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Management of craniopharyngiomas

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Abstract

Craniopharyngiomas are rare epithelial tumours arising along the path of the craniopharyngeal duct. Their pathogenesis remains uncertain and they can present with a variety of manifestations attributed to pressure effects to surrounding structures. The optimal management of craniopharyngiomas remains challenging mainly due to their sharp, irregular borders and their tendency to adhere to vital neurovascular structures making surgical manipulations potentially hazardous to vital brain areas. Non-aggressive surgery followed by radiotherapy is currently the most widely used option possibly achieving the most optimal long-term outcome. Other treatment modalities including intracystic irradiation, intracystic instillation of antineoplasmatic agents and stereotactic radiotherapy are also available in our armamentarium. The long-term morbidities related with the craniopharyngiomas and their treatment remain significant, with hypothalamic damage playing the protagonist role and requiring further studies to identify measures that will improve the prognosis of the patients.

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References

  1. Karavitaki N, Cudlip S, Adams CB, Wass JA (2006) Craniopharyngiomas. Endocr Rev 2006(27):371–397

    Article  Google Scholar 

  2. Larkin SJ, Ansorge O (2013) Pathology and pathogenesis of craniopharyngiomas. Pituitary 16(1):9–17

    PubMed  Google Scholar 

  3. Crotty TB, Scheithauer BW, Young WF Jr et al (1997) Papillary craniopharyngioma: a clinicopathological study of 48 cases. J Neurosurg 83:206–214

    Article  Google Scholar 

  4. Petito CK, De Girolami U, Earle KM (1976) Craniopharyngiomas: a clinical and pathological review. Cancer 37:1944–1952

    Article  CAS  PubMed  Google Scholar 

  5. Weiner HL, Wisoff JH, Rosenberg ME et al (1994) Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery 35:1001–1011

    Article  CAS  PubMed  Google Scholar 

  6. 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 

  7. Karavitaki N, Brufani C, Warner JT et al (2005) Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clin Endocrinol 62:397–409

    Article  CAS  Google Scholar 

  8. Van Effentere R, Boch AL (2002) Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97(1):3–11

    Google Scholar 

  9. Sartoretti-Schefer S, Wichmann W, Aguzzi A, Valavanis A (1997) MRI differentiation of adamantinous and squamous-papillary craniopharyngiomas. Am J Neuroradiol 18(1):77–87

    CAS  PubMed  Google Scholar 

  10. Byrne JV (2002) Imaging of the pituitary. In: Wass JAH, Shalet SM (eds) Oxford textbook of endocrinology and diabetes, 1st edn. Oxford University Press, Oxford, pp 136–145

    Google Scholar 

  11. Komotar RJ, Starke RM (2012) Endoscopic endonasal compared with microscopic transsphenoidal and open transcranial resection of craniopharyngiomas. World Neurosurg 77(2):329–341

    Article  PubMed  Google Scholar 

  12. Buchfelder M, Schlaffer SM, Lin F, Kleindienst A (2013) Surgery for craniopharyngioma. Pituitary 16:18–25

    Article  PubMed  Google Scholar 

  13. Elliott RE, Jane JA Jr, Wisoff JH (2011) Surgical management of craniopharyngiomas in children: meta-analysis and comparison of transcranial and transsphenoidal approaches. Neurosurgery 69(3):630–643

    Article  PubMed  Google Scholar 

  14. Carpenter RC, Chamberlin GW, Frazier CH (1937) The treatment of hypophyseal stalk tumours by evacuation and irradiation. Am J Roent 38:162–167

    Google Scholar 

  15. Kramer S, Mckissock W, Concanon JP (1961) Craniopharyngiomas: treatment by combined surgery and radiation therapy. J Neurosurg 18:217–226

    Article  CAS  PubMed  Google Scholar 

  16. Costine LS, Randall SH, Rubin P, McDonald J (1989) Craniopharyngiomas: fluctuation in cyst size following surgery and radiation therapy. Neurosurgery 24:53–59

    Article  Google Scholar 

  17. Rajan B, Ashley S, Thomas DGT et al (1997) Craniopharyngioma: improving outcome by early recognition and treatment of acute complications. Int J Radiat Oncol Biol Phys 37:517–521

    Article  CAS  PubMed  Google Scholar 

  18. Shi Z, Esiashvili N, Janss AJ et al (2012) Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation. J Neurooncol 109(2):349–355

    Article  CAS  PubMed  Google Scholar 

  19. Minniti G, Saran F, Traish D et al (2007) Fractionated stereotactic conformal radiotherapy following conservative surgery in the control of craniopharyngiomas. Radiother Oncol 82:90–95

    Article  PubMed  Google Scholar 

  20. Combs SE, Thilmann C, Huber PE et al (2007) Achievement of long term control in patients with craniopharyngiomas using high precision stereotactic radiotherapy. Cancer 109:2308–2314

    Article  PubMed  Google Scholar 

  21. Rajan B, Ashley S, Gorman C et al (1993) Craniopharyngioma—long-term results following limited surgery and radiotherapy. Radiother Oncol 26:1–10

    Article  CAS  PubMed  Google Scholar 

  22. Jose CC, Rajan B, Ashley S, Marsh H, Brada M (1992) Radiotherapy for the treatment of recurrent craniopharyngioma. Clin Oncol 4:287–289

    Article  CAS  Google Scholar 

  23. Gutin DH, Klemme WM, Lagger RI, Mackay AR, Pitts LH, Hojobuchi Y (1980) Management of unresectable cystic craniopharyngioma by aspiration through an Ommaya reservoir drainage system. J Neurosurg 52:36–40

    Article  CAS  PubMed  Google Scholar 

  24. Spaziante R, Irace C, De Divitiis E (1993) Brachytherapy of cystic craniopharyngiomas. J Neurosurg 79:966–967

    CAS  PubMed  Google Scholar 

  25. 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 33:437–446

    Article  CAS  PubMed  Google Scholar 

  26. 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 b-emitting radioactive sources. Neurosurgery 40:263–270

    Article  CAS  PubMed  Google Scholar 

  27. Van den Berge JH, Blaauw G, Breeman WAP, Rahmy A, Wijngaarde R (1992) Intracavitary brachytherapy of cystic craniopharyngiomas. J Neurosurg 77:545–550

    Article  PubMed  Google Scholar 

  28. Hasegawa T, Kondzilka D, Hadjipanayis CG, Lunsford LD (2004) Management of cystic craniopharyngiomas with phosphorus-32 intracavitary irradiation. Neurosurgery 54:813–822

    Article  PubMed  Google Scholar 

  29. Julow JV (2013) Intracystic irradiation for craniopharyngiomas. Pituitary 16(1):34–35

    PubMed  Google Scholar 

  30. Kickingereder P, Maarouf M, El Majdoub F et al (2012) Intracavitary brachytherapy using stereotactically applied phosphorus-32 colloid for treatment of cystic craniopharyngiomas in 53 patients. J Neurooncol 109(2):365–367

    Article  CAS  PubMed  Google Scholar 

  31. Takahashi H, Nakazawa S, Shimura T (1985) Evaluation of postoperative injection of bleomycin for craniopharyngioma in children. J Neurosurg 62:120–127

    Article  CAS  PubMed  Google Scholar 

  32. Hader WJ, Steinbok P, Hukin J, Fryer C (2000) Intratumoral therapy with bleomycin for cystic craniopharyngiomas in children. Pediatr Neurosurg 33:211–218

    Article  CAS  PubMed  Google Scholar 

  33. Broggi G, Franzini A, Cajola L, Pluchino F (1994) Cell kinetic investigations in craniopharyngioma: preliminary results and considerations. Pediatr Neurosurg 21:21–23

    Article  PubMed  Google Scholar 

  34. Leber KA, Bergloeff J, Pendl G (1998) Dose response tolerance of the visual pathways and cranial nerves of the cavernous sinus to stereotactic radiosurgery. J Neurosurg 88:43–50

    Article  CAS  PubMed  Google Scholar 

  35. Xu Z, Yen CP, Schlesinger D et al (2011) Outcomes of gamma knife surgery for craniopharyngioma. J Neurooncol 104(1):305–313

    Article  CAS  PubMed  Google Scholar 

  36. Niranjan A, Kano H, Mathieu D et al (2010) Radiosurgery for craniopharyngioma. Int J Radiat Oncol Biol Phys 78(1):64–71

    Article  PubMed  Google Scholar 

  37. Kobayashi T, Kida Y, Mori Y et al (2005) Long-term results of gamma knife surgery for treatment of craniopharyngioma in 98 consecutive cases. J Neurosurg Pediatr 103(suppl):482–488

    Article  Google Scholar 

  38. Aggarwal A, Fersht N, Brada M (2013) Radiotherapy for craniopharyngioma. Pituitary 16(1):26–33

    PubMed  Google Scholar 

  39. Bremmer AM, Nguyen TQ, Balsys R (1984) Therapeutic benefits of combination chemotherapy with vincristine, BCNU, and procarbazine on recurrent cystic craniopharyngioma. A case report. J Neurooncol 2:47–51

    Article  Google Scholar 

  40. Lippens RJ, Rotteveel JJ, Otten BJ, Merx H (1998) Chemotherapy with adriamycin (doxorubicin) and CCNU (lomustine) in four children with recurrent craniopharyngioma. Eur J Paediatr Neurol 2:263–268

    Article  CAS  PubMed  Google Scholar 

  41. Yeung JT, Pollack IF, Panigraphy et al (2012) Pegylated interferon-a-2b for children with recurrent craniopharyngioma. J Neurosurg Pediatr 10(6):498–503

    Article  PubMed  Google Scholar 

  42. Jakacki RI, Cohen BH, Jamison C et al (2000) Phase II evaluation of interferon-α-2a for progressive recurrent craniopharyngiomas. J Neurosurg 92:250–260

    Article  Google Scholar 

  43. 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):767–774

    Article  PubMed  Google Scholar 

  44. Toledano Y, Lubetsky A, Shimon I (2007) Acquired prolactin deficiency in patients with disorders of the hypothalamic-pituitary axis. J Endocrinol Invest 30:268–273

    CAS  PubMed  Google Scholar 

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

    Article  Google Scholar 

  46. Schoenle EJ, Zapf J, Prader A, Torresani T, Werder EA, Zachemann M (1995) Replacement of growth hormone (GH) in normally growing GH-deficient patients operated for craniopharyngioma. J Clin Endocrinol Metab 80:374–378

    CAS  PubMed  Google Scholar 

  47. Sklar CA (1994) Craniopharyngioma: endocrine sequelae of treatment. Pediatr Neurosurg 21:120–123

    Article  PubMed  Google Scholar 

  48. Kendall-Taylor P, Jönsson PJ, Abs R et al (2005) The clinical, metabolic and endocrine features and the quality of life in adults with childhood-onset craniopharyngioma compared with adult-onset craniopharyngioma. Eur J Endocrinol 152:557–567

    Article  CAS  PubMed  Google Scholar 

  49. Price DA, Wilton P, Jonsson P et al (1998) Efficacy and safety of growth hormone treatment in children with prior craniopharyngioma: an analysis of Pharmacia and Upjohn International Growth Database (KIGS) from 1988 to 1996. Horm Res 49:91–97

    Article  Google Scholar 

  50. Geffner M, Lundberg M, Koltowska-Haggstrom M et al (2004) Changes in height, weight, and body mass index in children with craniopharyngioma after three years of growth hormone therapy: analysis of KIGS (Pfizer International Growth Database). J Clin Endocrinol Metab 89:5435–5440

    Article  CAS  PubMed  Google Scholar 

  51. Verhelst J, Kendall-Taylor P, Erfurth EM et al (2005) Baseline characteristics and response to 2 years of growth hormone (GH) replacement of hypopituitary patients with GH deficiency due to adult-onset craniopharyngioma in comparison with patients with nonfunctioning pituitary adenoma: data from KIMS (Pfizer International Metabolic Database). J Clin Endocrinol Metab 90:4636–4643

    Article  CAS  PubMed  Google Scholar 

  52. Ulfarsson E, Karstrom A, Yin S et al (2005) Expression and growth dependency of the insulin-like growth factor I receptor in craniopharyngioma cells: a novel therapeutic approach. Clin Cancer Res 11:4674–4680

    Article  CAS  PubMed  Google Scholar 

  53. Karavitaki N (2007) Hypopituitarism oddities: craniopharyngiomas. Horm Res 68(Suppl 5):151–153

    Article  PubMed  Google Scholar 

  54. Karavitaki N, Warner JT, Marland A et al (2006) GH replacement does not increase the risk of recurrence in patients with craniopharyngiomas. Clin Endocrinol 64:556–560

    Article  CAS  Google Scholar 

  55. 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. JAMA 118:45–50

    Article  CAS  Google Scholar 

  56. Stripp DC, Maity A, Janss AJ et al (2004) Surgery with or without radiation therapy in the management of craniopharyngiomas in children and young adults. Int J Radiat Oncol Biol Phys 28:714–720

    Article  Google Scholar 

  57. Pereira AM, Scmid EM, Schutte PJ et al (2005) High prevalence of long-term cardiovascular, neurological and psychosocial morbidity after treatment for craniopharyngiomas. Clin Endocrinol 62:197–204

    Article  Google Scholar 

  58. De Vile CJ, Grant DB, Hayward RD, Kendall BE, Neville BGR, 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

    PubMed  Google Scholar 

  59. Roth C, Wilken B, Hanefeld F, Schroter F, Leonhardt U (1998) Hyperphagia in children with craniopharyngioma is associated with hyperleptinaemia and a failure in the downregulation of appetite. Eur J Endocrinol 138:89–91

    Article  CAS  PubMed  Google Scholar 

  60. Pinkney J, Wilding J, Williams G, MacFarlane I (2002) Hypothalamic obesity in humans: what do we know and what can be done? Obes Rev 3:27–34

    Article  CAS  PubMed  Google Scholar 

  61. Harz KJ, Muller 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 

  62. Holmer H, Pozarek G, Wirfält E et al (2010) Reduced energy expenditure and impaired feeding-related signals but not high energy intake reinforces hypothalamic obesity in adults with childhood onset craniopharyngioma. J Clin Endocrinol Metab 95:5395–5402

    Article  CAS  PubMed  Google Scholar 

  63. Piguel P, Abraham P, Bouchours-Nouet N et al (2012) Impaired aerobic exercise adaptation in children and adolescents with craniopharyngioma is associated with hypothalamic involvement. Eur J Endocrinol 166:215–222

    Article  CAS  PubMed  Google Scholar 

  64. Goldstone AP, Patterson M, Kalingag N et al (2005) Fasting and post-prandial hyperghrelinemia in Prader–Willi syndrome is partially explained by hypoinsulinemia, and is not due to PYY deficiency or seen in hypothalamic obesity due to craniopharyngioma. J Clin Endocrinol Metab 90:2681–2690

    Article  CAS  PubMed  Google Scholar 

  65. 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(6):2376–2382

    Article  CAS  PubMed  Google Scholar 

  66. Bretault M, Boillot A, Muzzard L et al (2013) Clinical review: bariatric surgery following treatment for craniopharyngioma: a systematic review and individual-level data meta-analysis. J Clin Endocrinol Metab 98(6):2239–2246

    Article  CAS  PubMed  Google Scholar 

  67. Weismann D, Pelka T, Bender G et al (2013) Bariatric surgery for morbid obesity in craniopharyngioma. Clin Endocrinol 78:385–390

    Article  Google Scholar 

  68. Mason PW, Krawiecki N, Meacham LR (2002) The use of dextroamphetamine to treat obesity and hyperphagia in children treated for craniopharyngioma. Arch Pediatr Adolesc Med 156:887–892

    Article  PubMed  Google Scholar 

  69. Hamilton JK, Conwell LS, Syme C, Ahmet A, Jeffery A, Daneman D (2011) Hypothalamic obesity following craniopharyngioma surgery: results of a pilot trial of combined diazoxide and metformin therapy. Int J Pediatr Endocrinol 2011:417949

    Article  PubMed Central  PubMed  Google Scholar 

  70. Lustig RH, Hinds PS, Ringwald-Smith K et al (2003) Octreotide therapy of pediatric hypothalamic obesity: a double-blind, placebo-controlled trial. J Clin Endocrinol Metab 88:2586–2592

    Article  CAS  PubMed  Google Scholar 

  71. Zoicas F, Droste M, Mayr B, Buchfelder M, Schofl C (2013) GLP-1 analogues as a new treatment option for hypothalamic obesity in adults: report of nine cases. Eur J Endocrinol 168(5):699–706

    Article  CAS  PubMed  Google Scholar 

  72. Duff JM, Meyer FB, Ilstrup DM, Laws ERJr, Scleck CD, Scheithauer BW (2000) Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46(2):291–305

    Article  CAS  PubMed  Google Scholar 

  73. Gautier A, Godbout A, Grosheny C et al (2012) Markers of recurrence and long-term morbidity in craniopharyngioma: a systematic analysis of 171 patients. J Clin Endocrinol Metab 97(4):1258–1267

    Article  CAS  PubMed  Google Scholar 

  74. Di Pinto M, Conklin HM, Merchant TE (2012) Learning and memory following conformal radiation therapy for paediatric craniopharyngioma and low-grade glioma. Int J Radiat Oncol Biol Phys 84(3):363–369

    Article  Google Scholar 

  75. Varlotto JM, Flickinger JC, Kondziolka D, Lunsford LD, Deutch M (2002) External beam irradiation of craniopharyngiomas: long-term analysis of tumour control and morbidity. Int J Radiat Oncol Biol Phys 54:492–499

    Article  PubMed  Google Scholar 

  76. Erfurth EM, Holmer H, Fjalldal SB (2013) Mortality and morbidity in adult craniopharyngioma. Pituitary 16(1):46–55

    Article  PubMed  Google Scholar 

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  1. Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, Old Rd, Headington, Oxford, OX3 7LJ, UK

    N. Karavitaki

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Karavitaki, N. Management of craniopharyngiomas. J Endocrinol Invest 37, 219–228 (2014). https://doi.org/10.1007/s40618-013-0050-9

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