Español
Questions About Cancer? 1-800-4-CANCER
  • View entire document
  • Print
  • Email
  • Facebook
  • Twitter
  • Google+
  • Pinterest

Childhood Craniopharyngioma Treatment (PDQ®)

Newly Diagnosed Childhood Craniopharyngioma Treatment

Treatment Options for Newly Diagnosed Childhood Craniopharyngioma

There is no consensus on the optimal treatment for newly diagnosed craniopharyngioma, in part because of the lack of prospective randomized trials that compare the different treatment options. Treatment is individualized on the basis of factors that include the following:

  • Tumor size.
  • Tumor location.
  • Extension of the tumor.
  • Potential short-term and long-term toxicity.

Treatment options for newly diagnosed childhood craniopharyngioma include the following:

Radical surgery with or without radiation therapy

It may be possible to remove all visible tumor and achieve long-term disease control because these tumors are histologically benign.[1][Level of evidence: 3iA]; [2][Level of evidence: 3iiiB]; [3][Level of evidence: 3iiiC] A 5-year progression-free survival (PFS) rate of about 65% has been reported.[4] Gross-total resection is often 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. These structures may limit the ability to remove the entire tumor.

Many surgical approaches have been described, and the choice is determined by tumor size, location, and extension. Radical surgical approaches include the following:

  • Transsphenoidal approach: A transsphenoidal approach may be possible for some small tumors located entirely within the sella.[5][Level of evidence: 3iiiC] The development of expanded endonasal techniques with endoscopic visualization have allowed this approach to be increasingly used, even for sizeable childhood tumors, which is similar to the experience in adults.[6] When an endonasal approach is not possible, a craniotomy is required.
  • Craniotomy: As noted above, gross-total resection may be technically challenging because the tumor is surrounded by vital structures. The surgeon often has a limited view of the hypothalamic and sellar regions, and portions of the mass may remain after surgery, 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.[2,7]

    Complications of radical surgery include the following:

    • Obesity, which can be life-threatening.[8] Hypothalamic-sparing surgical techniques may result in reduced postoperative obesity without an increase in tumor recurrence.[9][Level of evidence: 3iiDi]
    • Hormone replacement therapy.[10]
    • Severe behavioral problems.[10]
    • Blindness.
    • Seizures.
    • Spinal fluid leak.
    • False aneurysms.
    • Difficulty with eye movements.
    • Death from intraoperative hemorrhage, hypothalamic damage, or stroke (rare).

If the surgeon indicates that the tumor was not completely removed or if postoperative imaging reveals residual craniopharyngioma, radiation therapy may be recommended to prevent early progression.[11][Level of evidence: 3iiiDiii]

Periodic surveillance using magnetic resonance imaging is performed for several years after radical surgery because of the possibility of tumor recurrence.

Subtotal resection with 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 the late effects associated with radical surgery.[9]

The surgical procedure is often followed by radiation therapy, with a 5-year PFS rate of about 70% to 90%[4,12]; [13][Level of evidence: 3iDiii] and 10-year overall survival rates higher than 90%.[14][Level of evidence: 3iiA]; [15][Level of evidence: 3iiiDiii] Conventional radiation therapy is fractionated external-beam radiation, with a recommended dose of 54 Gy to 55 Gy in 1.8-Gy fractions.[16] Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention.[17][Level of evidence: 3iDiv]

A systematic review of 109 reports that described extent of resection found that subtotal resection plus radiation therapy was associated with rates of tumor control similar to those for gross-total resection. It was also reported that both approaches were associated with higher PFS rates than was subtotal resection alone.[15][Level of evidence: 3iiiDiii]

Surgical complications with a subtotal resection are less likely than with radical surgery. Complications of radiation therapy include the following:

  • Loss of pituitary hormonal function.
  • Cognitive dysfunction.
  • Development of late strokes and vascular malformations.
  • Delayed blindness.
  • Development of second tumors.
  • Malignant transformation of the primary tumor within the radiation field (rare).[18,19]

Newer radiation technologies such as intensity-modulated proton therapy may reduce scatter during whole-brain and whole-body irradiation and result in the sparing of normal tissues. It is unknown whether such technologies result in reduced late effects from radiation.[13,20,21]

Tumor progression remains a concern, 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 small field.[22][Level of evidence: 3iC] Proximity of the craniopharyngioma to vital structures, particularly the optic nerves, limits this to small tumors within the sella.[23][Level of evidence: 3iiiDiii]

Primary cyst drainage with or without radiation therapy

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. Benefits include temporary relief of fluid pressure by serial drainage, and in some cases, for intracystic instillation of sclerosing agents as a means to postpone or obviate radiation treatment. This procedure may also allow the surgeon to use a two-staged approach: first draining the cyst via the implanted catheter, to relieve pressure and complicating symptoms; and then later resecting the tumor or employing radiation therapy.[24]

References

  1. 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]
  2. 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]
  3. 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]
  4. 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]
  5. 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]
  6. Chivukula S, Koutourousiou M, Snyderman CH, et al.: Endoscopic endonasal skull base surgery in the pediatric population. J Neurosurg Pediatr 11 (3): 227-41, 2013. [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. 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]
  10. 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]
  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. 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]
  13. 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]
  14. 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]
  15. 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]
  16. Kiehna EN, Merchant TE: Radiation therapy for pediatric craniopharyngioma. Neurosurg Focus 28 (4): E10, 2010. [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. 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]
  19. 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]
  20. 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]
  21. 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]
  22. 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]
  23. 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]
  24. 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]
  • Updated: January 15, 2015