Sections

Presentation

History

Craniopharyngioma typically is a slow-growing tumor. Symptoms frequently develop insidiously and usually become obvious only after the tumor attains a diameter of about 3cm. The time interval between the onset of symptoms and diagnosis usually ranges from 1-2 years.

The most common presenting symptoms are headache (55-86%), endocrine dysfunction (66-90%), and visual disturbances (37-68%). Headache is slowly progressive, dull, continuous, and positional; it becomes severe in most patients when endocrine symptoms become obvious.

On presentation, 40% of patients have symptoms related to hypothyroidism (i.e., weight gain, fatigue, cold intolerance, constipation). Almost 25% have associated signs and symptoms of adrenal failure (i.e., orthostatic hypotension, hypoglycemia, hyperkalemia, cardiac arrhythmias, lethargy, confusion, anorexia, nausea, and vomiting), and 20% have diabetes insipidus (i.e. excessive fluid intake and urination). Most young patients present with growth failure and delayed puberty.^[21]

As disease progress, 80% of adults complain of decreased sexual drive, and almost 90% of men complain of impotence, while most women complain of amenorrhea.

Optic pathway dysfunction is noted in 40-70% of patients on presentation. Children rarely become aware of visual problems (only 20-30%) and often present after almost complete visual damage becomes irreversible. The manifestation of optic pathway dysfunction usually varies from papilledema to visual field deficits and even optic nerve atrophy in severe cases.

Other manifestations relate to the various connections of the hypothalamic-pituitary complex and surrounding structures. When the thalamus, hypothalamus and frontal lobes are affected, patients experience endocrine, autonomic, and behavioral problems (i.e. hyperphagia and obesity, psychomotor retardation, emotional immaturity, apathy, short-term memory deficits, incontinence).^[22] Short stature is present in 23-45% of patients, and obesity affects 11-18% of patients.^[23]

The following relationships are seen between the anatomic location of the craniopharyngioma and major clinical syndromes:

Prechiasmal localization - Typically results in associated findings of optic atrophy (i.e., progressive decline of visual acuity, constriction of visual fields). The chiasm stretches and gets displaced away from the optic nerve, which eventually causes vascular deprivation of the nerves and damage to nerve fibers.
Retrochiasmal location - Commonly is associated with hydrocephalus with signs of increased intracranial pressure (i.e. papilledema, horizontal double vision). The tumor compresses the floor of the third ventricle and can lead to obstruction of CSF flow through the Foramen of Monroe.
Intrasellar craniopharyngioma - Usually manifests as headache and/or endocrinopathy.

Physical Examination

Neurologic and general examinations are both indicated.

Neurologic examination

Signs suggestive of increased intracranial pressure—horizontal double vision (unilateral/bilateral) and papilledema (unilateral/bilateral)—should be sought for in any patient suspected of having an intracranial mass.

Visual field examination may reveal various patterns of visual loss (most frequently bitemporal hemianopsia) suggestive of involvement (i.e., compression) of the optic chiasm and/or tracts. Formal visual field testing by ophthalmology is recommended as part of the initial work up and serial testing can be used in follow up to monitor tumor growth/recurrence.

General examination

Signs and symptoms may be related to various endocrinopathies.

Hypothyroidism

Symptoms of hypothyroidism include the following:

▪ Puffiness and non-pitting edema

▪ Slow return phase of deep tendon reflexes

▪ Hypoventilation and decrease in cardiac output

▪ Pericardial and pleural effusions

▪ Constipation

▪ Anemia – i.e., normochromic normocytic anemia

▪ Decreased mental function

▪ Psychiatric changes

Cortisol-related deficiency

The signs and symptoms of cortisol deficiency include the following:

Hypotension, which is often orthostatic
Gastrointestinal symptoms, which include anorexia, nausea, and vomiting
Weight loss
Hypoglycemia
Lethargy
Confusion
Psychological disturbances, i.e.. psychosis and intolerance to stress

Changes in volume and sodium control

The signs and symptoms of aldosterone deficiency include the following:

▪ Hypovolemia

▪ Decreased cardiac output

▪ Decreased renal blood flow with azotemia

▪ Fatigue

▪ Weight loss

▪ Cardiac arrhythmias due to hyperkalemia

Compression of the infundibulum can lead to the common presentation of diabetes insipidus.

Differential Diagnoses

Stamm AC, Vellutini E, Balsalobre L. Craniopharyngioma. Otolaryngol Clin North Am. 2011 Aug. 44(4):937-52, viii. [Medline].
Müller HL. Craniopharyngioma. Handb Clin Neurol. 2014. 124:235-53. [Medline].
Jung TY, Jung S, Jang WY, Moon KS, Kim IY, Kang SS. Operative outcomes and adjuvant treatment of purely third ventricle craniopharyngioma after a transcallosal approach. Br J Neurosurg. 2011 Nov 21. [Medline].
Meuric S, Brauner R, Trivin C, et al. Influence of tumor location on the presentation and evolution of craniopharyngiomas. J Neurosurg. 2005 Nov. 103(5 Suppl):421-6. [Medline].
Yoshimoto M, de Toledo SR, da Silva NS, et al. Comparative genomic hybridization analysis of pediatric adamantinomatous craniopharyngiomas and a review of the literature. J Neurosurg. 2004 Aug. 101(1 Suppl):85-90. [Medline].
Rickert CH, Paulus W. Lack of chromosomal imbalances in adamantinomatous and papillary craniopharyngiomas. J Neurol Neurosurg Psychiatry. 2003 Feb. 74(2):260-1. [Medline].
Rienstein S, Adams EF, Pilzer D, et al. Comparative genomic hybridization analysis of craniopharyngiomas. J Neurosurg. 2003 Jan. 98(1):162-4. [Medline].
Oikonomou E, Barreto DC, Soares B, et al. Beta-catenin mutations in craniopharyngiomas and pituitary adenomas. J Neurooncol. 2005 Jul. 73(3):205-9. [Medline].
Sekine S, Shibata T, Kokubu A. Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol. 2002 Dec. 161(6):1997-2001. [Medline].
Sekine S, Takata T, Shibata T, et al. Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma:evidence for its odontogenic epithelial differentiation. Histopathology. 2004 Dec. 45(6):573-9. [Medline].
Hao J, Li TG, Qi X, et al. WNT/beta-catenin pathway up-regulates Stat3 and converges on LIF to prevent differentiation of mouse embryonic stem cells. Dev Biol. 2006 Feb 1. 290(1):81-91. [Medline].
Izumoto S, Suzuki T, Kinoshita M, et al. Immunohistochemical detection of female sex hormone receptors in craniopharyngiomas: correlation with clinical and histologic features. Surg Neurol. 2005 Jun. 63(6):520-5; discussion 525. [Medline].
Ulfarsson E, Karstrom A, Yin S, et al. Expression and growth dependency of the insulin-like growth factor I receptor in craniopharyngioma cells: a novel therapeutic approach. Clin Cancer Res. 2005 Jul 1. 11(13):4674-80. [Medline].
Darendeliler F, Karagiannis G, Wilton P, Ranke MB, Albertsson-Wikland K, Anthony Price D, et al. Recurrence of brain tumours in patients treated with growth hormone: analysis of KIGS (Pfizer International Growth Database). Acta Paediatr. 2006 Oct. 95(10):1284-90. [Medline].
Karavitaki N, Warner JT, Marland A, Shine B, Ryan F, Arnold J, et al. GH replacement does not increase the risk of recurrence in patients with craniopharyngioma. Clin Endocrinol (Oxf). 2006 May. 64(5):556-60. [Medline].
Smith ER, Manfredi M, Scott RM, Black PM, Moses MA. A recurrent craniopharyngioma illustrates the potential usefulness of urinary matrix metalloproteinases as noninvasive biomarkers: case report. Neurosurgery. 2007 Jun. 60(6):E1148-9; discussion E1149. [Medline].
Bunin GR, Surawicz TS, Witman PA, et al. The descriptive epidemiology of craniopharyngioma. J Neurosurg. 1998 Oct. 89(4):547-51. [Medline].
Ostrom QT, Gittleman H, Xu J, Kromer C, Wolinsky Y, Kruchko C et al. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2009-2013. Neuro Oncol. 2016 Oct 1. 18 (suppl_5):v1-v75. [Medline].
Larkin S, Karavitaki N. Recent advances in molecular pathology of craniopharyngioma. F1000Res. 2017. 6:1202. [Medline].
Pereira AM, Schmid EM, Schutte PJ, et al. High prevalence of long-term cardiovascular, neurological and psychosocial morbidity after treatment for craniopharyngioma. Clin Endocrinol (Oxf). 2005 Feb. 62(2):197-204. [Medline].
Piguel X, Abraham P, Bouhours-Nouet N, et al. Impaired aerobic exercise adaptation in children and adolescents with craniopharyngioma is associated with hypothalamic involvement. Eur J Endocrinol. 2011 Nov 17. [Medline].
Sands SA, Milner JS, Goldberg J, et al. Quality of life and behavioral follow-up study of pediatric survivors of craniopharyngioma. J Neurosurg. 2005 Oct. 103(4 Suppl):302-11. [Medline].
Page-Wilson G, Wardlaw SL, Khandji AG, Korner J. Hypothalamic obesity in patients with craniopharyngioma: treatment approaches and the emerging role of gastric bypass surgery. Pituitary. 2011 Sep 22. [Medline].
Lee HJ, Wu CC, Wu HM, Hung SC, Lirng JF, Luo CB, et al. Pretreatment Diagnosis of Suprasellar Papillary Craniopharyngioma and Germ Cell Tumors of Adult Patients. AJNR Am J Neuroradiol. 2014 Oct 22. [Medline].
Kinoshita Y, Tominaga A, Usui S, Kurisu K. A craniopharyngioma with spontaneous involution of a gadolinium-enhanced region on magnetic resonance imaging. Surg Neurol Int. 2014. 5:128. [Medline]. [Full Text].
Mori M, Takeshima H, Kuratsu J. Expression of interleukin-6 in human craniopharyngiomas: a possible inducerof tumor-associated inflammation. Int J Mol Med. 2004 Oct. 14(4):505-9. [Medline].
Cavalheiro S, Dastoli PA, Silva NS, et al. Use of interferon alpha in intratumoral chemotherapy for cystic craniopharyngioma. Childs Nerv Syst. 2005 Aug. 21(8-9):719-24. [Medline].
Waber DP, Pomeroy SL, Chiverton AM, et al. Everyday cognitive function after craniopharyngioma in childhood. Pediatr Neurol. 2006 Jan. 34(1):13-9. [Medline].
Laufer I, Anand VK, Schwartz TH. Endoscopic, endonasal extended transsphenoidal, transplanum transtuberculum approach for resection of suprasellar lesions. J Neurosurg. 2007 Mar. 106(3):400-6. [Medline].
Profka E, Giavoli C, Bergamaschi S, Ferrante E, Malchiodi E, Sala E, et al. Analysis of short- and long-term metabolic effects of growth hormone replacement therapy in adult patients with craniopharyngioma and non-functioning pituitary adenoma. J Endocrinol Invest. 2014 Oct 21. [Medline].
Julow J, Lányi F, Hajda M, Szeifert GT, Viola A, Bálint K, et al. Stereotactic intracavitary irradiation of cystic craniopharyngiomas with yttrium-90 isotope. Prog Neurol Surg. 2007. 20:289-96. [Medline].
Derrey S, Blond S, Reyns N, Touzet G, Carpentier P, Gauthier H, et al. Management of cystic craniopharyngiomas with stereotactic endocavitary irradiation using colloidal 186Re: a retrospective study of 48 consecutive patients. Neurosurgery. 2008 Dec. 63(6):1045-52; discussion 1052-3. [Medline].
Ierardi DF, Fernandes MJ, Silva IR, Thomazini-Gouveia J, Silva NS, Dastoli P, et al. Apoptosis in alpha interferon (IFN-alpha) intratumoral chemotherapy for cystic craniopharyngiomas. Childs Nerv Syst. 2007 Sep. 23(9):1041-6. [Medline].
Savas A, Erdem A, Tun K, Kanpolat Y. Fatal toxic effect of bleomycin on brain tissue after intracystic chemotherapy for a craniopharyngioma: case report. Neurosurgery. 2000 Jan. 46(1):213-6; discussion 216-7. [Medline].
Van den Berge JH, Blaauw G, Breeman WA, et al. Intracavitary brachytherapy of cystic craniopharyngiomas. J Neurosurg. 1992 Oct. 77(4):545-50. [Medline].
Lafay-Cousin L, Bartels U, Raybaud C, Kulkarni AV, Guger S, Huang A, et al. Neuroradiological findings of bleomycin leakage in cystic craniopharyngioma. Report of three cases. J Neurosurg. 2007 Oct. 107(4 Suppl):318-23. [Medline].
Hukin J, Steinbok P, Lafay-Cousin L, Hendson G, Strother D, Mercier C, et al. Intracystic bleomycin therapy for craniopharyngioma in children: the Canadian experience. Cancer. 2007 May 15. 109(10):2124-31. [Medline].
Takahashi H, Yamaguchi F, Teramoto A. Long-term outcome and reconsideration of intracystic chemotherapy with bleomycinfor craniopharyngioma in children. Childs Nerv Syst. 2005 Aug. 21(8-9):701-4. [Medline].
Bishop AJ, Greenfield B, Mahajan A, Paulino AC, Okcu MF, Allen PK et al. 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. 2014 Oct 1. 90 (2):354-61. [Medline].
Kobayashi T, Kida Y, Mori Y, Hasegawa T. Long-term results of gamma knife surgery for the treatment of craniopharyngioma in 98 consecutive cases. J Neurosurg. 2005 Dec. 103(6 Suppl):482-8. [Medline].
Fraioli MF, Moschettoni L, Catena E, Fraioli C. Cystic craniopharyngioma: trans-sphenoidal surgery and intra-cystic apposition of "bleomycin wax". Acta Neurochir (Wien). 2009 Apr 24. [Medline].
Cáceres A. Intracavitary therapeutic options in the management of cystic craniopharyngioma. Childs Nerv Syst. 2005 Aug. 21 (8-9):705-18. [Medline].
Wisoff JH. Commentary: intracystic bleomycin for cystic craniopharyngiomas in children (abridged republication of cochrane systematic review). Neurosurgery. 2012 Nov. 71 (5):E1063-4. [Medline].
Zhang S, Fang Y, Cai BW, Xu JG, You C. Intracystic bleomycin for cystic craniopharyngiomas in children. Cochrane Database Syst Rev. 2016 Jul 14. 7:CD008890. [Medline].
Chamberlain MC. A phase II trial of intra-cerebrospinal fluid alpha interferon in the treatment of neoplastic meningitis. Cancer. 2002 May 15. 94 (10):2675-80. [Medline].
Rodríguez-Villanueva J, McDonnell TJ. Induction of apoptotic cell death in non-melanoma skin cancer by interferon-alpha. Int J Cancer. 1995 Mar 29. 61 (1):110-4. [Medline].
Jakacki RI, Cohen BH, Jamison C, Mathews VP, Arenson E et al. Phase II evaluation of interferon-alpha-2a for progressive or recurrent craniopharyngiomas. J Neurosurg. 2000 Feb. 92 (2):255-60. [Medline].
Liu JK, Sevak IA, Carmel PW, Eloy JA. 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. 2016 Dec. 41 (6):E5. [Medline].
Chung WY, Pan DH, Shiau CY, Guo WY, Wang LW. Gamma knife radiosurgery for craniopharyngiomas. J Neurosurg. 2000 Dec. 93 Suppl 3:47-56. [Medline].
Lee M, Kalani MY, Cheshier S, Gibbs IC, Adler JR, Chang SD. Radiation therapy and CyberKnife radiosurgery in the management of craniopharyngiomas. Neurosurg Focus. 2008. 24(5):E4. [Medline].
de Divitiis E, Cappabianca P, Cavallo LM, Esposito F, de Divitiis O, Messina A. Extended endoscopic transsphenoidal approach for extrasellar craniopharyngiomas. Neurosurgery. 2007 Nov. 61(5 Suppl 2):219-27; discussion 228. [Medline].
Kenning TJ, Beahm DD, Farrell CJ, Schaberg MR, Rosen MR, Evans JJ. Endoscopic endonasal craniopharyngioma resection. J Neurosurg. 2012 Jan. 32(1):E5. [Medline].
Campanini ML, Colli LM, Paixao BM, Cabral TP, Amaral FC, Machado HR et al. CTNNB1 gene mutations, pituitary transcription factors, and MicroRNA expression involvement in the pathogenesis of adamantinomatous craniopharyngiomas. Horm Cancer. 2010 Aug. 1 (4):187-96. [Medline].
Esheba GE, Hassan AA. Comparative immunohistochemical expression of β-catenin, EGFR, ErbB2, and p63 in adamantinomatous and papillary craniopharyngiomas. J Egypt Natl Canc Inst. 2015 Sep. 27 (3):139-45. [Medline].
Zuhur SS, Tanik C, Erol RS, Musluman AM, Kabukcuoglu F, Altuntas Y. Immunohistochemical expression of ErbB2 in adamantinomatous craniopharyngiomas: a possible target for immunotherapy. Turk Neurosurg. 2013. 23 (1):55-60. [Medline].
Buslei R, Nolde M, Hofmann B, Meissner S, Eyupoglu IY, Siebzehnrübl F et al. Common mutations of beta-catenin in adamantinomatous craniopharyngiomas but not in other tumours originating from the sellar region. Acta Neuropathol. 2005 Jun. 109 (6):589-97. [Medline].
Cani CM, Matushita H, Carvalho LR, Soares IC, Brito LP, Almeida MQ et al. PROP1 and CTNNB1 expression in adamantinomatous craniopharyngiomas with or without β-catenin mutations. Clinics (Sao Paulo). 2011. 66 (11):1849-54. [Medline].
Cao J, Lin JP, Yang LX, Chen K, Huang ZS. Expression of aberrant beta-catenin and impaired p63 in craniopharyngiomas. Br J Neurosurg. 2010 Jun. 24 (3):249-56. [Medline].
Hölsken A, Kreutzer J, Hofmann BM, Hans V, Oppel F, Buchfelder M et al. Target gene activation of the Wnt signaling pathway in nuclear beta-catenin accumulating cells of adamantinomatous craniopharyngiomas. Brain Pathol. 2009 Jul. 19 (3):357-64. [Medline].
Hölsken A, Stache C, Schlaffer SM, Flitsch J, Fahlbusch R et al. Adamantinomatous craniopharyngiomas express tumor stem cell markers in cells with activated Wnt signaling: further evidence for the existence of a tumor stem cell niche?. Pituitary. 2014 Dec. 17 (6):546-56. [Medline].
Brastianos PK, Santagata S. ENDOCRINE TUMORS: BRAF V600E mutations in papillary craniopharyngioma. Eur J Endocrinol. 2016 Apr. 174 (4):R139-44. [Medline].
Gump JM, Donson AM, Birks DK, Amani VM, Rao KK, Griesinger AM et al. Identification of targets for rational pharmacological therapy in childhood craniopharyngioma. Acta Neuropathol Commun. 2015 May 21. 3:30. [Medline].
Brastianos PK, Horowitz PM, Santagata S, Jones RT, McKenna A, Getz G, et al. Genomic sequencing of meningiomas identifies oncogenic SMO and AKT1 mutations. Nat Genet. 2013 Mar. 45 (3):285-9. [Medline].
Clark VE, Erson-Omay EZ, Serin A, Yin J, Cotney J, Ozduman K, et al. Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO. Science. 2013 Mar 1. 339 (6123):1077-80. [Medline].
Robinson LC, Santagata S, Hankinson TC. Potential evolution of neurosurgical treatment paradigms for craniopharyngioma based on genomic and transcriptomic characteristics. Neurosurg Focus. 2016 Dec. 41 (6):E3. [Medline].
Hölsken A, Sill M, Merkle J, Schweizer L, Buchfelder M, Flitsch J et al. Adamantinomatous and papillary craniopharyngiomas are characterized by distinct epigenomic as well as mutational and transcriptomic profiles. Acta Neuropathol Commun. 2016 Feb 29. 4:20. [Medline].
Coy S, Du Z, Sheu SH, Woo T, Rodriguez FJ, Kieran MW, et al. Distinct patterns of primary and motile cilia in Rathke's cleft cysts and craniopharyngioma subtypes. Mod Pathol. 2016 Dec. 29 (12):1446-1459. [Medline].
Hölsken A, Gebhardt M, Buchfelder M, Fahlbusch R, Blümcke I, Buslei R. EGFR signaling regulates tumor cell migration in craniopharyngiomas. Clin Cancer Res. 2011 Jul 1. 17 (13):4367-77. [Medline].
Brastianos PK, Taylor-Weiner A, Manley PE, Jones RT, Dias-Santagata D et al. Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas. Nat Genet. 2014 Feb. 46 (2):161-5. [Medline].
Kim JH, Paulus W, Heim S. BRAF V600E mutation is a useful marker for differentiating Rathke's cleft cyst with squamous metaplasia from papillary craniopharyngioma. J Neurooncol. 2015 May. 123 (1):189-91. [Medline].
Schweizer L, Capper D, Hölsken A, Fahlbusch R, Flitsch J, Buchfelder M, et al. BRAF V600E analysis for the differentiation of papillary craniopharyngiomas and Rathke's cleft cysts. Neuropathol Appl Neurobiol. 2015 Oct. 41 (6):733-42. [Medline].
Jones RT, Abedalthagafi MS, Brahmandam M, Greenfield EA, Hoang MP et al. Cross-reactivity of the BRAF VE1 antibody with epitopes in axonemal dyneins leads to staining of cilia. Mod Pathol. 2015 Apr. 28 (4):596-606. [Medline].
Maiter D, Abs R, Johannsson G, Scanlon M, Jönsson PJ, Wilton P, et al. Baseline characteristics and response to GH replacement of hypopituitary patients previously irradiated for pituitary adenoma or craniopharyngioma: data from the Pfizer International Metabolic Database. Eur J Endocrinol. 2006 Aug. 155(2):253-60. [Medline].
Curtis J, Daneman D, Hoffman HJ, Ehrlich RM. The endocrine outcome after surgical removal of craniopharyngiomas. Pediatr Neurosurg. 1994. 21 Suppl 1:24-7. [Medline].
Gupta DK, Ojha BK, Sarkar C, Mahapatra AK, Mehta VS. Recurrence in craniopharyngiomas: analysis of clinical and histological features. J Clin Neurosci. 2006 May. 13(4):438-42. [Medline].
Hofmann BM, Höllig A, Strauss C, Buslei R, Buchfelder M, Fahlbusch R. Results after treatment of craniopharyngiomas: further experiences with 73 patients since 1997. J Neurosurg. 2012 Feb. 116(2):373-84. [Medline].
Jeon C, Kim S, Shin HJ, Nam DH, Lee JI, Park K, et al. The therapeutic efficacy of fractionated radiotherapy and gamma-knife radiosurgery for craniopharyngiomas. J Clin Neurosci. 2011 Dec. 18(12):1621-5. [Medline].
Larkin SJ, Preda V, Karavitaki N, Grossman A, Ansorge O. BRAF V600E mutations are characteristic for papillary craniopharyngioma and may coexist with CTNNB1-mutated adamantinomatous craniopharyngioma. Acta Neuropathol. 2014. 127 (6):927-9. [Medline].
Kato K, Nakatani Y, Kanno H, Inayama Y, Ijiri R, Nagahara N et al. Possible linkage between specific histological structures and aberrant reactivation of the Wnt pathway in adamantinomatous craniopharyngioma. J Pathol. 2004 Jul. 203 (3):814-21. [Medline].
Sekine S, Shibata T, Kokubu A, Morishita Y, Noguchi M et al. Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol. 2002 Dec. 161 (6):1997-2001. [Medline].
Katoh Y, Katoh M. Hedgehog target genes: mechanisms of carcinogenesis induced by aberrant hedgehog signaling activation. Curr Mol Med. 2009 Sep. 9 (7):873-86. [Medline].
Kato K, Nakatani Y, Kanno H, Inayama Y, Ijiri R, Nagahara N, et al. Possible linkage between specific histological structures and aberrant reactivation of the Wnt pathway in adamantinomatous craniopharyngioma. J Pathol. 2004 Jul. 203 (3):814-21. [Medline].
Gump JM, Donson AM, Birks DK, Amani VM, Rao KK, Griesinger AM et al. Identification of targets for rational pharmacological therapy in childhood craniopharyngioma. Acta Neuropathol Commun. 2015 May 21. 3:30. [Medline].
Larkin SJ, Ansorge O. Pathology and pathogenesis of craniopharyngiomas. Pituitary. 2013 Mar. 16 (1):9-17. [Medline].

Media Gallery

The adamantinomatous craniopharyngioma is a histologically complex epithelial lesion with several very distinctive morphologic features (hematoxylin-eosin, x40).
Adamantinomatous craniopharyngiomas. Peripheral palisading of the epithelium is a pronounced feature (hematoxylin-eosin, x100).
Adamantinomatous craniopharyngiomas. Frequently, the inner epithelium beneath the superficial palisade undergoes hydropic vacuolization and is referred to as the stellate reticulum (hematoxylin-eosin, x100).
Adamantinomatous craniopharyngiomas. Another distinctive feature of the adamantinomatous variant is scattered nodules of keratin. These nodules are referred to as "wet" keratin because of the plump appearance of the keratinocytes; this is in contrast to the flat, flaky keratin seen in epidermoid and dermoid cysts (hematoxylin-eosin, x100).
Adamantinomatous craniopharyngiomas. Nodules of "wet" keratin frequently calcify; in aggregate, this calcification often can be detected on CT scans and is a recognized radiologic feature of craniopharyngiomas (hematoxylin-eosin, x100).
Papillary craniopharyngioma. In contrast to the adamantinomatous variant, papillary craniopharyngiomas do not show complex heterogeneous architecture but rather are composed of simple squamous epithelium and fibrovascular islands of connective tissue (hematoxylin-eosin, x40).
Papillary craniopharyngiomas. Under high power, only simple squamous epithelium is seen in a papillary craniopharyngioma. The distinctive peripheral nuclear palisading, internal stellate reticulum, and nodules of "wet" keratin, which typify the adamantinomatous variant, are not seen in the papillary variant (hematoxylin-eosin, x100).
Rosenthal fibers in neuropils surrounding a craniopharyngioma. The brain parenchyma that surrounds both variants of craniopharyngioma is typically gliotic and often shows profuse numbers of eosinophilic Rosenthal fibers. The latter structures are composed of densely compacted bundles of glial filaments and typically are seen in astrocytic cell processes of neuropils that have been subjected to chronic compression from slowly expanding mass lesions. Rosenthal fibers are a characteristic feature of juvenile pilocytic astrocytomas (JPAs), which also may arise in the suprasellar/third ventricular region. Hence, a biopsy that samples only the surrounding neuropil of a craniopharyngioma may yield an erroneous diagnosis of JPA if the pathologist is unaware of the close association of craniopharyngioma with Rosenthal fiber formation (hematoxylin-eosin, x100).
T1-weighted MRI with gadolinium in sagittal (A) and coronal (B) views demonstrates the cystic nature of a craniopharyngioma. The calcified component is evident on axial CT imaging (C).
T1-weighted MRI with gadolinium reveals a large cystic craniopharyngioma in sagittal (A), axial (B), and coronal (C) views. There is associated elevation of the optic apparatus and displacement of the pituitary stalk.
Coronal views of T1-weighted MRI for a patient with craniopharyngioma before gross total resection (A) and at postoperative follow-up evaluation (B). There was no sign of tumor recurrence, and the patient was neurologically and endocrinologically intact.

of 11

Author

George I Jallo, MD Professor of Neurosurgery, Pediatrics, and Oncology, Director, Clinical Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine

George I Jallo, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Society of Pediatric Neurosurgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Franco DeMonte, MD, FRCSC, FACS Professor of Neurosurgery, Mary Beth Pawelek Chair in Neurosurgery, The University of Texas MD Anderson Cancer Center

Franco DeMonte, MD, FRCSC, FACS is a member of the following medical societies: American Academy of Neurological Surgery, American College of Surgeons, North American Skull Base Society, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Gregory N Fuller, MD, PhD Professor of Pathology, Chief, Section of Neuropathology, Department of Pathology, Division of Pathology and Laboratory Medicine, The University of Texas MD Anderson Cancer Center

Gregory N Fuller, MD, PhD is a member of the following medical societies: American Association of Neuropathologists, College of American Pathologists, International Academy of Pathology, Society for Neuro-Oncology, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Morris D Groves, MD, JD Associate Professor, Department of Neuro-Oncology, Division of Cancer Medicine, Associate Clinic Director for the Brain and Spine Center, The University of Texas MD Anderson Cancer Center

Morris D Groves, MD, JD is a member of the following medical societies: American Academy of Neurology, American Bar Association, American Medical Association, European Association of Neuro-Oncology, Harris County Medical Society, Houston Neurological Society, Society for Neuro-Oncology, Texas Medical Association, Texas Neurological Society

Disclosure: Received grant/research funds from Genentech for other; Received honoraria from Genentech for consulting; Received grant/research funds from GlaxoSmithKline for other; Received grant/research funds from AngioChem for other; Received grant/research funds from Pfizer/Celldex Therapeautics for other.

Meleine M Martinez-Sosa, MD Resident Physician, Department of Neurosurgery and Brain Repair, University of South Florida Morsani College of Medicine

Disclosure: Nothing to disclose.

Nir Shimony, MD Director of Pediatric Neurosurgery, Director of Epilepsy Surgery, Geisinger Health System

Nir Shimony, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Epilepsy Society, Congress of Neurological Surgeons, Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Chief Editor

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS † Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, American College of International Physicians, American College of Physicians, American Heart Association, American Stroke Association, National Association of Managed Care Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

George C Bobustuc, MD Consulting Staff, Department of Neuro-oncology, MD Anderson Cancer Center of Orlando

George C Bobustuc, MD is a member of the following medical societies: American Academy of Neurology, Texas Medical Association, Society for Neuro-Oncology, American Medical Association

Disclosure: Nothing to disclose.

Lee S Hwang University of Texas Southwestern Medical School

Disclosure: Nothing to disclose.

Acknowledgements

Jorge Kattah, MD Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Amy A Pruitt, MD Associate Professor of Neurology, University of Pennsylvania; Attending Neurologist, Hospital of the University of Pennsylvania

Amy A Pruitt, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Craniopharyngioma Clinical Presentation

History

Physical Examination

Neurologic examination

General examination

References

Tables

Contributor Information and Disclosures

What would you like to print?