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Childhood Craniopharyngioma Treatment (PDQ®)

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]
  • Updated: August 12, 2014