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Childhood Craniopharyngioma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment Options for Newly Diagnosed Childhood Craniopharyngioma

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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.[9][Level of evidence: 3iiiDiii] 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.[10]

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. The surgical procedure is followed by radiation therapy, with a 5-year PFS rate of about 70% to 90% [4,11]; [12][Level of evidence: 3iDiii] and 10-year overall survival rates higher than 90%.[13][Level of evidence: 3iiA] Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention.[14][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.[15] 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.[16,17] 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.[12,18,19] 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.[20][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.[21][Level of evidence: 3iiiDiii]

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