Because of their rarity, proximal location to critical structures, and various presenting symptoms, craniopharyngiomas remain a challenging disease entity. Scientific studies are available to support aggressive resection as well as limited resection and RT. Owing to the lack of prospective, randomized trials, it cannot be stated with confidence what treatment option is best. As shown in this study, patients can present with many different symptoms and conditions. Therefore, treatment must be individualized. Nevertheless, as suggested in this study and others  adverse outcome (local recurrence or treatment complication) may be determined by the initial presentation. Therefore, the outcome of patients with this disease entity may be predetermined. However, the type of treatment may play a role in the ultimate outcome of patients, as suggested in the survey of the American Society of Pediatric Neurosurgery. This survey of 11 pediatric neurosurgeons suggested that limited surgery followed by RT yields a better quality of life compared with aggressive surgery. Furthermore, when RT was used for salvage after aggressive surgery, the quality of life was also found to be decreased compared with limited resection followed by planned RT. It is obvious that future studies of patients who are treated with this disorder should perform formal neuropsychological testing both before and after any intervention.

The results of various series using fractionated RT are listed. It is difficult to compare the outcomes between these investigations because of the differences noted in patient and treatment characteristics. However, some conclusions can be made. Other than the recent Harvard experience as reported by Tarbell  with stereotactic RT, all the reports have been retrospective chart reviews with patients treated over a long interval (a 20–36-year period). Therefore, most reports were obtained using a variety of treatment techniques and before CT-based treatment planning was readily available. As with our series, two groups noted a dose response for local control. Habrand and Regine noted a higher rate of tumor control with doses >55 Gy and 54 Gy, respectively. Although our dose cutpoint of 60 Gy for local control is similar to their doses, it is unclear why it is higher. With the improved treatment planning and more precise tumor localization the stereotactic RT experience of Tarbell will be of great interest as they accumulate patients and longer treatment follow-up. Perhaps with such precise irradiation, the lower radiation doses used in the treatment of their patients (50–54 Gy) will be sufficient for long-term local control. Hetelekidis  noted no recurrences among 8 patients treated with EBRT without surgical intervention. Although our series had only 2 such patients, 1 patient had a recurrence 13.5 years after completing RT. It should be noted that Habrand noted impaired functional outcome in 30 of 35 patients who were treated with RT. However, most patients in their series were treated with low-energy megavoltage irradiation and/or with an opposed lateral technique. The use of such a technique is associated with excessive toxicity even for tumors that are generally treated with lower radiation dosages. Because we found that tumor control improved with a total dose ≥60 Gy, normal tissue toxicity increased with an NTD of >55 Gy, and consequently, adverse outcomes seemed minimized with an NTD of 55–63 Gy, we recommend irradiating craniopharyngiomas to approximately 60 Gy with lower fraction sizes of 1.6–1.7 Gy. This lower dose fractionation scheme should not interfere with the effects of radiation to control this tumor because of its known propensity for slow growth. Furthermore, because of the relatively high doses needed to control craniopharyngiomas and because of the proximity of critical, normal structures, high-precision techniques are recommended such as fractionated stereotactic photons, protons, or three-dimensional conformal RT. However, radiation is associated with neurocognitive impairment in very young children. Additionally, cranial radiation is also associated with a higher risk of radiation-induced carcinogenesis at younger ages. Therefore, delaying cranial radiation is recommended for young children. Experimentally, interferon-α2B may have a future role in delaying RT.

Usually gross total resection is recommended as the initial management. The ease of surgical management may depend on tumor location. Thirty percent of tumors are prechiasmatic and are generally more accessible and less adherent to vital suprasellar structures. Of the other 70% that are retrochiasmatic, surgical resection may be more difficult, because the tumors can extend superiorly into the third ventricle and along the hypothalamus. However, for those tumors limited to the sella (especially those tumors that are not associated with hypopituitarism), surgical resection may be associated with less morbidity. Usually after incomplete resection, RT is recommended because approximately 70% will recur within 2–3 years. Furthermore, delayed RT after subtotal resection may be associated with a decrement in survival in the pediatric population. Various surgical series are shown. The mortality rates vary between 1.7% and 3.2%. Most surgical series are difficult to compare with reports of RT, because only a small percentage of patients are treated for salvage. Both the Mayo Clinic series and the Erlangen, Germany report show that the chances of a complete resection and good outcome were higher in those patients who underwent transsphenoidal surgery. Likewise, a larger tumor size was found to be associated with a worse outcome in the report by Yasargil because of the higher likelihood of damage both preoperatively and intraoperatively to vital intracranial structures. Recurrence rates after complete resection without postoperative RT were noted to be between 18.7% and 50%. Long-term follow-up of the recent reports will be of great interest because complete resection was not only based on surgical impression, but on postoperative imaging as well. Postoperative imaging has revealed residual calcifications in 15–50% of cases that were thought to be completely resected at the time of surgery The lowest recurrence rates of 18.1% and 18.7% after complete resection were noted in the investigations that used postoperative imaging to determine whether residual disease was present. However, in a recent smaller report, with a mean follow-up of 52 months, 38.9% (14 of 36) of radically resected tumors recurred despite postoperative imaging. More recently, intracavitary irradiation has been used to treat cystic craniopharyngiomas that are >3 cm3.

The response rates range from 71% to 88% and recurrence rates from 9.7% to 33% with limited follow-up. However, this local radiation technique can be associated with the following toxicities: anterior and posterior pituitary dysfunction visual disturbance and cranial nerve palsy. Needless to say, longer follow-up with larger patient numbers will ultimately determine the efficacy and toxicity of this technique. Finally, all 3 patients whom we treated with salvage 32P experienced visual loss. Stereotactic radiosurgery has been also used to treat craniopharyngioma. The results of various series are noted. With limited follow-up, high rates of local control have been noted. Toxicity appears to be minimal. However, this technique is usually limited to solid, small (≤2.5 cm) tumors that are ≥3 mm from the optic chiasm. Longer follow-up with larger patient numbers is required to determine the role of radiosurgery in the treatment of craniopharyngioma.