Pediatric Neurosurgery最新文献

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Advances and Clinical Trials Update in the Treatment of Diffuse Intrinsic Pontine Gliomas. 弥漫性内生性脑桥胶质瘤的治疗进展及临床研究进展。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 Epub Date: 2023-01-13 DOI: 10.1159/000529099
Cecilia Dalle Ore, Christina Coleman, Nalin Gupta, Sabine Mueller
{"title":"Advances and Clinical Trials Update in the Treatment of Diffuse Intrinsic Pontine Gliomas.","authors":"Cecilia Dalle Ore, Christina Coleman, Nalin Gupta, Sabine Mueller","doi":"10.1159/000529099","DOIUrl":"10.1159/000529099","url":null,"abstract":"<p><strong>Background: </strong>Diffuse intrinsic pontine gliomas (DIPGs) are high-grade gliomas (HGGs) that occur primarily in children, and represent a leading cause of death in pediatric patients with brain tumors with a median overall survival of only 8-11 months.</p><p><strong>Summary: </strong>While these lesions were previously thought to behave similarly to adult HGG, emerging data have demonstrated that DIPG is a biologically distinct entity from adult HGG frequently driven by mutations in the histone genes H3.3 and H3.1 not found in adult glioma. While biopsy of DIPG was historically felt to confer unacceptable risk of morbidity and mortality, multiple studies have demonstrated that stereotactic biopsy of DIPG is safe, allowing not only for improved understanding of DIPG but also forming the basis for protocols for personalized medicine in DIPG. However, current options for personalized medicine in DIPG are limited by the lack of efficacious targeted therapies for the mutations commonly found in DIPG. Multiple treatment modalities including targeted therapies, immunotherapy, convection-enhanced delivery, and focused ultrasound are in various stages of investigation.</p><p><strong>Key message: </strong>Increasing frequency of biopsy for DIPG has identified distinct driving mutations that may serve as therapeutic targets. Novel treatment modalities are under investigation.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"259-266"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10664325/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10531384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Radiotherapy for Primary Pediatric Central Nervous System Malignancies: Current Treatment Paradigms and Future Directions. 小儿原发性中枢神经系统恶性肿瘤的放射治疗:目前的治疗模式和未来的方向。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 Epub Date: 2023-09-13 DOI: 10.1159/000533777
Kevin X Liu, Daphne A Haas-Kogan, Hesham Elhalawani
{"title":"Radiotherapy for Primary Pediatric Central Nervous System Malignancies: Current Treatment Paradigms and Future Directions.","authors":"Kevin X Liu, Daphne A Haas-Kogan, Hesham Elhalawani","doi":"10.1159/000533777","DOIUrl":"10.1159/000533777","url":null,"abstract":"<p><strong>Background: </strong>Central nervous system tumors are the most common solid tumors in childhood. Treatment paradigms for pediatric central nervous system malignancies depend on elements including tumor histology, age of patient, and stage of disease. Radiotherapy is an important modality of treatment for many pediatric central nervous system malignancies.</p><p><strong>Summary: </strong>While radiation contributes to excellent overall survival rates for many patients, radiation also carries significant risks of long-term side effects including neurocognitive decline, hearing loss, growth impairment, neuroendocrine dysfunction, strokes, and secondary malignancies. In recent decades, clinical trials have demonstrated that with better imaging and staging along with more sophisticated radiation planning and treatment set-up verification, smaller treatment volumes can be utilized without decrement in survival. Furthermore, the development of intensity-modulated radiotherapy and proton-beam radiotherapy has greatly improved conformality of radiation.</p><p><strong>Key messages: </strong>Recent changes in radiation treatment paradigms have decreased risks of short- and long-term toxicity for common histologies and in different age groups. Future studies will continue to develop novel radiation regimens to improve outcomes in aggressive central nervous system tumors, integrate molecular subtypes to tailor radiation treatment, and decrease radiation-associated toxicity for long-term survivors.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"356-366"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10234386","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Lipoblastomatosis Extended into the Lumbar Spinal Canal in a Child: A Case Report. 儿童成脂细胞瘤扩展至腰椎管1例报告。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 DOI: 10.1159/000531548
Marina Saga, Akira Yamaura, Tadashi Miyagawa
{"title":"Lipoblastomatosis Extended into the Lumbar Spinal Canal in a Child: A Case Report.","authors":"Marina Saga,&nbsp;Akira Yamaura,&nbsp;Tadashi Miyagawa","doi":"10.1159/000531548","DOIUrl":"https://doi.org/10.1159/000531548","url":null,"abstract":"<p><strong>Introduction: </strong>Lipoblastoma and lipoblastomatosis are rare benign mesenchymal adipose tumors that originate from embryonic white adipocytes and occur most commonly in infancy and early childhood. Lipoblastomas occur in the extremities and trunk, including the retroperitoneum and peritoneal cavity. Therefore, infiltration into the spinal canal has rarely been reported.</p><p><strong>Case presentation: </strong>A 4-year-old girl presented to our clinic because of difficulty sitting on the floor with her legs straight. She also complained of enuresis and constipation for the past 6 months with persistent headaches and back pain evoked by body anteflexion. A magnetic resonance imaging revealed a massive lesion of the psoas major muscle, retroperitoneal, and subcutaneous spaces, extending into the spinal epidural space between L2 and S1. The patient underwent surgery which resulted in gross total removal of the tumor from the spinal canal. The mass was yellowish, soft, lobulated, fatty, and easily removed from the surrounding structures. Pathology confirmed the diagnosis of lipoblastoma. The postoperative course was uneventful, and the patient was discharged without any signs of neurological deficit.</p><p><strong>Conclusion: </strong>We herein discuss a rare case of lipoblastoma extending into the spinal canal, resulting in neurological symptoms. Although this tumor is benign with no potential for metastasis, it is prone to local recurrence. Therefore, close postoperative observation should be performed.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":"58 3","pages":"168-172"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10107820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Extraskeletal Myxoid Chondrosarcoma of the Jugular Foramen in a Pediatric Patient: A Case Report and Comprehensive Review of the Literature. 小儿颈静脉孔骨外黏液样软骨肉瘤1例报告及文献综合复习。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 DOI: 10.1159/000530990
Kadir Oktay, Araz Aliyev, Halil Emre Alcan, Seyda Erdogan, Kerem Mazhar Ozsoy, Nuri Eralp Cetinalp, Tahsin Erman
{"title":"Extraskeletal Myxoid Chondrosarcoma of the Jugular Foramen in a Pediatric Patient: A Case Report and Comprehensive Review of the Literature.","authors":"Kadir Oktay,&nbsp;Araz Aliyev,&nbsp;Halil Emre Alcan,&nbsp;Seyda Erdogan,&nbsp;Kerem Mazhar Ozsoy,&nbsp;Nuri Eralp Cetinalp,&nbsp;Tahsin Erman","doi":"10.1159/000530990","DOIUrl":"https://doi.org/10.1159/000530990","url":null,"abstract":"<p><strong>Introduction: </strong>Extraskeletal myxoid chondrosarcoma of the jugular foramen is a rare clinical entity, especially in the pediatric population. Thus, it can be confused with other pathologies.</p><p><strong>Case presentation: </strong>We report an extremely rare case of a 14-year-old female patient with jugular foramen myxoid chondrosarcoma that was completely removed through microsurgical resection.</p><p><strong>Conclusion: </strong>The primary purpose of the treatment is gross total resection of the chondrosarcomas. However, adjuvant methods such as radiotherapy should additionally be applied in patients who have high-grade diseases or cannot undergo gross total resection because of anatomic localization.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":"58 3","pages":"173-178"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10113664","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Chronic Pleural Effusion in Ventriculoperitoneal Shunt due to Diaphragmatic CSF Fistula: Report of a Case Treated by Endoscopic Choroid Plexus Coagulation and Literature Review. 膈脑脊液瘘致脑室腹腔分流慢性胸腔积液1例:内镜下脉络膜丛凝血治疗并文献复习。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 DOI: 10.1159/000530387
Simon Schmid, Andrea Bevot, Felix Neunhoeffer, Jörg Michel, Matthias U Kumpf, Matthias Reimold, Michael Hofbeck, Martin U Schuhmann
{"title":"Chronic Pleural Effusion in Ventriculoperitoneal Shunt due to Diaphragmatic CSF Fistula: Report of a Case Treated by Endoscopic Choroid Plexus Coagulation and Literature Review.","authors":"Simon Schmid,&nbsp;Andrea Bevot,&nbsp;Felix Neunhoeffer,&nbsp;Jörg Michel,&nbsp;Matthias U Kumpf,&nbsp;Matthias Reimold,&nbsp;Michael Hofbeck,&nbsp;Martin U Schuhmann","doi":"10.1159/000530387","DOIUrl":"https://doi.org/10.1159/000530387","url":null,"abstract":"<p><strong>Introduction: </strong>Chronic pleural cerebrospinal fluid (CSF) effusion is a rare complication after ventriculoperitoneal (VP) shunt insertion and only 18 cases in children and adults have been described so far without catheter dislocation to the intrathoracic cavity.</p><p><strong>Case presentation: </strong>We report on a 4-year-old girl with a complex history of underlying neurogenetic disorder, a hypoxic-ischemic encephalopathy after influenza A infection with septic shock and severe acute respiratory distress syndrome, followed by meningitis at the age of 10 months. In consequence, she developed a severe cerebral atrophy and post-meningitic hydrocephalus requiring placement of a VP shunt. At age 4, she was admitted with community-acquired mycoplasma pneumonia and developed increasing pleural effusions leading to severe respiratory distress and requiring continuous chest tube drainage (up to 1,000-1,400 mL/day) that could not be weaned. β trace protein, in CSF present at concentrations &gt;6 mg/L, was found in the pleural fluid at low concentrations of 2.7 mg/L. An abdomino-thoracic CSF fistula was finally proven by single photon emission computerized tomography combined with low-dose computer tomography. After shunt externalization, the pleural effusion stopped and the chest tube was removed. CSF production rate remains high above 500 mL/24 h. An atrial CSF shunt could not be placed, since a hemodynamically relevant atrial septum defect with frail circulatory balance would not have tolerated the large CSF volumes. Therefore, she underwent a total bilateral endoscopic choroid plexus laser coagulation (CPC) within the lateral ventricles via bi-occipital burr holes. Postoperatively CSF production rate went close to 0 mL and after external ventricular drain removal no signs and symptoms of hydrocephalus developed during a follow-up of now 2.5 years.</p><p><strong>Conclusion: </strong>In summary, pleural effusions in patients with VP shunt can rarely be caused by an abdomino-thoracic fistula, with non-elevated β-trace protein in the pleural fluid. The majority of reported cases in literature were treated by ventriculoatrial shunt. This is the 2nd reported case, which has been successfully treated by radical CPC alone including the temporal horn choroid plexus, making the child shunt independent.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":"58 3","pages":"160-167"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10116360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Sex Differences in Comorbidities of Pediatric Craniosynostosis at Presentation. 儿童颅缝闭锁并发疾病的性别差异。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 DOI: 10.1159/000528745
Peyton Presto, Reagan A Collins, John Garza, Omar Fadi Zeitouni, Laszlo Nagy
{"title":"Sex Differences in Comorbidities of Pediatric Craniosynostosis at Presentation.","authors":"Peyton Presto,&nbsp;Reagan A Collins,&nbsp;John Garza,&nbsp;Omar Fadi Zeitouni,&nbsp;Laszlo Nagy","doi":"10.1159/000528745","DOIUrl":"https://doi.org/10.1159/000528745","url":null,"abstract":"<p><strong>Introduction: </strong>Craniosynostosis is a common pediatric presentation in which the premature fusion of one or more cranial sutures results in a misshapen skull. This birth defect is often associated with comorbidities due to structural impacts on nearby anatomical features. While there is some evidence for a male predominance among craniosynostosis patients, little has been investigated regarding sex differences in comorbidities of this condition. This study seeks to explore potential sexual dimorphisms in craniosynostosis patients at the time of presentation.</p><p><strong>Methods: </strong>We conducted a retrospective, cross-sectional review of male and female non-syndromic craniosynostosis (NSC) patients between the ages of 1 month and 9 years that were evaluated at a 500-bed academic hospital or a 977-bed private hospital in Lubbock, TX, USA. Common comorbidities including ophthalmologic diagnoses, developmental delays, obstructive sleep apnea, chronic otitis media, hearing loss, chronic headaches, and seizure disorders were evaluated. The NSC cohort was compared to a similarly aged trauma group that represented the normal population.</p><p><strong>Results: </strong>175 NSC patients fit the inclusion criteria, of which 109 (62%) were male. A diagnosis of craniosynostosis was significantly associated with ophthalmological diagnoses (p < 0.0001), chronic otitis media (p < 0.0001), developmental delays (p < 0.0001), and hearing loss (p = 0.0047). Male NSC patients were less likely to present with ophthalmological diagnoses (p = 0.0010) or hearing loss (p = 0.0052) than females.</p><p><strong>Conclusions: </strong>Our findings expand on current literature evaluating possible comorbidities of NSC, particularly supporting the association with ophthalmological diagnoses, chronic otitis media, developmental delays, and hearing loss. We also report sex differences in ophthalmological diagnoses and hearing loss for NSC patients. These findings can serve to educate physicians of symptoms requiring prompt recognition and management in these patients.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":"58 1","pages":"8-17"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10064380/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9883169","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pediatric Primary Intracranial Malignant Melanoma: Case Report and Literature Review. 儿童原发性颅内恶性黑色素瘤:病例报告和文献复习。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 Epub Date: 2023-06-14 DOI: 10.1159/000531544
Mervyn Jr Lim, Enrica Ek Tan, Ru Xin Wong, Kenneth Te Chang, Marielle V Fortier, Tien Meng Cheong, Lee Ping Ng, Sharon Yy Low
{"title":"Pediatric Primary Intracranial Malignant Melanoma: Case Report and Literature Review.","authors":"Mervyn Jr Lim,&nbsp;Enrica Ek Tan,&nbsp;Ru Xin Wong,&nbsp;Kenneth Te Chang,&nbsp;Marielle V Fortier,&nbsp;Tien Meng Cheong,&nbsp;Lee Ping Ng,&nbsp;Sharon Yy Low","doi":"10.1159/000531544","DOIUrl":"10.1159/000531544","url":null,"abstract":"<p><strong>Introduction: </strong>Primary intracranial malignant melanoma (PIMM) is an extremely rare primary brain tumor with most cases diagnosed in adults. To date, there are only a few cases reported in the pediatric population. Owing to its infrequency, there are no established guidelines to treat this aggressive neoplasm. Recent insights suggest that PIMM are molecularly different between adults and children, whereby NRAS mutations drive tumor growth in the latter group. We present a unique case of PIMM in a pediatric patient and discuss the case in corroboration with current literature.</p><p><strong>Case presentation: </strong>A previously well 15-year-old male presented with progressive symptoms of raised intracranial pressure. Neuroimaging reported a large solid-cystic lesion with significant mass effect. He underwent gross total resection of the lesion that was reported to be a PIMM with pathogenic single nucleotide variant NRAS p.Gln61Lys. Further workup for cutaneous, uveal, and visceral malignant melanoma was negative. A trial of whole-brain radiotherapy followed by dual immune checkpoint inhibitors was commenced. Despite concerted efforts, the patient had aggressive tumor progression and eventually demised from his disease.</p><p><strong>Conclusion: </strong>We therein report a case of pediatric PIMM, in the context of the patient's clinical, radiological, histopathological, and molecular findings. This case highlights the therapeutic difficulties faced in disease management and contributes to the very limited pool of medical literature for this devastating primary brain tumor.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"223-230"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10005377","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Recent Advancements in Ependymoma: Challenges and Therapeutic Opportunities. 室管膜瘤的最新进展:挑战和治疗机会。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 Epub Date: 2023-05-02 DOI: 10.1159/000530868
Kelsey C Bertrand, Paul Klimo
{"title":"Recent Advancements in Ependymoma: Challenges and Therapeutic Opportunities.","authors":"Kelsey C Bertrand, Paul Klimo","doi":"10.1159/000530868","DOIUrl":"10.1159/000530868","url":null,"abstract":"<p><strong>Background: </strong>Ependymoma is one of the most common malignant pediatric brain tumors and can be difficult to treat. Over the last decade, much progress has been made in the understanding of the underlying molecular drivers within this group of tumors, but clinical outcomes remain unchanged.</p><p><strong>Summary: </strong>Here, we review the most recent molecular advances in pediatric ependymoma, evaluate results of recent clinical trials and discuss the ongoing challenges in the field and questions that remain.</p><p><strong>Key messages: </strong>The field of ependymoma has vastly changed over the last several decades with ten distinct molecular subgroups now described, but much progress needs to be made in developing new therapeutic strategies and targets.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"307-312"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9527231","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Patient- and Caregiver-Reported Outcome Measures after Single-Level Selective Dorsal Rhizotomy in Pediatric and Young Adult Patients with Spastic Cerebral Palsy. 儿童和年轻成人痉挛性脑瘫患者单级选择性脊神经根切断术后患者和护理人员报告的结果测量。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 Epub Date: 2023-04-19 DOI: 10.1159/000530748
Abeelan Rasadurai, Nicole Alexandra Frank, Ladina Aurea Greuter, Maria Licci, Peter Weber, Stephanie Jünemann, Raphael Guzman, Jehuda Soleman
{"title":"Patient- and Caregiver-Reported Outcome Measures after Single-Level Selective Dorsal Rhizotomy in Pediatric and Young Adult Patients with Spastic Cerebral Palsy.","authors":"Abeelan Rasadurai,&nbsp;Nicole Alexandra Frank,&nbsp;Ladina Aurea Greuter,&nbsp;Maria Licci,&nbsp;Peter Weber,&nbsp;Stephanie Jünemann,&nbsp;Raphael Guzman,&nbsp;Jehuda Soleman","doi":"10.1159/000530748","DOIUrl":"10.1159/000530748","url":null,"abstract":"<p><strong>Introduction: </strong>The aim of this cohort study was to assess the outcome of single-level selective dorsal rhizotomy (SDR) in children and young adults with spastic cerebral palsy (CP) treated at our institution, focusing on patient-reported outcome measures (PROMs) and quality of life (QoL) of patients and their caregivers.</p><p><strong>Methods: </strong>We included consecutive patients undergoing SDR from 2018 to 2020 at our institution. Subjective outcome was measured through PROMs, while functional outcome was measured through baseline characteristics, operative outcome, as well as short- and long-term follow-up. Furthermore, the effect of age at the time of surgery on patient/caregiver satisfaction was analyzed.</p><p><strong>Results: </strong>Seven patients (3 female, 43%) with a median age at surgery of 11.9 years (IQR 8.7-15.5) were included. All patients had a Gross Motor Function Classification (GMFCS) score of at least IV before surgery. Five surgeries were palliative and two non-palliative. Based on PROMs, SDR showed very good QoL and health-related outcome measures for both palliative and non-palliative patients. Patient/caregiver satisfaction was higher for the early subgroup (age ≤11) than the late subgroup (age &gt;11). Functional outcome showed reduced spasticity in both groups. Blood transfusions were never needed, while no cerebrospinal fluid leak, infection, or permanent morbidity was seen.</p><p><strong>Conclusion: </strong>Based on PROMs, SDR leads to high satisfaction and improved QoL, especially if done at an early age. Further studies with larger cohorts are necessary to underline and confirm our observations.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":"58 3","pages":"128-135"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10614523/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10116370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Molecular Update and Review of Current Trials in Paediatric Low-Grade Gliomas. 当前儿科低级别胶质瘤临床试验的分子更新和综述。
IF 0.7 4区 医学
Pediatric Neurosurgery Pub Date : 2023-01-01 Epub Date: 2023-08-21 DOI: 10.1159/000533703
Sarah Al-Jilaihawi, Stephen Lowis
{"title":"A Molecular Update and Review of Current Trials in Paediatric Low-Grade Gliomas.","authors":"Sarah Al-Jilaihawi, Stephen Lowis","doi":"10.1159/000533703","DOIUrl":"10.1159/000533703","url":null,"abstract":"<p><strong>Background: </strong>Paediatric low-grade gliomas (pLGGs) are the most common primary brain tumour in children. Though considered benign, slow-growing lesions with excellent overall survival, their long-term morbidity can be significant, both from the tumour and secondary to treatment. Vast progress has been made in recent years to better understand the molecular biology underlying pLGGs, with promising implications for new targeted therapeutic strategies.</p><p><strong>Summary: </strong>A multi-layered classification system of biologic subgroups, integrating distinct molecular and histological features has evolved to further our clinical understanding of these heterogeneous tumours. Though surgery and chemotherapy are the mainstays of treatment for pLGGs, many tumours are not amenable to surgery and/or progress after conventional chemotherapy. Therapies targeting common genetic aberrations in the RAS-mitogen-activated protein kinase (RAS/MAPK) pathway have been the focus of many recent studies and offer new therapeutic possibilities. Here, we summarise the updated molecular classification of pLGGs and provide a review of current treatment strategies, novel agents, and open trials.</p><p><strong>Key messages: </strong>(1) There is a need for treatment strategies in pLGG that provide lasting tumour control and better quality of survival through minimising toxicity and protecting against neurological, cognitive, and endocrine deficits. (2) The latest World Health Organisation classification of pLGG incorporates a growing wealth of molecular genetic information by grouping tumours into more biologically and molecularly defined entities that may enable better risk stratification of patients, and consideration for targeted therapies in the future. (3) Novel agents and molecular-targeted therapies offer new therapeutic possibilities in pLGG and have been the subject of many recent and currently open clinical studies. (4) Adequate molecular characterisation of pLGG is therefore imperative in today's clinical trials, and treatment responses should not only be evaluated radiologically but also using neurological, visual, and quality of life outcomes to truly understand treatment benefits.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"290-298"},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10038764","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
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