Craniopharyngioma
Treatment

Treatment often requires surgery and sometimes radiation therapy. Depending on the severity of the symptoms at the time of diagnosis, patients can make a complete recovery. However, because craniopharyngiomas can involve important structures in the brain, tumor growth or treatments can cause permanent problems, such as lack of normal hormone control, vision loss or the need for a shunt to treat hydrocephalus.

In general, most children with craniopharyngiomas will require long-term monitoring and treatment of endocrine function (and treatment if the pituitary is not functioning properly), as well as regular MRI scans to check for any signs that the tumor has returned.

Choosing a treatment is often a challenge. The goals of a cure or good tumor control must be balanced against unacceptable side effects from the treatment.

If the tumor is not tightly attached to important brain structures, and can be removed completely by surgery, a cure is possible. This occurs in about a third of cases.

In the majority of the remaining cases, the tumor can be mostly removed, leaving a small amount behind. In general, if there is doubt about whether any part of the tumor is left after surgery, radiation is recommended to prevent recurrence of the tumor. The type of radiation used depends on the location of the residual tumor.

If a larger amount of tumor remains after surgery, or if vital structures are close by, then conformal external beam radiotherapy is used. This method uses many smaller doses of radiation to protect the surrounding brain tissue, but there is some spread of the radiation beyond the actual target. Nevertheless, this technique is very safe and has excellent results.

If there is a small, well-defined residual tumor remaining that is a safe distance from the optic nerves and hypothalamus, Gamma Knife radiosurgery is an option. However, radiation usually affects the pituitary function and can affect cognitive function. For young children who have had a good surgical resection (removal of most of the tumor), an alternative option is the "watch and wait" approach, with radiation treatment given only if there is clear evidence of recurrent tumor growth.

Reviewed by health care specialists at UCSF Benioff Children's Hospital.

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