Advances in the Management of Craniopharyngioma: A Narrative Review of Recent Developments and Clinical Strategies
Abstract
:1. Introduction
2. History
3. Epidemiology of Craniopharyngiomas
3.1. Age Distribution and Bimodal Peak
3.2. Global Incidence and Prevalence
3.3. Subtypes and Their Prevalence
3.4. Demographic Disparities
3.5. Diagnostic and Treatment Trends
3.6. Clinical Outcomes and Quality of Life
4. Genetic Mutations in Craniopharyngiomas
5. Clinical Signs and Symptoms
6. Diagnosis and Management of Craniopharyngioma
7. Histopathologic Features and Radiologic Features
8. Treatment
8.1. Targeted Therapies for Craniopharyngiomas Based on Genetic Mutations
8.2. Surgery
8.3. Intracavitary Adjuncts
8.4. Radiation Therapy
8.5. Multidisciplinary Approach
9. Prognosis and Long-Term Outcomes
10. Conclusions and Prospects
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
References
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Adamantinomatous Variant | Papillary Variant | |
---|---|---|
Age group | Children and adults | Adults |
Location | Pituitary stalk, subdiaphragmatic | Pituitary stalk, third ventricle |
Texture | Calcified Multicystic components | Not calcified Predominantly solid |
Hypothalamic and pituitary gland invasiveness | More with tumor noted to usually be very adherent to neurovascular structures | Less with tumor noted to usually be less adherent to neurovascular structures |
Mutation | CTNNB1 (chromosome 3p21) with upregulation of Wnt-β-catenin signaling pathway genes (including EGFR- and SHH-) | BRAF V600E (chromosome 7q34) with upregulation of MAPK/ERK signaling pathway genes |
Symptom/Sign | Frequency |
---|---|
Visual impairment (temporal hemianopsia most common from chiasmatic compression) | 62–84% |
Endocrine dysfunction (from involvement/compression of the pituitary stalk) | 40–87% |
| 85% |
| 40% |
| 25% |
| 25% |
| 20% |
Headaches | 50% |
Approach | Challenges/Limitations |
---|---|
TCA |
|
EEA |
|
Treatment Modality | Description | Advantages | Disadvantages/Limitations |
---|---|---|---|
Surgery | Subtotal or complete resection via the TCA or EEA | -tumor volume reduction and diagnosis -decreased RT dose post-resection -multiple corridors (TCA vs. EEA) with varying risk profiles -standard of care for adult and pediatric populations -potentially curative after GTR | -invasive -recurrence and risk for reoperation after STR -risk of endocrinologic disruption requiring hormone replacement -patient must meet surgical criteria and hold blood thinners perioperatively -added complexity in children 0–10 years old (incomplete paranasal sinus pneumatization) |
Radiation Therapy | Stereotactic radiosurgery or proton beam RT (preferred in children) | -noninvasive -targets postop residual invasive CP -precise targeting with proton beam used in children -excellent local tumor control (up to 91.1%) | -limited use in pediatrics (radiosensitive) -radiotoxicity -may induce secondary tumors -delayed onset vasculopathies |
Targeted Therapies | Genetic targets (CTNNB1 and BRAF V600E mutations) identified to interrupt molecular mechanisms of CP growth | -noninvasive -potentially decreased damage to non-CP tissue and preserved endocrinologic functioning -options for combination therapy | -requires crossing blood-brain barrier -potential acquired resistance -clinical trials ongoing, not currently approved for routine use in ACP treatment -adverse medication event |
Intracavitary Treatments | Beta–gamma radiation and/or bleomycin | -additional volume reduction, particularly for cystic portions, after surgical resection | -may induce secondary tumors -radiotoxicity -delayed onset vasculopathies -limited radiation use in children (radiosensitive) |
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Javidialsaadi, M.; Luy, D.D.; Smith, H.L.; Cecia, A.; Yang, S.D.; Germanwala, A.V. Advances in the Management of Craniopharyngioma: A Narrative Review of Recent Developments and Clinical Strategies. J. Clin. Med. 2025, 14, 1101. https://doi.org/10.3390/jcm14041101
Javidialsaadi M, Luy DD, Smith HL, Cecia A, Yang SD, Germanwala AV. Advances in the Management of Craniopharyngioma: A Narrative Review of Recent Developments and Clinical Strategies. Journal of Clinical Medicine. 2025; 14(4):1101. https://doi.org/10.3390/jcm14041101
Chicago/Turabian StyleJavidialsaadi, Mousa, Diego D. Luy, Heather L. Smith, Arba Cecia, Seunghyuk Daniel Yang, and Anand V. Germanwala. 2025. "Advances in the Management of Craniopharyngioma: A Narrative Review of Recent Developments and Clinical Strategies" Journal of Clinical Medicine 14, no. 4: 1101. https://doi.org/10.3390/jcm14041101
APA StyleJavidialsaadi, M., Luy, D. D., Smith, H. L., Cecia, A., Yang, S. D., & Germanwala, A. V. (2025). Advances in the Management of Craniopharyngioma: A Narrative Review of Recent Developments and Clinical Strategies. Journal of Clinical Medicine, 14(4), 1101. https://doi.org/10.3390/jcm14041101