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Surgical Treatment of Human ACP

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Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma

Abstract

Adamantinomatous craniopharyngiomas are complex cystic-solid lesions, typically located within the suprasellar cistern. Their growth causes symptoms by pressure and distortion of the optic nerves and chiasm, the hypothalamus and the pituitary gland. Involvement of the third ventricle causes hydrocephalus. Initial management is surgical and directed towards preservation of vision and control of raised intracranial pressure. Further surgical management is dictated by the extent of hypothalamic involvement, evident on clinical presentation and magnetic resonance imaging. Limited surgery, followed by radiotherapy, is as effective at long-term local control as aggressive surgery aimed at complete resection. The former approach is superior at protecting hypothalamic function and is preferable whenever hypothalamic involvement by the tumour is significant.

Surgery may be carried out transcranially or transsphenoidally. The development of endoscopic transsphenoidal surgery has allowed larger and higher craniopharyngiomas to be resected safely through this route. The management of recurrent craniopharyngiomas remains challenging and may include revisional surgery or administration of interferon into cystic components.

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References

  1. Barkhoudarian G, Laws ER (2013) Craniopharyngioma: history. Pituitary 16:1–8

    Article  PubMed  Google Scholar 

  2. Muller HL (2008) Childhood craniopharyngioma. Horm Res 69:193–202

    PubMed  Google Scholar 

  3. Ahmet A, Blaser S, Stephens D, Guger S, Rutkas JT, Hamilton J (2006) Weight gain in craniopharyngioma--a model for hypothalamic obesity. J Pediatr Endocrinol Metab 19:121–127

    Article  PubMed  Google Scholar 

  4. Elliott RE, Wisoff JH (2010) Surgical management of giant pediatric craniopharyngiomas. J Neurosurg Pediatr 6:403–416

    Article  PubMed  Google Scholar 

  5. Mortini P, Gagliardi F, Bailo M, Spina A, Parlangeli A, Falini A, Losa M (2016) Magnetic resonance imaging as predictor of functional outcome in craniopharyngiomas. Endocrine 51:148–162

    Article  CAS  PubMed  Google Scholar 

  6. Müller HL (2014) Childhood craniopharyngioma: treatment strategies and outcomes. Expert Rev Neurother 14:187–197

    Article  PubMed  Google Scholar 

  7. DeVile CJ, Grant DB, Hayward RD, Stanhope R (1996) Growth and endocrine sequelae of craniopharyngioma. Arch Dis Child 75:108–114

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. De Vile CJ, Grant DB, Hayward RD, Kendall BE, Neville BG, Stanhope R (1996) Obesity in childhood craniopharyngioma: relation to post-operative hypothalamic damage shown by magnetic resonance imaging. J Clin Endocrinol Metab 81:2734–2737

    CAS  PubMed  Google Scholar 

  9. Puget S, Garnett M, Wray A et al (2007) Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg 106:3–12

    PubMed  Google Scholar 

  10. Van Gompel JJ, Nippoldt TB, Higgins DM, Meyer FB (2010) Magnetic resonance imaging-graded hypothalamic compression in surgically treated adult craniopharyngiomas determining postoperative obesity. Neurosurg Focus 28:E3

    Article  PubMed  Google Scholar 

  11. Müller HL (2015) Preoperative staging in childhood craniopharyngioma: standardization as a first step towards improved outcome. Endocrine. doi:10.1007/s12020-015-0800-x

    PubMed  Google Scholar 

  12. Müller HL, Gebhardt U, Teske C et al (2011) 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

    Article  PubMed  Google Scholar 

  13. Šteňo J, Bízik I, Šteňo A, Matejčík V (2011) Craniopharyngiomas in children: how radical should the surgeon be? Childs Nerv Syst 27:41–54

    Article  PubMed  Google Scholar 

  14. Mallucci C, Pizer B, Blair J, Didi M, Doss A, Upadrasta S, Newman W, Avula S, Pettorini B (2012) 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

    Article  PubMed  Google Scholar 

  15. Pettorini BL, Tamburrini G, Massimi L, Caldarelli M, Di Rocco C (2009) Endoscopic transventricular positioning of intracystic catheter for treatment of craniopharyngioma. Technical note. J Neurosurg Pediatr 4:245–248

    Article  PubMed  Google Scholar 

  16. Margetis K, Souweidane MM (2013) Endoscopic treatment of intraventricular cystic tumors. World Neurosurg 79:S19.e1–S19.11

    Article  Google Scholar 

  17. Merchant TE, Kun LE, Hua C-H, 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:e187–e192

    Article  PubMed  Google Scholar 

  18. Hoffman HJ, De Silva M, Humphreys RP, Drake JM, Smith ML, Blaser SI (1992) Aggressive surgical management of craniopharyngiomas in children. J Neurosurg 76:47–52

    Article  CAS  PubMed  Google Scholar 

  19. 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 21:747–757

    Article  CAS  PubMed  Google Scholar 

  20. 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 35:1001–10 discussion 1010–1

    Google Scholar 

  21. De Vile CJ, Grant DB, Kendall BE, Neville BG, Stanhope R, Watkins KE, Hayward RD (1996) Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? J Neurosurg 85:73–81

    Article  CAS  PubMed  Google Scholar 

  22. Sainte-Rose C, Puget S, Wray A, Zerah M, Grill J, Brauner R, Boddaert N, Pierre-Kahn A (2005) Craniopharyngioma: the pendulum of surgical management. Childs Nerv Syst 21:691–695

    Article  PubMed  Google Scholar 

  23. Yaşargil 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 73:3–11

    Article  PubMed  Google Scholar 

  24. Fischer EG, Welch K, Shillito J, Winston KR, Tarbell NJ (1990) Craniopharyngiomas in children. Long-term effects of conservative surgical procedures combined with radiation therapy. J Neurosurg 73:534–540

    Article  CAS  PubMed  Google Scholar 

  25. Elliott RE, Sands SA, Strom RG, Wisoff JH (2010) Craniopharyngioma Clinical Status Scale: a standardized metric of preoperative function and posttreatment outcome. Neurosurg Focus 28:E2

    Article  PubMed  Google Scholar 

  26. Müller HL (2016) Risk-adapted treatment and follow-up management in childhood-onset craniopharyngioma. Expert Rev Neurother. doi:10.1586/14737175.2016.1166959

    Google Scholar 

  27. Kassam AB, Gardner PA, Snyderman CH, Carrau RL, Mintz AH, Prevedello DM (2008) Expanded endonasal approach, a fully endoscopic transnasal approach for the resection of midline suprasellar craniopharyngiomas: a new classification based on the infundibulum. J Neurosurg 108:715–728

    Article  PubMed  Google Scholar 

  28. Fahlbusch R, Honegger J, Paulus W, Huk W, Buchfelder M (1999) Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg 90:237–250

    Article  CAS  PubMed  Google Scholar 

  29. Gerganov V, Metwali H, Samii A, Fahlbusch R, Samii M (2014) Microsurgical resection of extensive craniopharyngiomas using a frontolateral approach: operative technique and outcome. J Neurosurg 120:559–570

    Article  PubMed  Google Scholar 

  30. Chamoun R, Couldwell WT (2013) Transcortical-transforaminal microscopic approach for purely intraventricular craniopharyngioma. Neurosurg Focus 34:Video 4

    Article  PubMed  Google Scholar 

  31. Milligan BD, Meyer FB (2010) Morbidity of transcallosal and transcortical approaches to lesions in and around the lateral and third ventricles: a single-institution experience. Neurosurgery 67:1483–1496

    Article  PubMed  Google Scholar 

  32. Patel P, Cohen-Gadol AA, Boop F, Klimo P (2014) Technical strategies for the transcallosal transforaminal approach to third ventricle tumors: expanding the operative corridor. J Neurosurg Pediatr 14:365–371

    Article  PubMed  Google Scholar 

  33. Laws ER (2010) Surgical outcome in 90 patients with craniopharyngiomas: an evaluation of the transsphenoidal approach. World Neurosurg 74:254–255

    Article  PubMed  Google Scholar 

  34. Komotar RJ, Starke RM, Raper DMS, Anand VK, Schwartz TH (2012) Endoscopic endonasal compared with microscopic transsphenoidal and open transcranial resection of craniopharyngiomas. World Neurosurg 77:329–341

    Article  PubMed  Google Scholar 

  35. Elliott RE, Jane JA, Wisoff JH (2011) Surgical management of craniopharyngiomas in children: meta-analysis and comparison of transcranial and transsphenoidal approaches. Neurosurgery 69:630–643 discussion 643

    Article  PubMed  Google Scholar 

  36. Ikeda H, Gotoh H, Watanabe K (2012) Outcome of endoscopy-assisted microscopic extended transsphenoidal surgery for suprasellar adult craniopharyngiomas. Front Endocrinol (Lausanne) 3:25

    Google Scholar 

  37. Laws ER (1997) Craniopharyngioma: transsphenoidal surgery. Curr Ther Endocrinol Metab 6:35–38

    PubMed  Google Scholar 

  38. Kenning TJ, Beahm DD, Farrell CJ, Schaberg MR, Rosen MR, Evans JJ (2012) Endoscopic endonasal craniopharyngioma resection. J Neurosurg 32(Suppl):E5

    PubMed  Google Scholar 

  39. Yang I, Sughrue ME, Rutkowski MJ, Kaur R, Ivan ME, Aranda D, Barani IJ, Parsa AT (2010) Craniopharyngioma: a comparison of tumor control with various treatment strategies. Neurosurg Focus 28:E5

    Article  PubMed  Google Scholar 

  40. 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 29:231–238

    Article  PubMed  Google Scholar 

  41. Iannalfi A, Fragkandrea I, Brock J, Saran F (2013) Radiotherapy in craniopharyngiomas. Clin Oncol (R Coll Radiol) 25:654–667

    Article  CAS  Google Scholar 

  42. Cage TA, Clark AJ, Aranda D, Gupta N, Sun PP, Parsa AT, Auguste KI (2013) A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas. J Neurosurg Pediatr 11:673–681

    Article  PubMed  Google Scholar 

  43. Sughrue ME, Yang I, Kane AJ, Fang S, Clark AJ, Aranda D, Barani IJ, Parsa AT (2011) Endocrinologic, neurologic, and visual morbidity after treatment for craniopharyngioma. J Neuro-Oncol 101:463–476

    Article  Google Scholar 

  44. Müller HL, Gebhardt U, Schröder S et al (2010) Analyses of treatment variables for patients with childhood craniopharyngioma--results of the multicenter prospective trial KRANIOPHARYNGEOM 2000 after three years of follow-up. Horm Res Paediatr 73:175–180

    Article  PubMed  Google Scholar 

  45. Hoffmann A, Warmth-Metz M, Gebhardt U, Pietsch T, Pohl F, Kortmann R-D, Calaminus G, Müller HL (2014) Childhood craniopharyngioma – changes of treatment strategies in the trials KRANIOPHARYNGEOM 2000/2007. Klin Padiatr 226:161–168

    Article  CAS  PubMed  Google Scholar 

  46. Elowe-Gruau E, Beltrand J, Brauner R et al (2013) Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity. J Clin Endocrinol Metab 98:2376–2382

    Article  CAS  PubMed  Google Scholar 

  47. Cohen M, Bartels U, Branson H, Kulkarni AV, Hamilton J (2013) Trends in treatment and outcomes of pediatric craniopharyngioma, 1975–2011. Neuro-Oncology 15:767–774

    Article  PubMed  PubMed Central  Google Scholar 

  48. Schoenfeld A, Pekmezci M, Barnes MJ et al (2012) The superiority of conservative resection and adjuvant radiation for craniopharyngiomas. J Neuro-Oncol 108:133–139

    Article  Google Scholar 

  49. Müller HL (2014) Craniopharyngioma. Endocr Rev 35:513–543

    Article  PubMed  Google Scholar 

  50. Laws ER, Trautmann JC, Hollenhorst RW (1977) Transsphenoidal decompression of the optic nerve and chiasm. Visual results in 62 patients. J Neurosurg 46:717–722

    Article  PubMed  Google Scholar 

  51. Poretti A, Grotzer MA, Ribi K, Schönle E, Boltshauser E (2004) Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev Med Child Neurol 46:220–229

    Article  PubMed  Google Scholar 

  52. Sutton LN (1994) Vascular complications of surgery for craniopharyngioma and hypothalamic glioma. Pediatr Neurosurg 21(Suppl 1):124–128

    PubMed  Google Scholar 

  53. 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 5:30–48

    Article  PubMed  Google Scholar 

  54. Müller HL (2015) Childhood craniopharyngioma – current status and recent perspectives in diagnostics and treatment. J Pediatr Endocrinol Metab 28:1–2

    Article  PubMed  Google Scholar 

  55. Pierre-Kahn A, Recassens C, Pinto G, Thalassinos C, Chokron S, Soubervielle JC, Brauner R, Zerah M, Sainte-Rose C (2005) Social and psycho-intellectual outcome following radical removal of craniopharyngiomas in childhood. A prospective series. Childs Nerv Syst 21:817–824

    Article  CAS  PubMed  Google Scholar 

  56. Müller HL (2016) Craniopharyngioma and hypothalamic injury: latest insights into consequent eating disorders and obesity. Curr Opin Endocrinol Diabetes Obes 23:81–89

    Article  PubMed  Google Scholar 

  57. Curtis J, Daneman D, Hoffman HJ, Ehrlich RM (1994) The endocrine outcome after surgical removal of craniopharyngiomas. Pediatr Neurosurg 21(Suppl 1):24–27

    PubMed  Google Scholar 

  58. Müller HL, Bueb K, Bartels U et al (2001) Obesity after childhood craniopharyngioma--German multicenter study on pre-operative risk factors and quality of life. Klin Padiatr 213:244–249

    Article  PubMed  Google Scholar 

  59. Harz KJ, Müller HL, Waldeck E, Pudel V, Roth C (2003) Obesity in patients with craniopharyngioma: assessment of food intake and movement counts indicating physical activity. J Clin Endocrinol Metab 88:5227–5231

    Article  CAS  PubMed  Google Scholar 

  60. Zoicas F, Droste M, Mayr B, Buchfelder M, Schöfl C (2013) GLP-1 analogues as a new treatment option for hypothalamic obesity in adults: report of nine cases. Eur J Endocrinol 168:699–706

    Article  CAS  PubMed  Google Scholar 

  61. Carpentieri SC, Waber DP, Scott RM, Goumnerova LC, Kieran MW, Cohen LE, Kim F, Billett AL, Tarbell NJ, Pomeroy SL (2001) Memory deficits among children with craniopharyngiomas. Neurosurgery 49:1053–1057 discussion 1057–8

    CAS  PubMed  Google Scholar 

  62. Sands SA, Milner JS, Goldberg J, Mukhi V, Moliterno JA, Maxfield C, Wisoff JH (2005) Quality of life and behavioral follow-up study of pediatric survivors of craniopharyngioma. J Neurosurg 103:302–311

    PubMed  Google Scholar 

  63. Merchant TE, Kiehna EN, Sanford RA, Mulhern RK, Thompson SJ, Wilson MW, Lustig RH, Kun LE (2002) Craniopharyngioma: the St. Jude Children’s Research Hospital experience 1984-2001. Int J Radiat Oncol Biol Phys 53:533–542

    Article  PubMed  Google Scholar 

  64. Liubinas SV, Munshey AS, Kaye AH (2011) Management of recurrent craniopharyngioma. J Clin Neurosci 18:451–457

    Article  PubMed  Google Scholar 

  65. Duff J, Meyer FB, Ilstrup DM, Laws ER, Schleck CD, Scheithauer BW (2000) Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46:291–302 discussion 302–5

    Article  CAS  PubMed  Google Scholar 

  66. Kalapurakal JA, Goldman S, Hsieh YC, Tomita T, Marymont MH (2003) Clinical outcome in children with craniopharyngioma treated with primary surgery and radiotherapy deferred until relapse. Med Pediatr Oncol 40:214–218

    Article  PubMed  Google Scholar 

  67. Vinchon M, Dhellemmes P (2008) Craniopharyngiomas in children: recurrence, reoperation and outcome. Childs Nerv Syst 24:211–217

    Article  PubMed  Google Scholar 

  68. Klimo P, Venable GT, Boop FA, Merchant TE (2015) Recurrent craniopharyngioma after conformal radiation in children and the burden of treatment. J Neurosurg Pediatr 15:499–505

    Article  PubMed  Google Scholar 

  69. Cavalheiro S, Di Rocco C, Valenzuela S et al (2010) Craniopharyngiomas: intratumoral chemotherapy with interferon-alpha: a multicenter preliminary study with 60 cases. Neurosurg Focus 28:E12

    Article  PubMed  Google Scholar 

  70. Murphy ES, Chao ST, Angelov L, Vogelbaum MA, Barnett G, Jung E, Recinos VR, Mohammadi A, Suh JH (2015) Radiosurgery for pediatric brain tumors. Pediatr Blood Cancer 63:398–405. doi:10.1002/pbc.25831

    Article  PubMed  Google Scholar 

  71. Lee C-C, Yang H-C, Chen C-J, Hung Y-C, Wu H-M, Shiau C-Y, Guo W-Y, Pan DH-C, Chung W-Y, Liu K-D (2014) Gamma Knife surgery for craniopharyngioma: report on a 20-year experience. J Neurosurg 121(Suppl):167–178

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, Great Ormond Street Hospital for Children, Great Ormond Street, London, WC1N 3JH, UK

    Kristian Aquilina

  2. Department of Neurosurgery, University Hospital Erlangen, Schwabachanlage 6, Erlangen, 91054, Germany

    Michael Buchfelder

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  1. Kristian Aquilina
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Correspondence to Kristian Aquilina .

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Editors and Affiliations

  1. Developmental Biology and Cancer Programme, Birth Defects Research Centre, Institute of Child Health, University College London, London, United Kingdom

    Juan Pedro Martinez-Barbera

  2. Craniofacial Development and Stem Cell Biology, King’s College London, London, United Kingdom

    Cynthia Lilian Andoniadou

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Aquilina, K., Buchfelder, M. (2017). Surgical Treatment of Human ACP. In: Martinez-Barbera, J., Lilian Andoniadou, C. (eds) Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma. Springer, Cham. https://doi.org/10.1007/978-3-319-51890-9_8

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  • DOI: https://doi.org/10.1007/978-3-319-51890-9_8

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