:511–519CrossRefPubMedGoogle Scholar
  142. Yuen KC, Koltowska-Häggström M, Cook DM, Fox JL, Jönsson PJ, Geffner ME, Abs R (2014) Primary treatment regimen and diabetes insipidus as predictors of health outcomes in adults with childhood-onset craniopharyngioma. J Clin Endocrinol Metab 99(4):1227–1235CrossRefPubMedGoogle Scholar
  143. Zhou ZQ, Shi XE (2004) Changes of hypothalamus-pituitary hormones in patients after total removal of craniopharyngiomas. Chin Med J (Engl) 117(3):357–360Google Scholar
  144. Zuccaro G (2005) Radical resection of craniopharyngioma. Childs Nerv Syst 21(8–9):679–690CrossRefPubMedGoogle Scholar
  145. Zuccaro G, Jaimovich R, Mantese B, Monges J (1996) Complications in paediatric craniopharyngioma treatment. Childs Nerv Syst 12(7):385–390CrossRefPubMedGoogle Scholar
  146. Zuhur SS, Müslüman AM, Tanık C, Karaman O, Oztürk FY, Ozderya A, Ozkayalar H, Aydın Y, Altuntaş Y (2011) MGMT immunoexpression in adamantinomatous craniopharyngiomas. Pituitary 14(4):323–327CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing 2017

Authors and Affiliations

  • Lauren Ostling
    • 1
    • 2
  • Daphne A. Haas-Kogan
    • 3
  • Robert H. Lustig
    • 4
    • 5
  • Nalin Gupta
    • 6
    Email author
  1. 1.Department of Neurological SurgeryUniversity of California San FranciscoSan FranciscoUSA
  2. 2.Department of Neurological SurgeryUCSF Benioff Children’s Hospital OaklandOaklandUSA
  3. 3.Department of Radiation OncologyBrigham and Women’s Hospital, Dana-Farber Cancer Institute and Boston Children’s HospitalBostonUSA
  4. 4.Department of Pediatrics & Institute for Health Policy StudiesUniversity of CaliforniaSan FranciscoUSA
  5. 5.UCSF Benioff Children’s Hospital San FranciscoSan FranciscoUSA
  6. 6.Division of Pediatric Neurosurgery, Departments of Neurological Surgery and PediatricsUniversity of California San Francisco & UCSF Benioff Children’s HospitalSan FranciscoUSA

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