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Treatment Options for Newly Diagnosed Childhood Craniopharyngioma

    Note: Some citations in the text of this section are followed by a level of evidence. The PDQ Editorial Boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)

    There is no consensus as to the optimal treatment of newly diagnosed craniopharyngioma. Little data exist to compare the different modalities in terms of recurrence rate or quality of life. For this reason, treatment is individualized.

    Recommended Related to Brain Cancer

    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...

    Read the Treatment Options for Recurrent Childhood Craniopharyngioma article > >

    Radical Surgery

    Because these tumors are histologically benign, it may be possible to remove all the visible tumor resulting in long-term disease control.[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] 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,[5][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.[2,6]

    Complications of radical surgery include the need for hormone replacement, obesity (which can be life threatening),[7] severe behavioral problems, blindness, seizures, spinal fluid leak, false aneurysms, and difficulty with eye movements. Rare complications include death from intraoperative hemorrhage, hypothalamic damage, or stroke.

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