9. Future directions

However, the endoscopic approach has certain disadvantages in that it is not well suited for masses that are postchiasmatic or for lesions with prominent lateral extensions [16]. In situations of a recurrent craniopharyngioma after craniotomy, the trans-nasal approach may offer the distinct advantage of a previously untouched approach to the lesion, and an endoscopy enables the surgeon to assess the anatomy of the subchiasmatic and retrochiasmatic regions more closely. If preservation or restoration of vision is the primary goal, this should be strongly considered. Endoscopic approaches also offer the advantage of reducing morbidity associated

A comprehensive literature review performed by Komotar et al. claimed higher rates of GTR with endoscopic surgery compared to open resection (66 vs. 48%), higher likelihood of visual improvement (56 vs. 33%), although with a higher risk of CSF leak (18.4 vs. 2.6%). However, it is important to note that the paper suffers from systematic methodological flaws and selection bias since the mean follow-up time for the patients who underwent open resection in this study was 65 months, as opposed to 25.1 months for the endoscopically treated group [20]. More recent series comparing the two approaches were not able to establish similar significant

In conclusion, the endoscopic approach may be most appropriate in certain patients who present with intrasellar and Type I lesions, whereas an open resection, employing a frontotemporal craniotomy, may be more suitable for intra-infundibular or post-infundibular lesions. In patients presenting with cystic CP, stereotactic management is appropriate to aspirate the cystic compo-

For much of the intervening decades since neurosurgery for CP was described, the debate largely revolved about the optimal treatment strategy, whether aggressive surgical resection or conservative surgery offered patients the best option. Among the two fundamental schools of thought regarding the optimal approach to treat craniopharyngioma, one advocated for GTR for all patients with radiation reserved for salvage therapy due to anticipated adverse effects of radiation [21]. The alternative management suggested was that of a subtotal resection or biopsy and cyst decompression in combination with adjuvant radiation therapy. Advantages of this approach include lower morbidity and improved quality of life [22]. Although the surgical goal remains maximal tumor resection with minimal morbidity, it is estimated that 33% of patients will present with some form of recurrence within the first few years. It is especially clear that radiation therapy is a key element of treatment for these patients with

In a comprehensive review of a published series of CP patients, Yang et al. were able to demonstrate that subtotal surgery in conjunction with post-operative radiation results in improved survival in patients with CP [23]. This approach employing rather conservative surgery has the advantage of reducing the risks of hypopituitarism and hypothalamic injury. The results from various meta-analyses were corroborated and further expanded with evidence obtained from a

nent of the mass before pursuing other avenues of treatment (e.g., radiation therapy).

8. Radiation therapy for craniopharyngioma

with brain retraction typically employed during transcranial approaches [16].

differences in treatment outcomes.

208 Brain Tumors - An Update

recurrent craniopharyngioma.

As with many other cancers, targeted molecular therapies offer the promise of effective treatment without the need for extensive surgery or radiation. Genetic studies of aCP and pCP identified genetic characteristics of each subtype, that may eventually be targeted by specific molecular therapies for CP. Genomic analysis of aCP revealed mutations in CTNNB1 (βcatenin) in nearly all cases and BRAF mutations in most pCPs. These signaling pathways are currently being interrogated for targeted molecular therapies. Inhibitors of the BRAF protooncogene employing modern drugs such as Dabrafenib or Vemurafenib, or by prescribing MEK inhibitors, such as Trametinib, are already being studied as therapies for pCP. Inhibition of similar molecular pathways in melanoma, amelanoblastoma, hairy cell leukemia, and pleomorphic xanthoastrocytoma has already demonstrated the clinical promise of these therapies [27, 28]. Multicenter phase-2 clinical trials at the National Cancer Institute are currently underway evaluating BRAF/MEK inhibition in the treatment of craniopharyngioma [27].

Regardless of the therapeutic strategies that are utilized, it is evident that craniopharyngiomas continue to present a distinct oncologic challenge that still needs to be overcome. Quality of life is a key consideration in this disease, and long-term follow up, involving a multidisciplinary team, is a necessary element of care of these patients. The combination of minimally invasive surgery and radiosurgery will, in the near future, result in a minimally morbid approach to this disease to allow patients improved quality of life.

## 10. Conclusions

Since Cushing's early writings, describing surgery for craniopharyngioma, our strategies to treat this challenging disease have evolved with modern technology to include endoscopic and radiation therapy. The widespread availability and adoption of these techniques have led to endoscopic treatment and radiation therapy becoming indispensable facets of treatment of craniopharyngioma. As our molecular understanding of craniopharyngioma continues to grow, there is considerable hope for the development of effective targeted therapies.

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