Child's Nervous SystemPub Date : 2024-11-01Epub Date: 2024-08-26DOI: 10.1007/s00381-024-06588-1
Gabriele Gaggero, Alessandro Consales, Mila Gualandri, Valerio Gaetano Vellone
{"title":"Rasmussen's encephalitis: a histological perspective in a 1-year-old child.","authors":"Gabriele Gaggero, Alessandro Consales, Mila Gualandri, Valerio Gaetano Vellone","doi":"10.1007/s00381-024-06588-1","DOIUrl":"10.1007/s00381-024-06588-1","url":null,"abstract":"","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142055073","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}
Child's Nervous SystemPub Date : 2024-11-01Epub Date: 2024-07-20DOI: 10.1007/s00381-024-06524-3
Addison Stewart, Andrew T Hale, Benjamin W Saccomano, Ariana S Barkley, Betsy D Hopson, Anastasia Arynchyna-Smith, James M Johnston, Brandon G Rocque, Jeffrey P Blount, Curtis J Rozzelle
{"title":"Neurosurgical management of Myelomeningocele in premature infants: a case series.","authors":"Addison Stewart, Andrew T Hale, Benjamin W Saccomano, Ariana S Barkley, Betsy D Hopson, Anastasia Arynchyna-Smith, James M Johnston, Brandon G Rocque, Jeffrey P Blount, Curtis J Rozzelle","doi":"10.1007/s00381-024-06524-3","DOIUrl":"10.1007/s00381-024-06524-3","url":null,"abstract":"<p><strong>Introduction: </strong>Myelomeningocele (MMC) is the most common neural tube defect, but rarely seen in premature infants. Most centers advocate for closure of MMC within 24 h of birth. However, this is not always possible in severely premature infants. Given the rarity of this patient population, we aimed to share our institutional experience and outcomes of severely premature infants with MMC.</p><p><strong>Methods: </strong>We performed a retrospective, observational review of premature infants (≤ 32 weeks gestational age) identified through our multidisciplinary spina bifida clinic (1995-2021) and surgical logs. Descriptive statistics were compiled about this sample including timing of MMC closure and incidence of adverse events such as sepsis, CSF diversion, meningitis, and death.</p><p><strong>Results: </strong>Eight patients were identified (50% male) with MMC who were born ≤ 32 weeks gestational age. Mean gestational age of the population was 27.3 weeks (SD 3.5). Median time to MMC closure was 1.5 days (IQR = 1-80.8). Five patients were taken for surgery within the recommended 48 h of birth; 2 patients underwent significantly delayed closure (107 and 139 days); and one patient's defect epithelized without surgical intervention. Six of eight patients required permanent cerebrospinal fluid (CSF) diversion (2 patients were treated with ventriculoperitoneal shunting (VPS), three were treated with endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) and 1 patient treated with ETV; mean of 3 years after birth, ranging from 1 day to 16 years). Two patients required more than one permanent CSF diversion procedure. Two patients developed sepsis (defined as meeting at least 2/4 SIRS criteria). In both cases of sepsis, patients developed signs and symptoms more than 72 h after birth. Notably, both instances of sepsis occurred unrelated to operative intervention as they occurred before permanent MMC closure. Two patients had intraventricular hemorrhage (both grade III). No patients developed meningitis (defined as positive CSF cultures) prior to MMC closure. Median follow up duration was 9.7 years. During this time epoch, 3 patients died: Two before 2 years of age of causes unrelated to surgical intervention. One of the two patients with grade III IVH died within 24 h of MMC closure.</p><p><strong>Conclusions: </strong>In our institutional experience with premature infants with MMC, some patients underwent delayed MMC closure. The overall rate of meningitis, sepsis, and mortality for preterm children with MMC was similar to MMC patients born at term.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11538150/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141726973","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}
Child's Nervous SystemPub Date : 2024-11-01Epub Date: 2024-06-08DOI: 10.1007/s00381-024-06446-0
Bilal Abbasoglu, Hazan Basak, Bahir Mete, Elif Gokalp, Murat Zaimoglu, Suha Beton, Cem Meco, Gokmen Kahilogulları
{"title":"Effect of endoscopic endonasal skull base approaches on olfactory function and facial development in children.","authors":"Bilal Abbasoglu, Hazan Basak, Bahir Mete, Elif Gokalp, Murat Zaimoglu, Suha Beton, Cem Meco, Gokmen Kahilogulları","doi":"10.1007/s00381-024-06446-0","DOIUrl":"10.1007/s00381-024-06446-0","url":null,"abstract":"<p><strong>Introduction: </strong>Endoscopic approaches for skull base pathologies are increasingly being performed, and the subsequent complications, especially in the long term, have also been partially clarified. However, there is no information on the effects of endoscopic endonasal skull base surgeries (EESBS) in children on odor and facial development in parallel with long-term development. We conducted this study to investigate postoperative olfactory function and facial development in pediatric patients who underwent skull base surgery using the endoscopic endonasal method.</p><p><strong>Methods: </strong>We evaluated the smell test, sinonasal symptoms, and facial development of pediatric patients who underwent endoscopic endonasal skull base surgery after long-term follow-up. Odor was evaluated using the \"Sniffin' Sticks\" test kit, which assessed the T (odor threshold), D (odor discrimination), and I (odor identification) parameters. Sinonasal symptoms were evaluated using the SNOT-22 (sinus-nose outcome test) questionnaire. SNA (sella-nasion-A point), SNB (sella-nasion-B point), and ANB (A point-nasion-B point) angles were calculated from maxillofacial tomography and magnetic resonance imaging) to evaluate facial development. Data were compared with those of the healthy control group.</p><p><strong>Results: </strong>We included 30 patients comprising 19 (63.3%) boys and 11 (36.7%) girls, with no age difference between case and control groups. The mean follow-up period was 7 years. Odor test data, cephalometric measurements, and SNOT-22 analysis results showed no statistically significant differences between the two groups.</p><p><strong>Conclusion: </strong>To our knowledge, this is a comprehensive study with the longest follow-up period in terms of evaluation of facial development after EESBS in children to analyze odor using the Sniffin' Sticks test kit and the quality of life using SNOT-22. Olfactory function, facial development, and quality of life remained unaffected after long-term follow-up after EESBS in children. Although this surgical approach is minimally invasive, we recommend considering the possibility of complications, and the procedure should be performed by an experienced surgical team with adequate equipment.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141293211","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}
Child's Nervous SystemPub Date : 2024-11-01Epub Date: 2024-06-25DOI: 10.1007/s00381-024-06512-7
Eeshan Khurana, Shaan Mody, Tanisha Shah, John-Paul Bouffard, Maria Pedemonte, Gianna Holover, Jessica S Lee, Gregg Jacob, Sara Scheid, Robert Morin, Catherine Mazzola
{"title":"Pediatric skull inflammatory myofibroblastic tumor: a rare case report and literature review.","authors":"Eeshan Khurana, Shaan Mody, Tanisha Shah, John-Paul Bouffard, Maria Pedemonte, Gianna Holover, Jessica S Lee, Gregg Jacob, Sara Scheid, Robert Morin, Catherine Mazzola","doi":"10.1007/s00381-024-06512-7","DOIUrl":"10.1007/s00381-024-06512-7","url":null,"abstract":"<p><p>Inflammatory myofibroblastic tumors (IMTs) represent rare neoplasms, particularly infrequent in the pediatric skull. We present a novel case of a newborn male with a 5 cm right temporal mass and discuss current diagnostic and treatment options for IMTs. A multidisciplinary effort to surgically remove the lesion was successful, and the patient's skull defect healed without neurological deficits. The etiology of IMTs remains elusive, with proposed associations with chromosomal mutations in the anaplastic lymphoma kinase (ALK) gene. Surgical excision remains the primary treatment for IMTs. Promising pharmacological treatments, like Crizotinib, warrant further research into understanding potential alternatives in IMT management.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141449847","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}
Child's Nervous SystemPub Date : 2024-11-01Epub Date: 2024-07-27DOI: 10.1007/s00381-024-06541-2
Bheru Dan Charan, Savyasachi Jain, Leve Joseph Devarajan Sebastian, Sushant Agarwal, Ajay Garg
{"title":"Coexistence of neural tube defects and spinal arteriovenous shunts: a case series and review of literature.","authors":"Bheru Dan Charan, Savyasachi Jain, Leve Joseph Devarajan Sebastian, Sushant Agarwal, Ajay Garg","doi":"10.1007/s00381-024-06541-2","DOIUrl":"10.1007/s00381-024-06541-2","url":null,"abstract":"<p><strong>Background: </strong>Spinal arteriovenous shunts and spinal dysraphism both have a different underlying cause, disease spectrum and developmental process; hence, these entities rarely coexist in a patient. Here, we reported four cases of coexistence of adult-onset spinal arteriovenous shunt and spinal dysraphism in the same patient along with their therapeutic embolisation. Additionally, we conducted an extensive literature review to explore the potential theories and explanations for this coexistence.</p><p><strong>Methods: </strong>We retrospectively searched our imaging database from January 2015 to December 2023 to identify instances of spinal arteriovenous shunts occurring in patients with spinal dysraphism or neural tube defect disorders. MRI and angiographic imaging, clinical presentation, treatment and follow-up were analysed.</p><p><strong>Results: </strong>Four patients with arteriovenous fistula/shunt and spinal dysraphism were included in the study. The mean age of presentation was 35.5 years. The most common symptoms were sensory disturbance and motor weakness. Arteriovenous fistula or shunt was located at the lumber region in one patient and at the sacral region in three cases. Two patients have a prior history of surgery in first decade. Two patients were treated with glue embolisation. The internal iliac artery was a common feeder in all cases.</p><p><strong>Conclusions: </strong>The rare coexistence of neural tube defects with spinal vascular abnormalities should be considered when assessing a middle-aged patient with neural tube defect and myelopathy. Correct diagnosis can help in treatment planning and thereby improve prognosis.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141765637","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}
Child's Nervous SystemPub Date : 2024-11-01Epub Date: 2024-08-26DOI: 10.1007/s00381-024-06585-4
Yamila Basilotta Márquez, Agustin Ruiz Johnson, Guillermo Neumann Cafferata, Sebastian G Jaimovich
{"title":"Analysis of the different pathways of ectopic recurrence of craniopharyngioma in pediatric patients: presentation of cases and review of the literature.","authors":"Yamila Basilotta Márquez, Agustin Ruiz Johnson, Guillermo Neumann Cafferata, Sebastian G Jaimovich","doi":"10.1007/s00381-024-06585-4","DOIUrl":"10.1007/s00381-024-06585-4","url":null,"abstract":"<p><strong>Purpose: </strong>Craniopharyngioma is a tumor derived from the squamous epithelium of Rathke's pouch. Despite successful excision, recurrence is common, typically occurring at the original tumor site. More rarely, recurrences can manifest at distant locations. This article reports on three distinct types of ectopic recurrence and reviews the existing literature.</p><p><strong>Methods: </strong>We reviewed clinical records and neuroimaging data of craniopharyngioma patients at our institution, identifying three cases of ectopic recurrence. Additionally, we conducted a literature review of similar cases published between 1975 and 2023, focusing on historical background, pathophysiology, clinical and radiological features, and treatment options.</p><p><strong>Results: </strong>We identified nineteen articles detailing ectopic recurrence of craniopharyngiomas in pediatric patients. The right frontal lobe was the most frequently reported site of recurrence. The shortest interval to recurrence was 11 months, while the longest was 14 years. Most cases were managed with surgical resection, yielding positive outcomes. In our cases, the recurrence sites were temporal intraparenchymal, intraosseous orbital, and occipital intraventricular. All were successfully treated with surgery, with no subsequent recurrences.</p><p><strong>Conclusion: </strong>Although craniopharyngiomas are histologically benign, they can recur locally and, more rarely, at distant sites. Surgical intervention is generally well-tolerated. Further research into tumor cell dissemination mechanisms is essential to develop strategies for preventing ectopic recurrence.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142072211","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}
Child's Nervous SystemPub Date : 2024-11-01Epub Date: 2024-09-30DOI: 10.1007/s00381-024-06637-9
Salaar Ahmed, Muhammad Abdullah, Muhammad Ali Akbar Khan, Shahzadi Resham, Bilal Mazhar Qureshi, Naureen Mushtaq
{"title":"The trends in diagnosis, management, and care of patients with diffuse intrinsic pontine gliomas: Perspectives from a tertiary care hospital of pakistan.","authors":"Salaar Ahmed, Muhammad Abdullah, Muhammad Ali Akbar Khan, Shahzadi Resham, Bilal Mazhar Qureshi, Naureen Mushtaq","doi":"10.1007/s00381-024-06637-9","DOIUrl":"10.1007/s00381-024-06637-9","url":null,"abstract":"<p><strong>Background: </strong>Diffuse intrinsic pontine glioma (DIPG) primarily affects pediatric patients. Data on the global incidence of DIPG remain sparse, especially in South Asia and low-middle-income countries like Pakistan.</p><p><strong>Methods: </strong>After exemption from the Ethics Review Committee, a retrospective study was conducted. Records of patients with DIPG at the Aga Khan Hospital in Karachi, from January 2010 to December 2022, were reviewed.</p><p><strong>Results: </strong>A total of 35 pediatric patients were managed for DIPG. The median age of the patients was 9, with 19 (54.3%) males and 16 (45.7%) females. Cranial nerve palsies were the most common complaint and were present in 19 (54.3%) patients, followed by headaches in 18 (51.4%), long tract signs in 14 (40%), ataxia/cerebellar symptoms in 14 (40%), and seizures in 5 (14.3%). MRI was the primary diagnostic tool, used alone or with CT in 32 (94.1%) patients; CT alone was used in only 2 (5.7%) patients. Biopsy was performed in 10 (28.6%) patients. Primary radiation therapy was administered to 14 (40%) patients with 5400 cGy in 30 fractions. All these patients received steroids while none of them received reirradiation. VP shunt surgery for hydrocephalus was performed in 9 (25.7%) patients. Over half (54.3%) refused treatment post-diagnosis, and 71.4% were lost to follow-up.</p><p><strong>Conclusion: </strong>Providing timely, quality multi-disciplinary care to DIPG patients within resource constraints remains challenging in Pakistan. However, recent developments show promise for improving DIPG care in the country.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342667","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}
Child's Nervous SystemPub Date : 2024-11-01Epub Date: 2024-10-03DOI: 10.1007/s00381-024-06629-9
Natália Dassi, Andrea Maria Cappellano, Fabiola Isabel S de Sousa, Rosana Fiorini Puccini, Nasjla Saba da Silva, Maria Wany Louzada Strufaldi
{"title":"Unlocking delays: revealing barriers to early diagnosis of childhood central nervous system tumors in an upper-middle-income country.","authors":"Natália Dassi, Andrea Maria Cappellano, Fabiola Isabel S de Sousa, Rosana Fiorini Puccini, Nasjla Saba da Silva, Maria Wany Louzada Strufaldi","doi":"10.1007/s00381-024-06629-9","DOIUrl":"10.1007/s00381-024-06629-9","url":null,"abstract":"<p><strong>Purpose: </strong>Childhood central nervous system (CNS) tumors tend to have a longer time interval until diagnosis than other pediatric malignancies. The aim is to describe the time to diagnosis among Brazilian pediatric patients treated at a tertiary center and explore associated factors.</p><p><strong>Methods: </strong>Cross-sectional study; application of questionnaires to parents of children with CNS tumors during outpatient visit or inpatient care.</p><p><strong>Results: </strong>One hundred parents participated between August and November 2023. The median age of the children at diagnosis was 7.2 years old. Low-grade glioma (LGG) was the most common tumor type (37%), followed by medulloblastoma (24%). The most frequent symptoms were morning and/or persistent vomiting and headache. The mean prediagnostic symptomatic interval (PSI) was 150 days. The mean parental interval was shorter than the medical (58.1 days vs 92.8 days). LGGs and tumors located in the central area had longer intervals to diagnosis than other tumors (296 vs 54 days) (p = 0.005) and (206 vs 155 days) (p = 0.007), respectively. Despite 81% of the patients undergoing pediatric routine follow-up, 87% of them had been diagnosed at an emergency department. Children attended by the same physician had a shorter mean interval (18.2 vs 88.3 days) than those assisted by different professionals (p = 0.015). The mean time for referral to our specialized center was 23 days.</p><p><strong>Conclusions: </strong>This study is a crucial step in recognizing barriers to early diagnosis of CNS tumors in a middle-income country as low awareness of signs/symptoms by parents and health professionals, aiming to provide opportunities for intervention strategies to reduce the time to diagnosis.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142364588","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}
Nikhila Reddy, Shailesh B Gaikwad, Savyasachi Jain, Bheru Dan Charan, Shariq Ahmad Shah
{"title":"High flow pial arteriovenous fistula with dural sinus malformation of the posterior circulation.","authors":"Nikhila Reddy, Shailesh B Gaikwad, Savyasachi Jain, Bheru Dan Charan, Shariq Ahmad Shah","doi":"10.1007/s00381-024-06650-y","DOIUrl":"https://doi.org/10.1007/s00381-024-06650-y","url":null,"abstract":"<p><p>Posterior fossa congenital pial arteriovenous fistulas are rare vascular anomalies associated with high morbidity. These anomalies often present challenges to neurointerventionists due to their complex morphological features. We successfully treated two technically challenging, infratentorial large pial arteriovenous fistulas (AVFs) associated with complete flow steal in the basilar artery. The first case involved an 18-year-old male with a posterior fossa single-hole AVF characterized by dilated venous pouches and retrograde venous reflux. After an unsuccessful initial coiling attempt, this patient was treated using a double microcatheter coiling technique, supported by a large balloon for flow control. The second case involved a 9-month-old infant with a complex two-hole posterior fossa pial AVF and a large venous sac. This patient was treated with coil embolization of the fistula and with balloon protection of the vertebrobasilar junction. Both patients recovered without postoperative complications and showed significant improvement on follow-up. These cases provide valuable insights into the management and characteristics of pial arteriovenous fistulas.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142544063","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}
Ziting He, Xian Jiang, Zhouyu Wang, Ningning Luo, Ye Song
{"title":"A patient with heterochronous double primary tumor of basal ganglia germ cell tumors followed by diffuse hemispheric glioma: a case report.","authors":"Ziting He, Xian Jiang, Zhouyu Wang, Ningning Luo, Ye Song","doi":"10.1007/s00381-024-06644-w","DOIUrl":"https://doi.org/10.1007/s00381-024-06644-w","url":null,"abstract":"<p><strong>Background: </strong>Basal ganglia germ cell tumor (BGGCT) is a rare central nervous system (CNS) tumor. Diffuse hemispheric gliomas, H3 G34-mutant (DHGs) is an invasive glioma involving the cerebral hemispheres. The diagnosis of DHGs depends on the integration of histopathology and molecular pathology.</p><p><strong>Case report: </strong>We reported a patient with an initial diagnosis of BGGCT that was sensitive to subsequent chemoradiotherapy. Unfortunately, a second high-grade glioma was found on magnetic resonance imaging (MRI) six years later. Subsequently, the tumor was completely removed after surgery and the following histopathology plus next generation sequencing (NGS) testing confirmed the diagnosis of DHGs. Interestingly, we found a germline likely pathogenic variant in FANCA. After surgery, the patient received Stupp regimen. The patient had a relapse 13 months after the Stupp regimen and was doing well after surgery.</p><p><strong>Conclusions: </strong>This is the first report of a patient with heterochronous double primary tumor of BGGCT followed by DHGs.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142521119","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}