, Volume 16, Issue 4, pp 528–535 | Cite as

Acute presentation of craniopharyngioma in children and adults in a Danish national cohort

  • E. H. NielsenEmail author
  • J. O. Jørgensen
  • P. Bjerre
  • M. Andersen
  • C. Andersen
  • U. Feldt-Rasmussen
  • L. Poulsgaard
  • L. Ø. Kristensen
  • J. Astrup
  • J. Jørgensen
  • P. Laurberg


We aimed to study the occurrence of acute-onset symptoms at initial presentation in a national Danish cohort of patients with childhood- or adult-onset craniopharyngioma, and to investigate potential risk factors for acute presentation. Medical records of 189 consecutive patients (39 children, 150 adults) presenting with craniopharyngioma during the period 1985–2004 were reviewed, and data regarding initial symptoms, neuroimaging results, vision and pituitary function were systematically collected. Acute symptoms preceding hospital admission were noted. Subgroup analyses were based on age, gender and calendar year period. Potential risk factors for acute presentation were analysed through uni- and multivariate analyses. Acute symptoms were reported in 24 (13 %) patients. Acute visual symptoms, headache, nausea or vomiting were most frequently reported, and acute symptoms were more frequent among children (28 %) than among adults (9 %) (P < 0.01). There were no differences according to sex or calendar year period. Hydrocephalus was present in half of childhood cases and one-fifth of adult patients (P < 0.001). Intra-tumour haemorrhage was seen in two cases. Acute symptoms were more frequent among patients with tumours occupying the third ventricle (P < 0.01), radiologic signs of calcification (P < 0.05) or hydrocephalus (P < 0.01). In multivariate analysis, however, only childhood onset (P < 0.05) and calcification (P < 0.05) were independent risk factors for acute presentation. Craniopharyngioma presented with acute symptoms in 13 % of patients. Childhood onset and radiologic signs of calcification were independent risk factors for acute presentation. Intra-tumour haemorrhage was rare.


Craniopharyngioma Symptoms Presentation Children Adults Hydrocephalus Pituitary tumour haemorrhage 



We appreciate constructive discussions with Dr. Jörgen Lindholm on this publication.


  1. 1.
    Love JG, Marshall TM (1950) Craniopharyngiomas. Surg Gynecol Obstet 90:591–601PubMedGoogle Scholar
  2. 2.
    Ammirati M, Samii M, Sephernia A (1990) Surgery of large retrochiasmatic craniopharyngiomas in children. Childs Nerv Syst 6:13–17PubMedCrossRefGoogle Scholar
  3. 3.
    Banna M (1973) Craniopharyngioma in adults. Surg Neurol 1:202–204PubMedGoogle Scholar
  4. 4.
    Banna M, Hoare RD, Stanley P, Till K (1973) Craniopharyngioma in children. J Pediatr 83:781–785PubMedCrossRefGoogle Scholar
  5. 5.
    Baskin DS, Wilson CB (1986) Surgical management of craniopharyngiomas. A review of 74 cases. J Neurosurg 65:22–27PubMedCrossRefGoogle Scholar
  6. 6.
    Caldarelli M, Massimi L, Tamburrini G, Cappa M, Di RC (2005) Long-term results of the surgical treatment of craniopharyngioma: the experience at the Policlinico Gemelli, Catholic University. Rome. Childs Nerv Syst 21:747–757CrossRefGoogle Scholar
  7. 7.
    Cherninkova S, Tzekov H, Karakostov V (1990) Comparative ophthalmologic studies on children and adults with craniopharyngiomas. Ophthalmologica 201:201–205PubMedCrossRefGoogle Scholar
  8. 8.
    Crane TB, Yee RD, Hepler RS, Hallinan JM (1982) Clinical manifestations and radiologic findings in craniopharyngiomas in adults. Am J Ophthalmol 94:220–228PubMedGoogle Scholar
  9. 9.
    Crotty TB, Scheithauer BW, Young WF Jr et al (1995) Papillary craniopharyngioma: a clinicopathological study of 48 cases. J Neurosurg 83:206–214PubMedCrossRefGoogle Scholar
  10. 10.
    de Vries L, Lazar L, Phillip M (2003) Craniopharyngioma: presentation and endocrine sequelae in 36 children. J Pediatr Endocrinol Metab 16:703–710PubMedCrossRefGoogle Scholar
  11. 11.
    Ersahin Y, Yurtseven T, Ozgiray E, Mutluer S (2005) Craniopharyngiomas in children: Turkey experience. Childs Nerv Syst 21:766–772PubMedCrossRefGoogle Scholar
  12. 12.
    Fahlbusch R, Hofmann BM (2008) Surgical management of giant craniopharyngiomas. Acta Neurochir (Wien) 150:1213–1226CrossRefGoogle Scholar
  13. 13.
    Defoort-Dhellemmes S, Moritz F, Bouacha I, Vinchon M (2006) Craniopharyngioma: ophthalmological aspects at diagnosis. J Pediatr Endocrinol Metab 19(Suppl 1):321–324PubMedGoogle Scholar
  14. 14.
    Galatzer A, Nofar E, Beit-Halachmi N et al (1981) Intellectual and psychosocial functions of children, adolescents and young adults before and after operation for craniopharyngioma. Child Care Health Dev 7:307–316PubMedCrossRefGoogle Scholar
  15. 15.
    Hoffman HJ, De SM, Humphreys RP, Drake JM, Smith ML, Blaser SI (1992) Aggressive surgical management of craniopharyngiomas in children. J Neurosurg 76:47–52PubMedCrossRefGoogle Scholar
  16. 16.
    Iwasaki K, Kondo A, Takahashi JB, Yamanobe K (1992) Intraventricular craniopharyngioma: report of two cases and review of the literature. Surg Neurol 38:294–301PubMedCrossRefGoogle Scholar
  17. 17.
    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 (Oxf) 62:397–409CrossRefGoogle Scholar
  18. 18.
    Kendall-Taylor P, Jonsson 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 152:557–567PubMedCrossRefGoogle Scholar
  19. 19.
    Kennedy HB, Smith RJ (1975) Eye signs in craniopharyngioma. Br J Ophthalmol 59:689–695PubMedCrossRefGoogle Scholar
  20. 20.
    Larijani B, Bastanhagh MH, Pajouhi M, Kargar SF, Vasigh A, Aghakhani S (2004) Presentation and outcome of 93 cases of craniopharyngioma. Eur J Cancer Care (Engl) 13:11–15CrossRefGoogle Scholar
  21. 21.
    Mottolese C, Szathmari A, Berlier P, Hermier M (2005) Craniopharyngiomas: our experience in Lyon. Childs Nerv Syst 21:790–798PubMedCrossRefGoogle Scholar
  22. 22.
    Muller 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 213:244–249PubMedCrossRefGoogle Scholar
  23. 23.
    Paja M, Lucas T, Garcia-Uria J, Salame F, Barcelo B, Estrada J (1995) Hypothalamic-pituitary dysfunction in patients with craniopharyngioma. Clin Endocrinol (Oxf) 42:467–473CrossRefGoogle Scholar
  24. 24.
    Puget S, Garnett M, Wray A et al (2007) Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg 106(1 Suppl):3–12PubMedGoogle Scholar
  25. 25.
    Shin JL, Asa SL, Woodhouse LJ, Smyth HS, Ezzat S (1999) Cystic lesions of the pituitary: clinicopathological features distinguishing craniopharyngioma, Rathke’s cleft cyst, and arachnoid cyst. J Clin Endocrinol Metab 84:3972–3982PubMedCrossRefGoogle Scholar
  26. 26.
    Sorva R, Heiskanen O, Perheentupa J (1987) Craniopharyngioma in adults. Ann Clin Res 19:339–343PubMedGoogle Scholar
  27. 27.
    Sorva R (1988) Children with craniopharyngioma. Early growth failure and rapid postoperative weight gain. Acta Paediatr Scand 77:587–592PubMedCrossRefGoogle Scholar
  28. 28.
    Tavangar SM, Larijani B, Mahta A, Hosseini SM, Mehrazine M, Bandarian F (2004) Craniopharyngioma: a clinicopathological study of 141 cases. Endocr Pathol 15:339–344PubMedCrossRefGoogle Scholar
  29. 29.
    Van ER, Boch AL (2002) Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97:3–11CrossRefGoogle Scholar
  30. 30.
    Zuccaro G (2005) Radical resection of craniopharyngioma. Childs Nerv Syst 21:679–690PubMedCrossRefGoogle Scholar
  31. 31.
    Buhl R, Lang EW, Barth H, Mehdorn HM (2000) Giant cystic craniopharyngiomas with extension into the posterior fossa. Childs Nerv Syst 16:138–142PubMedCrossRefGoogle Scholar
  32. 32.
    Cabezudo JM, Perez C, Vaquero J, Garcia-de-Sola R, Bravo G (1981) Pubertas praecox in craniopharyngioma. Case report. J Neurosurg 55:127–131PubMedCrossRefGoogle Scholar
  33. 33.
    Connolly ES Jr, Winfree CJ, Carmel PW (1997) Giant posterior fossa cystic craniopharyngiomas presenting with hearing loss. Report of three cases and review of the literature. Surg Neurol 47:291–299PubMedCrossRefGoogle Scholar
  34. 34.
    Maiuri F, Corriero G, Elefante R, Cirillo S, Giamundo A (1987) Craniopharyngioma of the cranial base and nasopharynx. Surg Neurol 27:191–194PubMedCrossRefGoogle Scholar
  35. 35.
    Rohrer TR, Fahlbusch R, Buchfelder M, Dorr HG (2006) Craniopharyngioma in a female adolescent presenting with symptoms of anorexia nervosa. Klin Padiatr 218:67–71PubMedCrossRefGoogle Scholar
  36. 36.
    Nielsen EH, Feldt-Rasmussen U, Poulsgaard L et al (2011) Incidence of craniopharyngioma in Denmark (n = 189) and estimated world incidence of craniopharyngioma in children and adults. J Neurooncol 104:755–763PubMedCrossRefGoogle Scholar
  37. 37.
    Nielsen EH, Lindholm J, Laurberg P (2011) Use of combined search criteria improved validity of rare disease (craniopharyngioma) diagnosis in a national registry. J Clin Epidemiol 64:1118–1126PubMedCrossRefGoogle Scholar
  38. 38.
    Carmichael JD (2011) Anterior pituitary failure. In: Melmed Shlomo (ed) The pituitary, 3rd edn. Elsevier/Academic Press, Amsterdam, New York, pp 343–381CrossRefGoogle Scholar
  39. 39.
    Ghigo E, Aimaretti G, Corneli G (2008) Diagnosis of adult GH deficiency. Growth Horm IGF Res 18:1–16PubMedCrossRefGoogle Scholar
  40. 40.
    Ghigo E, Aimaretti G, Arvat E, Camanni F (2001) Growth hormone-releasing hormone combined with Arginine or growth hormone secretagogues for the diagnosis of growth hormone deficiency in adults. Endocrine 15:29–38PubMedCrossRefGoogle Scholar
  41. 41.
    Hoeck HC et al (2000) Diagnosis of Growth Hormone (GH) deficiency in adults with hypothalamic-pituitary disorders: comparison of test results using pyridostigmine plus GH-releasing hormone (GHRH), clonidine plus GHRH, and insulin-induced hypoglycemia as GH secretagogues. J Clin Endocrinol Metab 85:1467–1472PubMedCrossRefGoogle Scholar
  42. 42.
    Hartman ML (2002) Which patients do not require a GH stimulation test for the diagnosis of adult GH deficiency? J Clin Endocrinol Metab 87:477–485PubMedCrossRefGoogle Scholar
  43. 43.
    Aimaretti G et al (1998) Usefulness of IGF-1 assay for the diagnosis of GH deficiency in adults. J Endocrinol Invest 21:506–5011PubMedGoogle Scholar
  44. 44.
    Shalet SM, Toogood A, Brennan BMD (1998) The diagnosis of growth hormone deficiency in children and adults. Endocr Rev 19:203–223PubMedCrossRefGoogle Scholar
  45. 45.
    Richmond EJ, Rogol AD (2008) Growth hormone deficiency in children. Pituitary 11:115–120PubMedCrossRefGoogle Scholar
  46. 46.
    Stanley T (2012) Diagnosis of growth hormone deficiency in childhood. Curr Opin Endocrinol Diabetes Obes 19:47–52PubMedCrossRefGoogle Scholar
  47. 47.
    GH Research Society (2000) Consensus guidelines for the diagnosis and treatment of growth hormone (GH) deficiency in childhood and adolescence: summary statement of the GH Research Society. J Clin Endocrinol Metab 85:3990–3993CrossRefGoogle Scholar
  48. 48.
    Binder G, Huller E, Blumenstock G, Schweizer R (2011) Auxology-based cut-off values for biochemical testing of GH secretion in childhood. Growth Horm IGF Res 21:212–218PubMedCrossRefGoogle Scholar
  49. 49.
    Grant FC (1948) Surgical experience with tumors of the pituitary gland. J Am Med Assoc 136:668–672PubMedCrossRefGoogle Scholar
  50. 50.
    Müller R, Wohlfart G (1950) Craniopharyngiomas. Acta Med Scand 138:121–138PubMedCrossRefGoogle Scholar
  51. 51.
    Vieira JP, Lopes P, Silva R (2012) Primary ciliary dyskinesia and hydrocephalus with acqueductal stenosis. J Child Neurol. doi: 10.1177/0883073811429856
  52. 52.
    Karaman A, Kahveci H (2011) Klippel-Feil syndrome and Dandy-Walker malformation. Genet Couns 22:411–415PubMedGoogle Scholar
  53. 53.
    Wong TT, Liang ML, Chen HH, Chang FC (2011) Hydrocephalus with brain tumors in children. Childs Nerv Syst 27:1723–1734PubMedCrossRefGoogle Scholar
  54. 54.
    Marmarou A, Foda MA, Bandoh K et al (1996) Posttraumatic ventriculomegaly: hydrocephalus or atrophy? A new approach for diagnosis using CSF dynamics. J Neurosurg 85:1026–1035PubMedCrossRefGoogle Scholar
  55. 55.
    Balami JS, Buchan AM (2012) Complications of intracerebral haemorrhage. Lancet Neurol 11:101–118PubMedCrossRefGoogle Scholar
  56. 56.
    Tandon V, Mahapatra AK (2011) Management of post-tubercular hydrocephalus. Childs Nerv Syst 27:1699–1707PubMedCrossRefGoogle Scholar
  57. 57.
    Aliabadi H, Reynolds R, Powers CJ, Grant G, Fuchs H, Kurtzberg J (2010) Clinical outcome of cerebrospinal fluid shunting for communicating hydrocephalus in mucopolysaccharidoses I, II, and III: a retrospective analysis of 13 patients. Neurosurgery 67:1476–1481PubMedCrossRefGoogle Scholar
  58. 58.
    Weiss SL, Wegg-Uzunlar L, Bowman RM, Brannen ML (2011) Diagnosis of a craniopharyngioma after acute brainstem herniation in an emergency department. Pediatr Emerg Care 27:747–750PubMedCrossRefGoogle Scholar
  59. 59.
    Rekate HL (2011) A consensus on the classification of hydrocephalus: its utility in the assessment of abnormalities of cerebrospinal fluid dynamics. Childs Nerv Syst 27:1535–1541PubMedCrossRefGoogle Scholar
  60. 60.
    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 83:3897–3904PubMedCrossRefGoogle Scholar
  61. 61.
    Duff J, Meyer FB, Ilstrup DM, Laws ER Jr, Schleck CD, Scheithauer BW (2000) Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46:291–302PubMedCrossRefGoogle Scholar
  62. 62.
    Elliott RE, Wisoff JH (2010) Surgical management of giant pediatric craniopharyngiomas. J Neurosurg Pediatr 6:403–416PubMedCrossRefGoogle Scholar
  63. 63.
    Tomita T, Bowman RM (2005) Craniopharyngiomas in children: surgical experience at Children’s Memorial Hospital. Childs Nerv Syst 21:729–746PubMedCrossRefGoogle Scholar
  64. 64.
    Nielsen EH, Lindholm J, Bjerre P et al (2006) Frequent occurrence of pituitary apoplexy in patients with non-functioning pituitary adenoma. Clin Endocrinol (Oxf) 64:319–322CrossRefGoogle Scholar
  65. 65.
    Semple PL, Webb MK, De Villiers JC, Laws ER Jr (2005) Pituitary apoplexy. Neurosurgery 56:65–72PubMedGoogle Scholar
  66. 66.
    Nishioka H, Ito H, Haraoka J, Hashimoto T, Kato Y (2000) Repeated hemorrhage in ciliated craniopharyngioma–case report. Neurol Med Chir (Tokyo) 40:324–328CrossRefGoogle Scholar
  67. 67.
    Ishii K, Isono M, Hori S, Kinba Y, Mori T (1999) A case of craniopharyngioma with intra tumoral hemorrhage. No Shinkei Geka 27:73–77PubMedGoogle Scholar
  68. 68.
    Kellen RI, Burde RM, Hodges FJ III (1988) Occult pituitary apoplexy associated with craniopharyngioma. J Clin Neuroophthalmol 8:99–104Google Scholar
  69. 69.
    Makwane UK, Singh AK, Puri V, Kumar S (1996) Primary brain tumors presenting as intra cerebral haemorrhage. J Assoc Physicians India 44:729–733PubMedGoogle Scholar
  70. 70.
    Masuda R, Tsukamoto E, Takeda S, Furuichi S, Endo S, Takaku A (1990) An elderly case of recurrent craniopharyngioma suffering from hemorrhage. No Shinkei Geka 18:1151–1155PubMedGoogle Scholar
  71. 71.
    Podgorski JK, Rudnicki SZ, Potakiewicz Z, Delimat L, Siwik JW (1991) A case of intrasellar craniopharyngioma with the symptoms of pituitary apoplexy. Neurol Neurochir Pol 25:689–693PubMedGoogle Scholar
  72. 72.
    Rangel-Castilla L, Rios-Alanis M, Torres-Corzo J, Rodriguez-Della VR, Chavez-Lopez R (2004) Pituitary apoplexy as the presenting symptom of a recurrent craniopharyngioma. Rev Neurol 39:297–298PubMedGoogle Scholar
  73. 73.
    Yamamoto T, Yoneda S, Funatsu N (1989) Spontaneous haemorrhage in craniopharyngioma. J Neurol Neurosurg Psychiatry 52:803–804PubMedCrossRefGoogle Scholar
  74. 74.
    Patrick BS, Smith RR, Bailey TO (1974) Aseptic meningitis due to spontaneous rupture of craniopharyngioma cyst. Case report. J Neurosurg 41:387–390PubMedCrossRefGoogle Scholar
  75. 75.
    Tadmor R, Harwood-Nash DC, Scotti G et al (1982) Intracranial neoplasms in children: the effect of computed tomography on age distribution. Radiology 145:371–373PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • E. H. Nielsen
    • 1
    Email author
  • J. O. Jørgensen
    • 2
  • P. Bjerre
    • 3
  • M. Andersen
    • 4
  • C. Andersen
    • 5
  • U. Feldt-Rasmussen
    • 6
  • L. Poulsgaard
    • 7
  • L. Ø. Kristensen
    • 8
  • J. Astrup
    • 9
  • J. Jørgensen
    • 10
  • P. Laurberg
    • 1
  1. 1.Department of EndocrinologyAalborg Hospital, Aarhus University HospitalAalborgDenmark
  2. 2.Medical Department M (Endocrinology and Diabetes)Aarhus Sygehus, Aarhus University HospitalAarhusDenmark
  3. 3.Department of NeurosurgeryAarhus Sygehus, Aarhus University HospitalAarhusDenmark
  4. 4.Department of EndocrinologyOdense University HospitalCopenhagenDenmark
  5. 5.Department of NeurosurgeryOdense University HospitalCopenhagenDenmark
  6. 6.Department of EndocrinologyRigshospitalet, Copenhagen University HospitalCopenhagenDenmark
  7. 7.Department of NeurosurgeryRigshospitalet, Copenhagen University HospitalCopenhagenDenmark
  8. 8.Department of EndocrinologyHerlev Hospital, University of CopenhagenCopenhagenDenmark
  9. 9.Department of NeurosurgeryGlostrup Hospital, University of CopenhagenCopenhagenDenmark
  10. 10.Department of NeurosurgeryAalborg Hospital, Aarhus University HospitalAalborgDenmark

Personalised recommendations