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Childhood Craniopharyngioma Treatment (PDQ®)

  • Last Modified: 08/12/2014

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General Information

The PDQ childhood brain tumor treatment summaries are organized primarily according to the World Health Organization classification of nervous system tumors.[1,2] For a full description of the classification of nervous system tumors and a link to the corresponding treatment summary for each type of brain tumor, refer to the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview.

Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality has decreased by more than 50%.[3] Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)

Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification.

References
  1. Louis DN, Ohgaki H, Wiestler OD, et al., eds.: WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon, France: IARC Press, 2007. 

  2. Louis DN, Ohgaki H, Wiestler OD, et al.: The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114 (2): 97-109, 2007.  [PUBMED Abstract]

  3. Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014.  [PUBMED Abstract]

Background Information About Childhood Craniopharyngioma



Incidence and Presentation

Craniopharyngiomas are relatively rare pediatric tumors, accounting for about 6% of all intracranial tumors in children.[1-3] They are believed to be congenital in origin, arising from ectodermal remnants, Rathke cleft, or other embryonal epithelium in the sellar and/or parasellar area. No predisposing factors have been identified.

Because craniopharyngiomas occur in the region of the pituitary gland, endocrine function and growth may be affected. Additionally, the close proximity of the tumor to the optic nerves and chiasm may result in vision problems. Some patients present with obstructive hydrocephalus due to tumor obstruction of the third ventricle. Extremely rarely, the tumor may predominate in the posterior fossa with presenting symptoms of headache, diplopia, ataxia, and hearing loss.[4]

Prognosis

Regardless of the treatment modality, long-term survival is approximately 85% in children [2,3] with 5- and 10-year overall survival rates greater than 90%.[5-8]

References
  1. Bunin GR, Surawicz TS, Witman PA, et al.: The descriptive epidemiology of craniopharyngioma. J Neurosurg 89 (4): 547-51, 1998.  [PUBMED Abstract]

  2. Karavitaki N, Wass JA: Craniopharyngiomas. Endocrinol Metab Clin North Am 37 (1): 173-93, ix-x, 2008.  [PUBMED Abstract]

  3. Garnett MR, Puget S, Grill J, et al.: Craniopharyngioma. Orphanet J Rare Dis 2: 18, 2007.  [PUBMED Abstract]

  4. Zhou L, Luo L, Xu J, et al.: Craniopharyngiomas in the posterior fossa: a rare subgroup, diagnosis, management and outcomes. J Neurol Neurosurg Psychiatry 80 (10): 1150-4, 2009.  [PUBMED Abstract]

  5. Muller HL: Childhood craniopharyngioma. Recent advances in diagnosis, treatment and follow-up. Horm Res 69 (4): 193-202, 2008.  [PUBMED Abstract]

  6. Müller HL: Childhood craniopharyngioma--current concepts in diagnosis, therapy and follow-up. Nat Rev Endocrinol 6 (11): 609-18, 2010.  [PUBMED Abstract]

  7. Sanford RA, Muhlbauer MS: Craniopharyngioma in children. Neurol Clin 9 (2): 453-65, 1991.  [PUBMED Abstract]

  8. Zacharia BE, Bruce SS, Goldstein H, et al.: Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program. Neuro Oncol 14 (8): 1070-8, 2012.  [PUBMED Abstract]

Histopathologic Classification of Childhood Craniopharyngioma

Craniopharyngiomas are histologically benign and do not metastasize to remote brain locations or to areas outside the sellar region except by direct extension. They may be invasive, however, and may recur locally. They may be classified as adamantinomatous or squamous papillary, with the former being the predominant form in children.[1] They are typically composed of both a solid portion with an abundance of calcification, and a cystic component which is filled with a dark, oily fluid. Recent evidence suggests that adamantinomatous craniopharyngiomas are locally more aggressive with a significantly higher rate of recurrence compared with the squamous papillary subtype.[2]

The molecular basis for craniopharyngioma differs by histologic subtype. Activating beta-catenin gene mutations are found in virtually all adamantinomatous tumors.[3,4] Conversely, BRAF V600E mutations are observed in nearly all squamous papillary craniopharyngiomas.[4]

References
  1. Karavitaki N, Wass JA: Craniopharyngiomas. Endocrinol Metab Clin North Am 37 (1): 173-93, ix-x, 2008.  [PUBMED Abstract]

  2. Pekmezci M, Louie J, Gupta N, et al.: Clinicopathological characteristics of adamantinomatous and papillary craniopharyngiomas: University of California, San Francisco experience 1985-2005. Neurosurgery 67 (5): 1341-9; discussion 1349, 2010.  [PUBMED Abstract]

  3. Sekine S, Shibata T, Kokubu A, et al.: Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol 161 (6): 1997-2001, 2002.  [PUBMED Abstract]

  4. Brastianos PK, Taylor-Weiner A, Manley PE, et al.: Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas. Nat Genet 46 (2): 161-5, 2014.  [PUBMED Abstract]

Diagnostic Evaluation of Childhood Craniopharyngioma

The results of imaging studies (computerized tomography scans and magnetic resonance imaging [MRI] scans) are often diagnostic for childhood craniopharyngiomas, with most demonstrating intratumoral calcifications and a solid and cystic component. The most common location is suprasellar, with an intrasellar portion. Craniopharyngiomas without calcification may be confused with other tumor types, such as germinoma or hypothalamic/chiasmatic astrocytoma, and biopsy may be required.[1] MRI of the spinal axis is not routinely performed.

Apart from imaging, patients often undergo formal visual examination including visual field evaluation and endocrine testing.

References
  1. Rossi A, Cama A, Consales A, et al.: Neuroimaging of pediatric craniopharyngiomas: a pictorial essay. J Pediatr Endocrinol Metab 19 (Suppl 1): 299-319, 2006.  [PUBMED Abstract]

Stage Information

There is no generally applied staging system for childhood craniopharyngiomas. For treatment purposes, patients are classified as having newly diagnosed or recurrent disease.

Treatment Options for Newly Diagnosed Childhood Craniopharyngioma

There is no consensus as to the optimal treatment of newly diagnosed craniopharyngioma, in part due to the lack of prospective randomized trials comparing different treatment options. A systematic review of 109 reports that described extent of resection found that subtotal resection plus radiation therapy was associated with similar rates of tumor control as gross total resection and that both approaches were associated with higher progression-free survival (PFS) rates than subtotal resection alone.[1][Level of evidence: 3iiiDiii] Treatment is individualized based on factors such as the size, location, and extension of the tumor and potential short-term and long-term toxicity.

Radical Surgery

Because these tumors are histologically benign, it may be possible to remove all the visible tumor resulting in long-term disease control.[2][Level of evidence: 3iA]; [3][Level of evidence: 3iiiB]; [4][Level of evidence: 3iiiC] A 5-year PFS rate of about 65% has been reported.[5] Many surgical approaches have been described, and the route should be determined by the size, location, and extension of the tumor. A transsphenoidal approach may be possible in some small tumors located entirely within the sella,[6][Level of evidence: 3iiiC] but this is not usually possible in children, in which case a craniotomy is usually required.

Gross total resection is technically challenging because the tumor is surrounded by vital structures, including the optic nerves and chiasm, the carotid artery and its branches, the hypothalamus, and the third cranial nerve. The tumor may be adherent to these structures, which may cause complications, and may limit the ability to remove the entire tumor. The surgeon often has limited visibility in the region of the hypothalamus and in the sella, and portions of the mass may be left in these areas, accounting for some recurrences. Almost all craniopharyngiomas have an attachment to the pituitary stalk, and of the patients who undergo radical surgery, virtually all will require life-long pituitary hormone replacement with multiple medications.[3,7]

Complications of radical surgery include the need for hormone replacement, obesity (which can be life threatening),[8] severe behavioral problems,[9] blindness, seizures, spinal fluid leak, false aneurysms, and difficulty with eye movements. Rare complications include death from intraoperative hemorrhage, hypothalamic damage, or stroke. Hypothalamic-sparing surgical techniques may show a decrease in severe postoperative obesity without an increase in tumor recurrence.[10][Level of evidence: 3iiDi]

If the surgeon feels that tumor remains, or if postoperative imaging reveals residual craniopharyngioma that was not resected, radiation therapy may be recommended to prevent early progression.[11][Level of evidence: 3iiiDiii] It can be difficult to determine whether a tumor is progressive; carbon-11 methionine positron emission tomography is being evaluated for its use in these cases.[12] Periodic surveillance magnetic resonance imaging is performed for several years after radical surgery because of the possibility of tumor recurrence.

Surgery with Cyst Drainage

For large cystic craniopharyngiomas, particularly in children younger than 3 years and in those with recurrent cystic tumor after initial surgery, stereotactic or open implantation of an intracystic catheter with a subcutaneous reservoir may be a valuable alternative treatment option. The benefits of this procedure include temporary relief of fluid pressure by serial drainage, and in some cases, for intracystic instillation of sclerosing agents as a means to prolong the interval to or obviate the need for radiation. This procedure may also be helpful in allowing the surgeon to perform a two-staged approach, whereby first the cyst is drained by the implanted catheter to relieve pressure and complicating symptoms, followed by tumor resection.[13]

Limited Surgery and Radiation Therapy

The goal of limited surgery is to establish a diagnosis, drain any cysts, and decompress the optic nerves. No attempt is made to remove tumor from the pituitary stalk or hypothalamus in an effort to minimize certain late effects associated with radical surgery.[10] The surgical procedure is followed by radiation therapy, with a 5-year PFS rate of about 70% to 90% [5,14]; [15][Level of evidence: 3iDiii] and 10-year overall survival rates higher than 90%.[16][Level of evidence: 3iiA]; [1][Level of evidence: 3iiiDiii] Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention.[17][Level of evidence: 3iDiv] Conventional radiation is fractionated external-beam radiation with a recommended dose of 54 Gy to 55 Gy in 1.8 Gy fractions.[18] Surgical complications are less likely than with radical surgery. Complications of radiation include loss of pituitary hormonal function, cognitive dysfunction, development of late strokes and vascular malformations, delayed blindness, development of second tumors, and, rarely, malignant transformation of the primary tumor within the radiation field.[19,20] Newer radiation technologies such as intensity-modulated proton therapy may reduce scatter whole-brain and whole-body irradiation and result in the sparing of normal tissues. It is unknown whether such technologies result in decreased late effects from irradiation.[15,21,22] Tumor progression remains a possibility, and it is usually not possible to repeat the radiation dose. In selected cases, stereotactic radiation therapy can be delivered as a single large dose of radiation to a very small field.[23][Level of evidence: 3iC] Proximity of the craniopharyngioma to vital structures, particularly the optic nerves, limits this to very small tumors that are in the sella.[24][Level of evidence: 3iiiDiii]

Intracavitary Radiation Therapy and/or Chemotherapy

Some craniopharyngiomas with a large cystic component may be treated by stereotaxic delivery of P-32 or other radioactive compounds.[25,26]; [27][Level of evidence: 2A]; [28][Level of evidence: 3iiiDiii] Nonradioactive agents such as bleomycin and interferon-alpha have also been used.[29-31]; [32][Level of evidence: 2C] These strategies have been found to be useful in certain cases and are with low reported risk of complications. However, none have shown efficacy against solid portions of the tumor.

References
  1. Clark AJ, Cage TA, Aranda D, et al.: A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma. Childs Nerv Syst 29 (2): 231-8, 2013.  [PUBMED Abstract]

  2. Mortini P, Losa M, Pozzobon G, et al.: Neurosurgical treatment of craniopharyngioma in adults and children: early and long-term results in a large case series. J Neurosurg 114 (5): 1350-9, 2011.  [PUBMED Abstract]

  3. Elliott RE, Hsieh K, Hochm T, et al.: Efficacy and safety of radical resection of primary and recurrent craniopharyngiomas in 86 children. J Neurosurg Pediatr 5 (1): 30-48, 2010.  [PUBMED Abstract]

  4. Zhang YQ, Ma ZY, Wu ZB, et al.: Radical resection of 202 pediatric craniopharyngiomas with special reference to the surgical approaches and hypothalamic protection. Pediatr Neurosurg 44 (6): 435-43, 2008.  [PUBMED Abstract]

  5. Yang I, Sughrue ME, Rutkowski MJ, et al.: Craniopharyngioma: a comparison of tumor control with various treatment strategies. Neurosurg Focus 28 (4): E5, 2010.  [PUBMED Abstract]

  6. Locatelli D, Massimi L, Rigante M, et al.: Endoscopic endonasal transsphenoidal surgery for sellar tumors in children. Int J Pediatr Otorhinolaryngol 74 (11): 1298-302, 2010.  [PUBMED Abstract]

  7. Sands SA, Milner JS, Goldberg J, et al.: Quality of life and behavioral follow-up study of pediatric survivors of craniopharyngioma. J Neurosurg 103 (4 Suppl): 302-11, 2005.  [PUBMED Abstract]

  8. Müller HL, Gebhardt U, Teske C, et al.: 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 (1): 17-24, 2011.  [PUBMED Abstract]

  9. Clark AJ, Cage TA, Aranda D, et al.: Treatment-related morbidity and the management of pediatric craniopharyngioma: a systematic review. J Neurosurg Pediatr 10 (4): 293-301, 2012.  [PUBMED Abstract]

  10. Elowe-Gruau E, Beltrand J, Brauner R, et al.: Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity. J Clin Endocrinol Metab 98 (6): 2376-82, 2013.  [PUBMED Abstract]

  11. Lin LL, El Naqa I, Leonard JR, et al.: Long-term outcome in children treated for craniopharyngioma with and without radiotherapy. J Neurosurg Pediatr 1 (2): 126-30, 2008.  [PUBMED Abstract]

  12. Laser BS, Merchant TE, Indelicato DJ, et al.: Evaluation of children with craniopharyngioma using carbon-11 methionine PET prior to proton therapy. Neuro Oncol 15 (4): 506-10, 2013.  [PUBMED Abstract]

  13. Schubert T, Trippel M, Tacke U, et al.: Neurosurgical treatment strategies in childhood craniopharyngiomas: is less more? Childs Nerv Syst 25 (11): 1419-27, 2009.  [PUBMED Abstract]

  14. Winkfield KM, Tsai HK, Yao X, et al.: Long-term clinical outcomes following treatment of childhood craniopharyngioma. Pediatr Blood Cancer 56 (7): 1120-6, 2011.  [PUBMED Abstract]

  15. Merchant TE, Kun LE, Hua CH, et al.: Disease control after reduced volume conformal and intensity modulated radiation therapy for childhood craniopharyngioma. Int J Radiat Oncol Biol Phys 85 (4): e187-92, 2013.  [PUBMED Abstract]

  16. Schoenfeld A, Pekmezci M, Barnes MJ, et al.: The superiority of conservative resection and adjuvant radiation for craniopharyngiomas. J Neurooncol 108 (1): 133-9, 2012.  [PUBMED Abstract]

  17. Shi Z, Esiashvili N, Janss AJ, et al.: Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation. J Neurooncol 109 (2): 349-55, 2012.  [PUBMED Abstract]

  18. Kiehna EN, Merchant TE: Radiation therapy for pediatric craniopharyngioma. Neurosurg Focus 28 (4): E10, 2010.  [PUBMED Abstract]

  19. Ishida M, Hotta M, Tsukamura A, et al.: Malignant transformation in craniopharyngioma after radiation therapy: a case report and review of the literature. Clin Neuropathol 29 (1): 2-8, 2010 Jan-Feb.  [PUBMED Abstract]

  20. Aquilina K, Merchant TE, Rodriguez-Galindo C, et al.: Malignant transformation of irradiated craniopharyngioma in children: report of 2 cases. J Neurosurg Pediatr 5 (2): 155-61, 2010.  [PUBMED Abstract]

  21. Beltran C, Roca M, Merchant TE: On the benefits and risks of proton therapy in pediatric craniopharyngioma. Int J Radiat Oncol Biol Phys 82 (2): e281-7, 2012.  [PUBMED Abstract]

  22. Boehling NS, Grosshans DR, Bluett JB, et al.: 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): 643-52, 2012.  [PUBMED Abstract]

  23. Kobayashi T: Long-term results of gamma knife radiosurgery for 100 consecutive cases of craniopharyngioma and a treatment strategy. Prog Neurol Surg 22: 63-76, 2009.  [PUBMED Abstract]

  24. Hasegawa T, Kobayashi T, Kida Y: Tolerance of the optic apparatus in single-fraction irradiation using stereotactic radiosurgery: evaluation in 100 patients with craniopharyngioma. Neurosurgery 66 (4): 688-94; discussion 694-5, 2010.  [PUBMED Abstract]

  25. Julow J, Backlund EO, Lányi F, et al.: Long-term results and late complications after intracavitary yttrium-90 colloid irradiation of recurrent cystic craniopharyngiomas. Neurosurgery 61 (2): 288-95; discussion 295-6, 2007.  [PUBMED Abstract]

  26. Barriger RB, Chang A, Lo SS, et al.: Phosphorus-32 therapy for cystic craniopharyngiomas. Radiother Oncol 98 (2): 207-12, 2011.  [PUBMED Abstract]

  27. Kickingereder P, Maarouf M, El Majdoub F, et al.: Intracavitary brachytherapy using stereotactically applied phosphorus-32 colloid for treatment of cystic craniopharyngiomas in 53 patients. J Neurooncol 109 (2): 365-74, 2012.  [PUBMED Abstract]

  28. Zhao R, Deng J, Liang X, et al.: Treatment of cystic craniopharyngioma with phosphorus-32 intracavitary irradiation. Childs Nerv Syst 26 (5): 669-74, 2010.  [PUBMED Abstract]

  29. Ierardi DF, Fernandes MJ, Silva IR, et al.: Apoptosis in alpha interferon (IFN-alpha) intratumoral chemotherapy for cystic craniopharyngiomas. Childs Nerv Syst 23 (9): 1041-6, 2007.  [PUBMED Abstract]

  30. Linnert M, Gehl J: Bleomycin treatment of brain tumors: an evaluation. Anticancer Drugs 20 (3): 157-64, 2009.  [PUBMED Abstract]

  31. Steinbok P, Hukin J: Intracystic treatments for craniopharyngioma. Neurosurg Focus 28 (4): E13, 2010.  [PUBMED Abstract]

  32. Cavalheiro S, Di Rocco C, Valenzuela S, et al.: Craniopharyngiomas: intratumoral chemotherapy with interferon-alpha: a multicenter preliminary study with 60 cases. Neurosurg Focus 28 (4): E12, 2010.  [PUBMED Abstract]

Treatment Options for Recurrent Childhood Craniopharyngioma

Recurrence of craniopharyngioma occurs in approximately 35% of patients regardless of primary therapy.[1] Management is determined in large part by prior therapy. Repeat attempts at gross total resection are difficult and long-term disease control is less often achieved.[2][Level of evidence: 3iiiDi] Complications are more frequent than with initial surgery.[3][Level of evidence: 3iiiDi] External-beam radiation therapy is an option if this has not been previously employed, including consideration of radiosurgery in selected circumstances.[4][Level of evidence: 3iiiDiii] Cystic recurrences may be treated with intracavitary instillation of radioactive P-32, bleomycin,[5][Level of evidence: 3iiiDiii] or interferon-alpha,[6][Level of evidence: 3iiiB] and a reservoir may be placed to permit intermittent outpatient aspiration. Although systemic therapy is generally not utilized, a small series has shown that the use of subcutaneous pegylated interferon alpha-2b to manage cystic recurrences can result in durable responses.[7][Level of evidence: 3iiiDiii]

Treatment Options Under Clinical Evaluation

The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.

  • PBTC-039 (NCT01964300) (Peginterferon Alfa-2b in Treating Younger Patients With Craniopharyngioma That is Recurrent or Cannot Be Removed By Surgery): This is a phase II clinical trial evaluating how well peginterferon alfa-2b works in treating children with craniopharyngioma that is recurrent or cannot be removed by surgery. This trial follows a small (N = 5) single-institution experience with peginterferon alfa-2b, in which prolonged complete responses were observed in some patients.[7]
References
  1. Yang I, Sughrue ME, Rutkowski MJ, et al.: Craniopharyngioma: a comparison of tumor control with various treatment strategies. Neurosurg Focus 28 (4): E5, 2010.  [PUBMED Abstract]

  2. Vinchon M, Dhellemmes P: Craniopharyngiomas in children: recurrence, reoperation and outcome. Childs Nerv Syst 24 (2): 211-7, 2008.  [PUBMED Abstract]

  3. Jang WY, Lee KS, Son BC, et al.: Repeat operations in pediatric patients with recurrent craniopharyngiomas. Pediatr Neurosurg 45 (6): 451-5, 2009.  [PUBMED Abstract]

  4. Xu Z, Yen CP, Schlesinger D, et al.: Outcomes of Gamma Knife surgery for craniopharyngiomas. J Neurooncol 104 (1): 305-13, 2011.  [PUBMED Abstract]

  5. Hukin J, Steinbok P, Lafay-Cousin L, et al.: Intracystic bleomycin therapy for craniopharyngioma in children: the Canadian experience. Cancer 109 (10): 2124-31, 2007.  [PUBMED Abstract]

  6. Cavalheiro S, Di Rocco C, Valenzuela S, et al.: Craniopharyngiomas: intratumoral chemotherapy with interferon-alpha: a multicenter preliminary study with 60 cases. Neurosurg Focus 28 (4): E12, 2010.  [PUBMED Abstract]

  7. Yeung JT, Pollack IF, Panigrahy A, et al.: Pegylated interferon-α-2b for children with recurrent craniopharyngioma. J Neurosurg Pediatr 10 (6): 498-503, 2012.  [PUBMED Abstract]

Late Effects in Patients Treated for Childhood Craniopharyngioma

Quality-of-life issues are important in this group of patients and are difficult to generalize due to various treatment modalities. Whereas intelligence quotient is usually maintained, behavioral issues and memory deficits attributed to the frontal lobe and hypothalamus are common.[1] Patients with hypothalamic involvement showed impairment in memory and executive functioning.[2] Other common problems include visual loss, obesity (which can be life threatening),[3] and the almost universal need for life-long endocrine replacement with multiple pituitary hormones.[4-6][Level of evidence: 3iiiC] Vasculopathies and secondary tumors may also result from local irradiation.[7] A recent report indicated that adults on long-term growth hormone replacement secondary to childhood craniopharyngioma involving the hypothalamus were at increased cardiovascular risk.[8]

Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.

References
  1. Winkfield KM, Tsai HK, Yao X, et al.: Long-term clinical outcomes following treatment of childhood craniopharyngioma. Pediatr Blood Cancer 56 (7): 1120-6, 2011.  [PUBMED Abstract]

  2. Özyurt J, Thiel CM, Lorenzen A, et al.: Neuropsychological outcome in patients with childhood craniopharyngioma and hypothalamic involvement. J Pediatr 164 (4): 876-881.e4, 2014.  [PUBMED Abstract]

  3. Elowe-Gruau E, Beltrand J, Brauner R, et al.: Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity. J Clin Endocrinol Metab 98 (6): 2376-82, 2013.  [PUBMED Abstract]

  4. Vinchon M, Weill J, Delestret I, et al.: Craniopharyngioma and hypothalamic obesity in children. Childs Nerv Syst 25 (3): 347-52, 2009.  [PUBMED Abstract]

  5. Dolson EP, Conklin HM, Li C, et al.: Predicting behavioral problems in craniopharyngioma survivors after conformal radiation therapy. Pediatr Blood Cancer 52 (7): 860-4, 2009.  [PUBMED Abstract]

  6. Kawamata T, Amano K, Aihara Y, et al.: Optimal treatment strategy for craniopharyngiomas based on long-term functional outcomes of recent and past treatment modalities. Neurosurg Rev 33 (1): 71-81, 2010.  [PUBMED Abstract]

  7. Kiehna EN, Merchant TE: Radiation therapy for pediatric craniopharyngioma. Neurosurg Focus 28 (4): E10, 2010.  [PUBMED Abstract]

  8. Holmer H, Ekman B, Björk J, et al.: Hypothalamic involvement predicts cardiovascular risk in adults with childhood onset craniopharyngioma on long-term GH therapy. Eur J Endocrinol 161 (5): 671-9, 2009.  [PUBMED Abstract]

Changes to This Summary (08/12/2014)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

General Information

Revised text to state that between 1975 and 2010, childhood cancer mortality has decreased by more than 50% (cited Smith et al. as reference 3).

Stage Information

Revised text to state that for treatment purposes, patients are classified as having newly diagnosed or recurrent disease.

Treatment Options for Newly Diagnosed Childhood Craniopharyngioma

Revised text to state that there is no consensus as to the optimal treatment of newly diagnosed craniopharyngioma, in part due to the lack of prospective randomized trials comparing different treatment options. Also added text to state that a systematic review of 109 reports that described extent of resection found that subtotal resection plus radiation therapy was associated with similar rates of tumor control as gross total resection and that both approaches were associated with higher progression-free survival rates than subtotal resection; treatment is individualized based on factors such as the size, location, and extension of the tumor and potential short-term and long-term toxicity.

Late Effects in Patients Treated for Childhood Craniopharyngioma

Added text to state that patients with hypothalamic involvement showed impairment in memory and executive functioning (cited Özyurt et al. as reference 2).

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.

About This PDQ Summary



Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood craniopharyngioma. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

  • be discussed at a meeting,
  • be cited with text, or
  • replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Childhood Craniopharyngioma Treatment are:

  • Kenneth J. Cohen, MD, MBA (Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Hospital)
  • Karen J Marcus, MD (Dana-Farber Cancer Institute/Boston Children's Hospital)
  • Roger J. Packer, MD (Children's National Medical Center)
  • Malcolm A. Smith, MD, PhD (National Cancer Institute)

Any comments or questions about the summary content should be submitted to Cancer.gov through the Web site's Contact Form. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

National Cancer Institute: PDQ® Childhood Craniopharyngioma Treatment. Bethesda, MD: National Cancer Institute. Date last modified <MM/DD/YYYY>. Available at: http://www.cancer.gov/cancertopics/pdq/treatment/child-cranio/healthprofessional. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Coping with Cancer: Financial, Insurance, and Legal Information page.

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Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.

Chat online

The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 8:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write to us

For more information from the NCI, please write to this address:

NCI Public Inquiries Office
9609 Medical Center Dr.
Room 2E532 MSC 9760
Bethesda, MD 20892-9760

Search the NCI Web site

The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.

There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).