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Childhood Craniopharyngioma Treatment (PDQ®)

Health Professional Version
Last Modified: 05/17/2012

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. Chemotherapy is generally not utilized.

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]





Glossary Terms

Level of evidence 3iiiB
Nonconsecutive case series with cause-specific mortality as an endpoint. See Levels of Evidence for Adult and Pediatric Cancer Treatment Studies (PDQ®) for more information.
Level of evidence 3iiiDi
Nonconsecutive case series with event-free survival as an endpoint. See Levels of Evidence for Adult and Pediatric Cancer Treatment Studies (PDQ®) for more information.
Level of evidence 3iiiDiii
Nonconsecutive case series with progression-free survival as an endpoint. See Levels of Evidence for Adult and Pediatric Cancer Treatment Studies (PDQ®) for more information.
stereotactic radiosurgery (STAYR-ee-oh-TAK-tik RAY-dee-oh-SER-juh-ree)
Stereotactic radiosurgery (SRS) implies the delivery of a single, high dose of radiation using stereotactic techniques. Classically, for cranial SRS, a rigid neurosurgical frame is attached to the patient’s skull, and a stereotactic localizing device is attached to the frame to allow for improved targeting accuracy. Commercial SRS systems are available in which immobilization is achieved without a rigid neurosurgical frame. While there are innumerable commercial SRS and stereotactic radiation therapy systems, all of these systems utilize the same underlying principles: (1) patient immobilization, (2) targeting accuracy, (3) delivery of high doses of radiation, and (4) heterogeneous dose distribution with a steep dose gradient. Commercial SRS systems include Gamma Knife, which utilizes 201 radioactive cobalt sources positioned in a semispherical array, and Cyberknife, which utilizes robotics technology allowing the linear accelerator (LINAC) to track the patient position in real time during treatment. A standard LINAC can be equipped with cones attached to its head, allowing for circular collimation of the beam. The dose distribution from Gamma Knife, Cyberknife, or a standard LINAC equipped with cones is spherical, and thus treating nonspherically shaped targets requires the superposition of multiple spherical dose distributions. A LINAC equipped with multi-leaf collimators can also be used for SRS, which allows more conformal beam shaping. Also called radiation surgery, radiosurgery, and stereotaxic radiosurgery.