Childhood Craniopharyngioma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - 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.
Recurrence of craniopharyngioma occurs in approximately 35% of patients regardless of primary therapy. 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.[Level of evidence: 3iiiDi] Complications are more frequent than with initial surgery.[Level of evidence: 3iiiDi] External-beam radiation therapy is an option if this has not been previously employed, including consideration...
Because these tumors are histologically benign, it may be possible to remove all the visible tumor resulting in long-term disease control.[Level of evidence: 3iA]; [Level of evidence: 3iiiB]; [Level of evidence: 3iiiC] A 5-year progression-free survival (PFS) rate of about 65% has been reported. 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,[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), 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.
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.[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.