From the Annals of The University of Toronto, The Division of NeurosurgeryCreative and Innovative Methods and Techniques for the Challenges in the Management of Adult Craniopharyngioma
Introduction
Craniopharyngioma is a rare brain tumor that can occur at the floor of the third ventricle, along the pituitary stalk, or in the sella turcica. The overall incidence in the United States is reported to be 0.13 per 100,000 person years.1 Although craniopharyngioma is a benign tumor histologically, its management poses significant challenges,2 as described in detail in the companion article on challenges and controversies. This situation is primarily because of its location close to visual, endocrine, pituitary stalk and gland, hypothalamic, and vascular structures.
Although long-term survival is high (>90% at 10 years), significant morbidity and poor quality of life are associated with both the tumor and its treatment. Although surgery remains the primary form of treatment, attempts at total removal are often difficult (<50% rates of GTR3,4 in some series) and can result in both short-term and long-term sequelae. Likewise, reported recurrence rates remain high (10-year control rate, 30%–50% after incomplete resection).5
In this article, we discuss possible solutions to the obstacles in treating craniopharyngioma and future possibilities for improving the care of these patients (Table 1).
Section snippets
Cerebrospinal Fluid/Blood Liquid Biopsy
The use of liquid biopsy for central nervous system tumors is rapidly expanding.6 In general, it has been described mainly in 2 types of fluids: blood and cerebrospinal fluid (CSF). Blood-based biomarkers include circulating tumor cells, nucleic acids (cell-free circulating tumor DNA and microRNA) and exosomes.7 Moreover, CSF-based liquid biopsy may have a superior yield compared with other fluids.8,9
In craniopharyngioma, which has a signature mutation in most cases (>90%),10 liquid biopsy
Operative Technological Innovation
In the last few decades, the main innovation in the surgical treatment of craniopharyngiomas has been the introduction of the endoscopic endonasal approach (EEA). Recently, several studies have confirmed the advantages of this technique for the management of craniopharyngiomas, in terms of extent of resection, functional sequelae, and complication rate.35, 36, 37, 38, 39
Furthermore, other technological advances could also rapidly change the field. New-generation endonasal transsphenoidal
Endocrinologic Treatment
Endocrinologic morbidity may develop during tumor growth or after surgical intervention or radiation therapy in patients with craniopharyngioma. Hypopituitarism and hypothalamic insufficiency can be lifelong morbidities. Hormone replacement is the standard therapy. Damage of the homeostatic function located in the hypothalamus causes impaired control of satiety and hunger, sleep disorders, and dysregulation of temperature.78
Hypothalamic Obesity
One of the most devastating sequelae is hypothalamic obesity, as
Preventive Medicine
Single-nucleotide polymorphisms (SNPs) of the germline genome are known be associated with a plethora of individual characteristics based on extensive data from genome-wide association studies.105 Many types of diseases, including brain tumors such as glioma and meningioma, have been found to be related to SNPs.106, 107, 108, 109 They were also shown to be associated with treatment response and prognosis in glioblastoma110 and medulloblastoma.111 No particular SNP has yet been found to be
Restoration/Regeneration Medicine
As discussed earlier, resection of tumors can result in high morbidity, and optic pathways and endocrinologic and visual functions are often most affected. Natural recovery is unlikely, because of limited self-renewal capacity in adult cells. However, the development of regenerative medicine may offer new treatments in the future.
Stem cell therapy is still in the stage of clinical trials, but it has already been tested for the treatment of demyelinating diseases/spinal cord injury, amyotrophic
Conclusions
Despite modern technological and medical advances, craniopharyngioma remains a considerable challenge for neurosurgeons, radiation oncologists, and endocrinologists in terms of tumor resection, planning the optimal radiation coverage, and endocrinologic management. Craniopharyngioma is one of the tumor types that are at the center of a treatment revolution, including advances in surgical techniques, surgical tools, diagnostic imaging, genomic analyses, gene-based treatments, and regeneration of
Acknowledgments
We acknowledge the contribution of Anne Stanford, E.L.S., who provided professional editing services.
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Cited by (1)
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
Hirokazu Takami and Carlos Velásquez are co–first authors.