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Keywords = craniopharyngiomas

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15 pages, 1582 KiB  
Article
Evaluation of the Effect of Optic Nerve Compression by Craniopharyngioma on Retinal Nerve Fiber Layer Thickness in Pediatric Patients
by Klaudia Rakusiewicz-Krasnodębska, Agnieszka Bogusz-Wójcik, Elżbieta Moszczyńska, Maciej Jaworski, Paweł Kowalczyk and Wojciech Hautz
Cancers 2025, 17(15), 2574; https://doi.org/10.3390/cancers17152574 - 5 Aug 2025
Abstract
Purpose: The present study aims to evaluate alterations in the peripapillary retinal nerve fiber layer (RNFL) thickness in pediatric patients following surgical resection of childhood-onset craniopharyngioma (CP) and to identify tumor characteristics and other factors influencing these alterations, including changes in the lesion’s [...] Read more.
Purpose: The present study aims to evaluate alterations in the peripapillary retinal nerve fiber layer (RNFL) thickness in pediatric patients following surgical resection of childhood-onset craniopharyngioma (CP) and to identify tumor characteristics and other factors influencing these alterations, including changes in the lesion’s location. Design: retrospective clinical cohort study. Methods: A retrospective analysis was conducted on 73 eyes from 38 patients with CP and 64 eyes from 32 age- and sex-matched healthy controls. The mean age of the CP patients was 10.3 ± 4.2 years (range 4–17), while the control group had a mean age of 10.5 ± 3.1 years (range 4–17). Optical coherence tomography (OCT) was used to assess the peripapillary RNFL thickness in the study and control groups. RNFL thickness was analyzed in the superior, inferior, and average sectors, as well as across eight optic nerve sectors. Tumor characteristics were evaluated to determine their correlation with changes in RNFL thickness in individual sectors. Results: Postoperative thickness of peripapillary RNFL in all individual sectors was significantly reduced in the CP group compared to healthy controls. Location, tumor volume, maximum tumor diameter, calcification, ventriculoperitoneal shunt, surgery technique, total resection, presence of Rosenthal fibers, and reoperation due to progression or recurrence correlated with damage to RNFL. Conclusions: CP is associated with significant reductions in RNFL thickness, indicating the tumor’s impact on optic nerve fibers. OCT is a valuable tool for monitoring visual pathway impairment and postoperative outcomes. Correlations between RNFL thickness in individual sectors and clinical parameters may offer valuable insights for diagnosis and monitoring, underlining their potential role in predicting visual outcomes. Regular RNFL evaluation should be integrated into the long-term care of CP patients to optimize visual prognosis and detect progressive or residual damage. Full article
(This article belongs to the Section Pediatric Oncology)
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21 pages, 13450 KiB  
Article
Distinctive Characteristics of Rare Sellar Lesions Mimicking Pituitary Adenomas: A Collection of Unusual Neoplasms
by Andrej Pala, Nadja Grübel, Andreas Knoll, Gregor Durner, Gwendolin Etzrodt-Walter, Johannes Roßkopf, Peter Jankovic, Anja Osterloh, Marc Scheithauer, Christian Rainer Wirtz and Michal Hlaváč
Cancers 2025, 17(15), 2568; https://doi.org/10.3390/cancers17152568 - 4 Aug 2025
Abstract
Background/Objectives: Pituitary tumors account for over 90% of all sellar region masses. However, a spectrum of rare neoplastic, inflammatory, infectious, and vascular lesions—benign and malignant—can arise in the intra- and parasellar compartments and clinically and radiologically mimic PitNETs. We report a cohort [...] Read more.
Background/Objectives: Pituitary tumors account for over 90% of all sellar region masses. However, a spectrum of rare neoplastic, inflammatory, infectious, and vascular lesions—benign and malignant—can arise in the intra- and parasellar compartments and clinically and radiologically mimic PitNETs. We report a cohort of 47 such rare and cystic midline intracranial lesions, emphasizing their distinctive morphological, clinical, and imaging features and the personalized treatment strategies applied. Methods: In this retrospective single-center study, we reviewed all patients treated for suspected PitNETs via transsphenoidal approach between 2015 and 2024. Of 529 surgical cases, we excluded confirmed PitNETs, meningiomas, and classical intradural craniopharyngiomas. Collected data encompassed patient demographics, tumor characteristics, presenting symptoms, extent of resection or medical therapy, endocrine outcomes, and follow-up information. Results: Among all 529 patients who underwent surgical treatment for sellar lesions from 2015 to 2024, 47 cases (8.9%) were identified as rare or cystic masses. Forty-six underwent transsphenoidal resection; one patient with hypophysitis received corticosteroid therapy alone. Presenting symptoms included headache (n = 16), dizziness (n = 5), oculomotor disturbances (n = 2), and visual impairment (n = 17). Endocrine dysfunction was found in 30 patients, 27 of whom required hydrocortisone replacement. Histopathological diagnoses were led by colloid cysts (n = 14) and Rathke’s cleft cysts (n = 11). The remaining 22 cases comprised plasmacytoma, germinoma, lymphoma, pituicytoma, inverted papilloma, metastatic carcinoma, chordoma, nasopharyngeal carcinoma, chloroma, and other rare entities. Preoperative imaging diagnosis proved incorrect in 38% (18/47) of cases, with several lesions initially misidentified as PitNETs. Conclusions: Nearly 9% of presumed PitNETs were rare, often benign or inflammatory lesions requiring distinct management. Most could be safely resected and demonstrated excellent long-term outcomes. Yet, despite advanced imaging techniques, accurate preoperative differentiation remains challenging, with over one-third misdiagnosed. Clinical red flags—such as early hormone deficits, rapid progression or atypical imaging findings—should prompt early interdisciplinary evaluation and, when indicated, image-guided biopsy to avoid unnecessary surgery and ensure tailored therapy. Full article
(This article belongs to the Special Issue Pituitary Tumors: Clinical and Surgical Challenges)
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10 pages, 1234 KiB  
Article
Comparative Outcomes of Gross Total Resection vs. Subtotal Resection Plus Radiotherapy for Preventing Craniopharyngioma Recurrence: A Meta-Analysis of the Endoscopic Endonasal Approach
by Ernest J. Bobeff, Bartosz Szmyd, Wojciech Młynarski, Emmanuel Jouanneau, Caroline Apra, Ming Shen, Zara M. Patel, Dariusz J. Jaskólski and Theodore H. Schwartz
Cancers 2025, 17(15), 2516; https://doi.org/10.3390/cancers17152516 - 30 Jul 2025
Viewed by 220
Abstract
Objective: Craniopharyngioma recurrence risk studies comparing gross total resection (GTR) vs. subtotal resection (STR) with radiotherapy (XRT) provide inconclusive or contradictory results. This may be an effect of the small group sizes and diversity in the approaches used. Currently, the endoscopic endonasal approach [...] Read more.
Objective: Craniopharyngioma recurrence risk studies comparing gross total resection (GTR) vs. subtotal resection (STR) with radiotherapy (XRT) provide inconclusive or contradictory results. This may be an effect of the small group sizes and diversity in the approaches used. Currently, the endoscopic endonasal approach (EEA) is preferred in craniopharyngioma management. Here, we aimed to perform a meta-analysis comparing recurrence risk after GTR vs. STR plus XRT in patients treated with the EEA regimen. Methods: We performed a systematic literature search of original English language papers on craniopharyngioma management published in the PubMed, Web of Science, and Scopus databases up to 18 October 2023. Eleven articles included data on recurrence rate after EEA: GTR vs. STR with XRT. We extracted the year of publication, number of patients, surgical approach, extent of resection, and follow-up duration. We used meta-analysis for the odds ratio (OR) in fixed and random effects models and Egger’s and Begg’s tests to assess heterogeneity and publication bias. Follow-up duration and time to recurrence were additionally included in Kaplan–Meier curves with log-rank test analysis. Results: We observed a lower recurrence rate in patients after GTR (10%) as compared to STR with XRT (30%), OR = 0.299, p < 0.001. To increase data reliability, we limited our analysis to studies with at least five patients in each subgroup and also observed lower recurrence in patients after GTR (12%) as compared to STR with XRT (27%), OR = 0.376, p = 0.011. Survival analysis confirmed significant differences in recurrence-free survival percentages between these groups (p = 0.008). Conclusions: To date, this is the largest meta-analysis evaluating the recurrence risk in patients undergoing EEA for craniopharyngioma resection, comparing outcomes between those treated with GTR and those treated with STR plus XRT. The results suggest that GTR significantly reduces recurrence risk. Full article
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19 pages, 1804 KiB  
Review
Management of Acquired Hypothalamic Obesity After Childhood-Onset Craniopharyngioma—A Narrative Review
by Hermann L. Müller
Biomedicines 2025, 13(5), 1016; https://doi.org/10.3390/biomedicines13051016 - 22 Apr 2025
Viewed by 1383
Abstract
Introduction: Craniopharyngiomas are rare sellar embryonic malformational tumors of low-grade histological malignancy. Despite high overall survival rates (92%), quality of life is frequently reduced due to adverse late effects caused by hypothalamic obesity. It is well known that morbid hypothalamic obesity is [...] Read more.
Introduction: Craniopharyngiomas are rare sellar embryonic malformational tumors of low-grade histological malignancy. Despite high overall survival rates (92%), quality of life is frequently reduced due to adverse late effects caused by hypothalamic obesity. It is well known that morbid hypothalamic obesity is associated with the grade of hypothalamic damage. Accordingly, craniopharyngioma should be considered a paradigmatic disease, reflecting challenges in the diagnosis and treatment of acquired hypothalamic obesity. Methods: A narrative review was performed after searching the MEDLINE/PubMed, Embase, and Web of Science databases for initial identifying articles. The search terms childhood-onset craniopharyngioma and hypothalamic obesity were used. Results: Despite the availability of promising therapeutic approaches, such as medication with central stimulating agents, antidiabetic drugs, glucagon-like peptide 1 (GLP1) receptor agonists, and Setmelanotide, it must be emphasized that there is currently no pharmaceutical treatment for hypothalamic obesity in craniopharyngioma proven to be effective in randomized controlled trials. For Setmelanotide, a prospective blinded randomized trial over a 12-month treatment period is ongoing. Bariatric interventions are effective, but non-reversible procedures such as bypass operations are controversial in the pediatric age group due to legal and ethical concerns. Recently, a treatment algorithm was introduced to improve the management of hypothalamic syndrome/obesity by offering more personalized treatment. Decisions on treatment strategies focusing on the preservation of visual, neuroendocrine, and hypothalamic integrity should be made by experienced multidisciplinary teams. Conclusions: Treatment approaches for hypothalamic obesity are limited. Further research on novel treatment approaches for hypothalamic obesity is warranted to improve the quality of life after childhood-onset craniopharyngioma. Full article
(This article belongs to the Special Issue Feature Reviews on Cardiovascular and Metabolic Diseases)
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22 pages, 11326 KiB  
Article
Isolation and Characterization of the Adamantinomatous Craniopharyngioma Primary Cells with Cancer-Associated Fibroblast Features
by Dongting Chen, Ting Lei, Yulin Wang, Zaitao Yu, Siqi Liu, Ling Ye, Wanfang Li, Qin Yang, Hongtao Jin, Fangjun Liu and Yan Li
Biomedicines 2025, 13(4), 912; https://doi.org/10.3390/biomedicines13040912 - 9 Apr 2025
Cited by 1 | Viewed by 882
Abstract
Backgrounds: Adamantinomatous craniopharyngiomas (ACPs) are benign intracranial tumors that behave aggressively due to their location, infiltration of the surrounding nervous tissue and high capacity for recurrence. In this study, we aimed to construct ACP primary cell models for further investigation of tumorigenic [...] Read more.
Backgrounds: Adamantinomatous craniopharyngiomas (ACPs) are benign intracranial tumors that behave aggressively due to their location, infiltration of the surrounding nervous tissue and high capacity for recurrence. In this study, we aimed to construct ACP primary cell models for further investigation of tumorigenic and recurrent mechanisms. Methods: Primary cells were isolated from primary (one case) and recurrent (one case) ACP. Short tandem repeat (STR) analysis was used to clarify the identity of the ACP primary cells we isolated. Whole exome sequencing (WES), immunofluorescence (IF) and immunohistochemistry (IHC) were performed on primary cells and corresponding ACP tissues, to determine the mutational profile and to clarify the tissue origin and phenotypic of primary cells. Transcriptome RNA-seq was performed to obtain the gene expression characteristics of ACP primary cells. Subsequently, a heterotopic ACP xenograft mouse model was established to confirm the tumorigenesis capacity of ACP primary cells. Results: ACP primary cells were successfully cultured. The genetic variants were similar to the original tumor tissue, and they owned expression of cancer-associated fibroblast (CAF) markers (FSP1/S100A4, Vimentin) and nuclear translocation β-catenin. Meanwhile, they had an high level expression of extracellular matrix components (Fibronectin). The tumor formation ability of ACP primary cells was verified. The transcriptional signatures of ACP primary cells were also explored. Conclusions: We successfully isolated and characterized ACP primary cells that acquired multiple CAF features and demonstrated stable propagation through dozens of passages. These PDC models laid the foundation for further research on ACP. Full article
(This article belongs to the Special Issue Clinical, Radiological, and Molecular Insights into Craniopharyngioma)
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30 pages, 1348 KiB  
Review
Artificial Intelligence for Neuroimaging in Pediatric Cancer
by Josue Luiz Dalboni da Rocha, Jesyin Lai, Pankaj Pandey, Phyu Sin M. Myat, Zachary Loschinskey, Asim K. Bag and Ranganatha Sitaram
Cancers 2025, 17(4), 622; https://doi.org/10.3390/cancers17040622 - 12 Feb 2025
Cited by 7 | Viewed by 3257
Abstract
Background/Objectives: Artificial intelligence (AI) is transforming neuroimaging by enhancing diagnostic precision and treatment planning. However, its applications in pediatric cancer neuroimaging remain limited. This review assesses the current state, potential applications, and challenges of AI in pediatric neuroimaging for cancer, emphasizing the unique [...] Read more.
Background/Objectives: Artificial intelligence (AI) is transforming neuroimaging by enhancing diagnostic precision and treatment planning. However, its applications in pediatric cancer neuroimaging remain limited. This review assesses the current state, potential applications, and challenges of AI in pediatric neuroimaging for cancer, emphasizing the unique needs of the pediatric population. Methods: A comprehensive literature review was conducted, focusing on AI’s impact on pediatric neuroimaging through accelerated image acquisition, reduced radiation, and improved tumor detection. Key methods include convolutional neural networks for tumor segmentation, radiomics for tumor characterization, and several tools for functional imaging. Challenges such as limited pediatric datasets, developmental variability, ethical concerns, and the need for explainable models were analyzed. Results: AI has shown significant potential to improve imaging quality, reduce scan times, and enhance diagnostic accuracy in pediatric neuroimaging, resulting in improved accuracy in tumor segmentation and outcome prediction for treatment. However, progress is hindered by the scarcity of pediatric datasets, issues with data sharing, and the ethical implications of applying AI in vulnerable populations. Conclusions: To overcome current limitations, future research should focus on building robust pediatric datasets, fostering multi-institutional collaborations for data sharing, and developing interpretable AI models that align with clinical practice and ethical standards. These efforts are essential in harnessing the full potential of AI in pediatric neuroimaging and improving outcomes for children with cancer. Full article
(This article belongs to the Topic AI in Medical Imaging and Image Processing)
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21 pages, 2812 KiB  
Review
Advances in the Management of Craniopharyngioma: A Narrative Review of Recent Developments and Clinical Strategies
by Mousa Javidialsaadi, Diego D. Luy, Heather L. Smith, Arba Cecia, Seunghyuk Daniel Yang and Anand V. Germanwala
J. Clin. Med. 2025, 14(4), 1101; https://doi.org/10.3390/jcm14041101 - 9 Feb 2025
Viewed by 2003
Abstract
Craniopharyngiomas (CPs) are rare intracranial tumors arising from remnants of Rathke’s pouch. Despite their benign histology, CPs present considerable clinical challenges due to their tendency to exert mass effect and adherence to critical neurovascular structures. There remains no clear consensus on the most [...] Read more.
Craniopharyngiomas (CPs) are rare intracranial tumors arising from remnants of Rathke’s pouch. Despite their benign histology, CPs present considerable clinical challenges due to their tendency to exert mass effect and adherence to critical neurovascular structures. There remains no clear consensus on the most effective management of CPs. We explore the latest developments in targeted treatment approaches, examining how innovations in surgery, radiation therapy, and emerging therapies are improving outcomes and management for these challenging tumors. In addition to providing our experience, we reviewed previously reported case series and reviews relevant to CPs. Studies show a bimodal age distribution for CP diagnosis, with peak incidence occurring in children aged 5–14 years and in adults aged 50–74 years. Surgical resection is the typical initial treatment for CPs, and adjuncts, including radiation therapy and intracavitary treatments, have been proven effective for tumor control. Additionally, genetic mutations associated with CPs offer an opportunity for novel strategies that address the underlying molecular mechanisms driving tumor growth through targeting the Wnt/β-catenin and/or MAPK/ERK pathways to disrupt the aberrant signaling that promotes tumor proliferation and survival. Survival rates for CPs are generally favorable (five-year survival of 80%), with recent studies showing improved outcomes and higher survival rates in children. CPs remain rare and challenging tumors. Although surgical resection is the main treatment, surgeons must weigh the benefits of achieving a gross total resection with the risks of iatrogenic injury. Adjuncts, including intracavitary and radiation treatments, may assist with subtotal resections and recurrences, as well as approved BRAF inhibitor therapy for the papillary variant. Many improvements in diagnostic and therapeutic methods were made after Dr. Cushing coined the term “craniopharyngioma”. Ongoing experiments, investigations, and advances in radiation techniques and molecular targeted therapies will provide patients with promise for safer and more effective treatments. Full article
(This article belongs to the Special Issue Updates on Brain Tumors: Diagnosis and Treatment)
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9 pages, 1987 KiB  
Article
Comparative Immunohistochemical Analysis of Craniopharyngioma and Ameloblastoma: Insights into Odontogenic Differentiation
by Ban A. Salih and Bashar H. Abdullah
Diagnostics 2024, 14(20), 2315; https://doi.org/10.3390/diagnostics14202315 - 17 Oct 2024
Viewed by 1031
Abstract
Background and objectives: Histopathological similarities between craniopharyngioma (CP) and ameloblastoma (AB) have long been recognized, particularly the shared features of palisading columnar epithelium and stellate reticulum-like areas. This study aimed to investigate potential odontogenic differentiation in CP akin to AB using immunohistochemical odontogenic [...] Read more.
Background and objectives: Histopathological similarities between craniopharyngioma (CP) and ameloblastoma (AB) have long been recognized, particularly the shared features of palisading columnar epithelium and stellate reticulum-like areas. This study aimed to investigate potential odontogenic differentiation in CP akin to AB using immunohistochemical odontogenic markers. Methods: We analyzed AMELX, ODAM, and CK19 expression in 44 cases (20 CP and 24 AB). Results: While AMELX and ODAM showed diffuse strong positive expression in both tumors with no significant statistical differences, CK19 expression was notably higher in CP. Conclusion: The markers AMELX and ODAM associated with odontogenic differentiation exhibited similar profiles in both tumors due to shared similar embryological origins. Notably, CK19, a biomarker of odontogenic epithelium, showed even higher expression, suggesting distinct pathways. These findings offer insights into tumor biology and may aid in diagnostic and therapeutic approaches. Full article
(This article belongs to the Special Issue Advances in the Diagnosis of Oral and Maxillofacial Disease)
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19 pages, 1159 KiB  
Systematic Review
Update on Neoadjuvant and Adjuvant BRAF Inhibitors in Papillary Craniopharyngioma: A Systematic Review
by Giulia Cossu, Daniele S. C. Ramsay, Roy T. Daniel, Ahmed El Cadhi, Luc Kerherve, Edouard Morlaix, Sayda A. Houidi, Clément Millot-Piccoli, Renan Chapon, Tuan Le Van, Catherine Cao, Walid Farah, Maxime Lleu, Olivier Baland, Jacques Beaurain, Jean Michel Petit, Brivaël Lemogne, Mahmoud Messerer and Moncef Berhouma
Cancers 2024, 16(20), 3479; https://doi.org/10.3390/cancers16203479 - 14 Oct 2024
Cited by 4 | Viewed by 1907
Abstract
Background/Objectives: The recent discovery of BRAF mutation in papillary craniopharyngiomas opened new avenues for targeted therapies to control tumour growth, decreasing the need for invasive treatments and relative complications. The aim of this systematic review was to summarize the recent scientific data [...] Read more.
Background/Objectives: The recent discovery of BRAF mutation in papillary craniopharyngiomas opened new avenues for targeted therapies to control tumour growth, decreasing the need for invasive treatments and relative complications. The aim of this systematic review was to summarize the recent scientific data dealing with the use of targeted therapies in papillary craniopharyngiomas, as adjuvant and neoadjuvant treatments. Methods: The PRISMA guidelines were followed with searches performed in Scopus, MEDLINE, and Embase, following a dedicated PICO approach. Results: We included 21 pertinent studies encompassing 53 patients: 26 patients received BRAF inhibitors (BRAFi) as adjuvant treatment, while 25 received them as neoadjuvant treatment. In the adjuvant setting, BRAFi were used to treat recurrent tumours after surgery or adjuvant radiation therapy. The most common regimen combined dabrafenib (BRAFi) with trametinib (MEK1 and 2 inhibitor) in 81% of cases. The mean treatment length was 8.8 months (range 1.6 to 28 months) and 32% were continuing BRAFi. A reduction of tumour volume variable from 24% to 100% was observed at cerebral MRI during treatment and volumetric reduction ≥80% was described in 64% of cases. Once the treatment was stopped, adjuvant treatments were performed to stabilize patients in remission in 11 cases (65%) or when a progression was detected in three cases (12%). In four cases no further therapies were administered (16%). Mean follow-up after the end of targeted therapy was 17.1 months. As neoadjuvant regimen, 36% of patients were treated with dabrafenib and trametinib with a near complete radiological response in all the cases with a mean treatment of 5.7 months. The neoadjuvant use of verumafenib (BRAFi) and cometinib (MEK1 inhibitor) induced a near complete response in 15 patients (94%), with a median volumetric reduction between 85% and 91%. Ten patients did not receive further treatments. Side effects varied among studies. The optimal timing, sequencing, and duration of treatment of these new therapies should be established. Moreover, questions remain about the choice of specific BRAF/MEK inhibitors, the optimal protocol of treatment, and the strategies for managing adverse events. Conclusions: Treatment is shifting to a wider multidisciplinary management, where a key role is played by targeted therapies, to improve outcomes and quality of life for patients with BRAF-mutated craniopharyngiomas. Future, larger comparative trials will optimize their protocol of use and integration into multimodal strategies of treatment. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and the Management of Intracranial Tumors)
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10 pages, 722 KiB  
Article
Internalizing Symptoms and Their Impact on Patient-Reported Health-Related Quality of Life and Fatigue among Patients with Craniopharyngioma During Proton Radiation Therapy
by Belinda N. Mandrell, Yian Guo, Yimei Li, Donna Hancock, Mary Caples, Jason M. Ashford, Thomas E. Merchant, Heather M. Conklin and Valerie Mc. Crabtree
Children 2024, 11(10), 1159; https://doi.org/10.3390/children11101159 - 25 Sep 2024
Cited by 2 | Viewed by 1092
Abstract
Objective: The aim of this study was to describe fatigue, health-related quality of life (HRQOL) and brain tumor-associated symptoms after surgical resection and during proton radiotherapy, using latent class analysis (LCA), and to determine if there is class membership change among pediatric patients [...] Read more.
Objective: The aim of this study was to describe fatigue, health-related quality of life (HRQOL) and brain tumor-associated symptoms after surgical resection and during proton radiotherapy, using latent class analysis (LCA), and to determine if there is class membership change among pediatric patients with craniopharyngioma. Methods: For all patients (n = 92), demographic and disease-related/clinical variables were attained, and patient reported outcomes were collected prior to proton therapy, at week three, and at the completion of proton therapy. The mean scores for fatigue, HRQOL, and brain tumor symptoms were compared over time and profiles were identified. Factors that influenced profile status and transition probability were examined. Results: Fatigue, HRQOL, and brain tumor symptoms improved over time during proton therapy; however, a subset remained in the lower profile, profile 1, associated with increased internalizing behaviors, compared to profile 2. Conclusions: Future study should explore the bidirectional relationship of sleep, worry and anxiety in the context of ongoing radiotherapy. Full article
(This article belongs to the Section Pediatric Hematology & Oncology)
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11 pages, 1775 KiB  
Perspective
From Hypothalamic Obesity to Metabolic Dysfunction-Associated Steatotic Liver Disease: Physiology Meets the Clinics via Metabolomics
by Amedeo Lonardo and Ralf Weiskirchen
Metabolites 2024, 14(8), 408; https://doi.org/10.3390/metabo14080408 - 26 Jul 2024
Cited by 2 | Viewed by 1654
Abstract
Metabolic health is tightly regulated by neuro-hormonal control, and systemic metabolic dysfunction may arise from altered function of the hypothalamic–anterior pituitary axis (HAPA). Ancient experimental observations of hypothalamic obesity (HO) and liver cirrhosis occurring among animals subjected to hypothalamic injury can now be [...] Read more.
Metabolic health is tightly regulated by neuro-hormonal control, and systemic metabolic dysfunction may arise from altered function of the hypothalamic–anterior pituitary axis (HAPA). Ancient experimental observations of hypothalamic obesity (HO) and liver cirrhosis occurring among animals subjected to hypothalamic injury can now be explained using the more recent concepts of lipotoxicity and metabolic dysfunction-associated steatotic liver disease (MASLD). Lipotoxicity, the range of abnormalities resulting from the harmful effects of fatty acids accumulated in organs outside of adipose tissue, is the common pathogenic factor underlying closely related conditions like hypothalamic syndrome, HO, and MASLD. The hormonal deficits and the array of metabolic and metabolomic disturbances that occur in cases of HO are discussed, along with the cellular and molecular mechanisms that lead, within the MASLD spectrum, from uncomplicated steatotic liver disease to steatohepatitis and cirrhosis. Emphasis is placed on knowledge gaps and how they can be addressed through novel studies. Future investigations should adopt precision medicine approaches by precisely defining the hormonal imbalances and metabolic dysfunctions involved in each individual patient with HO, thus paving the way for tailored management of MASLD that develops in the context of altered HAPA. Full article
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26 pages, 3258 KiB  
Review
Topography and Radiological Variables as Ancillary Parameters for Evaluating Tissue Adherence, Hypothalamic–Pituitary Dysfunction, and Recurrence in Craniopharyngioma: An Integrated Multidisciplinary Overview
by Rosalinda Calandrelli, Gabriella D’Apolito, Matia Martucci, Carolina Giordano, Chiara Schiarelli, Giammaria Marziali, Giuseppe Varcasia, Luca Ausili Cefaro, Sabrina Chiloiro, Simone Antonio De Sanctis, Simona Serioli, Francesco Doglietto and Simona Gaudino
Cancers 2024, 16(14), 2532; https://doi.org/10.3390/cancers16142532 - 13 Jul 2024
Cited by 3 | Viewed by 2032
Abstract
Craniopharyngiomas continue to present a challenge in clinical practice due to their heterogeneity and unpredictable adherence to vital neurovascular structures, particularly the hypothalamus. This results in different degrees of hypothalamus–pituitary axis dysfunction and a lack of uniform consensus and treatment guidelines regarding optimal [...] Read more.
Craniopharyngiomas continue to present a challenge in clinical practice due to their heterogeneity and unpredictable adherence to vital neurovascular structures, particularly the hypothalamus. This results in different degrees of hypothalamus–pituitary axis dysfunction and a lack of uniform consensus and treatment guidelines regarding optimal management. MRI and CT are complementary techniques in the preoperative diagnostic phase, enabling the precise definition of craniopharyngioma size, shape, and consistency, as well as guiding classification into histopathological subtypes and topographical categories. Meanwhile, MRI plays a crucial role in the immediate postoperative period and follow-up stages by identifying treatment-related changes and residual tumors. This pictorial essay aims to provide an overview of the role of imaging in identifying variables indicative of the adherence degree to the hypothalamus, hypothalamic–pituitary dysfunction, the extent of surgical excision, and prognosis. For a more comprehensive assessment, we choose to distinguish the following two scenarios: (1) the initial diagnosis phase, where we primarily discuss the role of radiological variables predictive of adhesions to the surrounding neurovascular structures and axis dysfunction which may influence the choice of surgical resection; (2) the early post-treatment follow-up phase, where we discuss the interpretation of treatment-related changes that impact outcomes. Full article
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18 pages, 7242 KiB  
Article
Serum Metabolomic and Lipidomic Profiling Reveals the Signature for Postoperative Obesity among Adult-Onset Craniopharyngioma
by Qiongyue Zhang, Yonghao Feng, Dou Wu, Yinyin Xie, Guoming Wu, Wei Wu, Hui Wang, Xiaoyu Liu, Linling Fan, Boni Xiang, Quanya Sun, Yiming Li, Yongfei Wang and Hongying Ye
Metabolites 2024, 14(6), 338; https://doi.org/10.3390/metabo14060338 - 17 Jun 2024
Cited by 2 | Viewed by 1566
Abstract
Craniopharyngioma patients often suffer from a diminished quality of life after surgery, which is usually associated with metabolic disorders and hypothalamic obesity. However, the precise etiology of these conditions remains elusive. To identify the metabolic changes after surgery, we conducted a cross-sectional study [...] Read more.
Craniopharyngioma patients often suffer from a diminished quality of life after surgery, which is usually associated with metabolic disorders and hypothalamic obesity. However, the precise etiology of these conditions remains elusive. To identify the metabolic changes after surgery, we conducted a cross-sectional study using metabolomic and lipidomic analysis to profile metabolic alterations in adult-onset craniopharyngioma patients with postoperative obesity. A cohort of 120 craniopharyngioma patients who had undergone surgery were examined. Differential analyses, including clinical characteristics, serum metabolome, and lipidome, were conducted across distinct body mass index (BMI) groups. Our findings indicated no statistically significant differences in age, sex, and fasting blood glucose among postoperative craniopharyngioma patients when stratified by BMI. However, a noteworthy difference was observed in uric acid and blood lipid levels. Further investigation revealed that alterations in metabolites and lipids were evidently correlated with increased BMI, indicating that postoperative obesity of craniopharyngioma patients affected their whole-body metabolism. Additionally, the multi-omics analysis identified specific metabolites and lipids, including uric acid and DG(18:2/20:4), as contributors to the metabolic disorders associated with postoperative obesity of craniopharyngioma patients. This work provides valuable insight into the involvement of metabolites and lipids in metabolic disorders subsequent to craniopharyngioma surgery. Full article
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16 pages, 874 KiB  
Systematic Review
Vascular Morbidity and Mortality in Craniopharyngioma Patients—A Scoping Review
by Julia Beckhaus, Carsten Friedrich and Hermann L. Müller
Cancers 2024, 16(6), 1099; https://doi.org/10.3390/cancers16061099 - 8 Mar 2024
Cited by 7 | Viewed by 2329
Abstract
Craniopharyngioma (CP) treatment, including surgery and radiotherapy, can have short- and long-term vascular side effects. Hypothalamic damage is related to morbid obesity and may increase the lifelong risk of experiencing vascular events in CP patients. This review summarized the available evidence regarding vascular [...] Read more.
Craniopharyngioma (CP) treatment, including surgery and radiotherapy, can have short- and long-term vascular side effects. Hypothalamic damage is related to morbid obesity and may increase the lifelong risk of experiencing vascular events in CP patients. This review summarized the available evidence regarding vascular complications in adamantinomatous or papillary CP patients, whatever their age at diagnosis. Three databases (Medline, CINAHL, Web of Science) were searched (06/2023) to retrieve eligible articles. The search was limited to peer-reviewed articles. Titles, abstracts, and full texts were screened by two independent reviewers, and data were extracted using a self-developed grid. Seventy-two studies were included in this review; the majority were case reports. Reported vascular sequela that occurred due to surgery were fusiform dilation of the carotid artery, stroke, vasospasm, hemorrhage, and aneurysm. Related conditions that emerged due to radiotherapy included Moyamoya syndrome and cavernoma. Cardiovascular morbidity and mortality often lead to hypothalamic obesity and metabolic syndrome in CP patients. Vascular damage is a rare complication of CP treatment. Surgical strategies should protect the surrounding hypothalamic and vascular structures. Patients receiving radiotherapy, particularly at a young age, should undergo magnetic resonance angiography monitoring to identify possible neurovascular sequela during post-treatment care. Full article
(This article belongs to the Section Systematic Review or Meta-Analysis in Cancer Research)
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21 pages, 1566 KiB  
Review
Advancing Craniopharyngioma Management: A Systematic Review of Current Targeted Therapies and Future Perspectives
by Edoardo Agosti, Marco Zeppieri, Sara Antonietti, Amedeo Piazza, Tamara Ius, Marco Maria Fontanella, Alessandro Fiorindi and Pier Paolo Panciani
Int. J. Mol. Sci. 2024, 25(2), 723; https://doi.org/10.3390/ijms25020723 - 5 Jan 2024
Cited by 14 | Viewed by 4799
Abstract
Craniopharyngiomas present unique challenges in surgical management due to their proximity to critical neurovascular structures. This systematic review investigates genetic and immunological markers as potential targets for therapy in craniopharyngiomas, assessing their involvement in tumorigenesis, and their influence on prognosis and treatment strategies. [...] Read more.
Craniopharyngiomas present unique challenges in surgical management due to their proximity to critical neurovascular structures. This systematic review investigates genetic and immunological markers as potential targets for therapy in craniopharyngiomas, assessing their involvement in tumorigenesis, and their influence on prognosis and treatment strategies. The systematic review adhered to PRISMA guidelines, with a thorough literature search conducted on PubMed, Ovid MED-LINE, and Ovid EMBASE. Employing MeSH terms and Boolean operators, the search focused on craniopharyngiomas, targeted or molecular therapy, and clinical outcomes or adverse events. Inclusion criteria encompassed English language studies, clinical trials (randomized or non-randomized), and investigations into adamantinomatous or papillary craniopharyngiomas. Targeted therapies, either standalone or combined with chemotherapy and/or radiotherapy, were examined if they included clinical outcomes or adverse event analysis. Primary outcomes assessed disease response through follow-up MRI scans, categorizing responses as follows: complete response (CR), near-complete response (NCR), partial response, and stable or progressive disease based on lesion regression percentages. Secondary outcomes included treatment type and duration, as well as adverse events. A total of 891 papers were initially identified, of which 26 studies spanning from 2000 to 2023 were finally included in the review. Two tables highlighted adamantinomatous and papillary craniopharyngiomas, encompassing 7 and 19 studies, respectively. For adamantinomatous craniopharyngiomas, Interferon-2α was the predominant targeted therapy (29%), whereas dabrafenib took precedence (70%) for papillary craniopharyngiomas. Treatment durations varied, ranging from 1.7 to 28 months. Positive responses, including CR or NCR, were observed in both types of craniopharyngiomas (29% CR for adamantinomatous; 32% CR for papillary). Adverse events, such as constitutional symptoms and skin changes, were reported, emphasizing the need for vigilant monitoring and personalized management to enhance treatment tolerability. Overall, the data highlighted a diverse landscape of targeted therapies with encouraging responses and manageable adverse events, underscoring the importance of ongoing research and individualized patient care in the exploration of treatment options for craniopharyngiomas. In the realm of targeted therapies for craniopharyngiomas, tocilizumab and dabrafenib emerged as prominent choices for adamantinomatous and papillary cases, respectively. While adverse events were common, their manageable nature underscored the importance of vigilant monitoring and personalized management. Acknowledging limitations, future research should prioritize larger, well-designed clinical trials and standardized treatment protocols to enhance our understanding of the impact of targeted therapies on craniopharyngioma patients. Full article
(This article belongs to the Special Issue Precision Medicine in Oncology)
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