Pediatric gliomas are highly heterogeneous and have unique molecular characteristics. They are not smaller versions of adult gliomas. This review emphasizes the basic points of surgery, radiotherapy, and chemotherapy for pediatric gliomas, introduces the similarities and differences with targeted therapy for adult gliomas, describes the important driver gene abnormalities involved in pediatric gliomas and the corresponding targeted therapy strategies, and focuses on the epigenetic treatment strategies for pediatric diffuse high-grade gliomas caused by histone H3 mutations, and looks forward to the future work of diagnosis and treatment of pediatric gliomas.
Craniopharyngioma is a kind of common benign intracranial tumor. Craniopharyngioma may originate anywhere in the pituitary stalk. The different origin sites of the tumor are related to its growth pattern, and the relationship between tumor and the suprasellar structures including vessels and nerves. At present, there are many different classifications of craniopharyngioma, which based on the origin of the tumor, anatomical location, and imaging characteristics. By reviewing and analyzing different classifications of craniopharyngioma, we attempt to analyze the relationship between tumor origin, growth pattern, imaging characteristics and microstructure. It was attempted to analyze the tumor origin and determine the adhesion with the surrounding structures by imaging characteristics, so as to guide the surgical method and prognosis.
Pediatric-type low-grade glioma (pLGG) is the most common central nervous system tumor in children, and most pLGG exhibits non-invasive clinical behavior with a good prognosis. Total surgical resection is an important prognostic factor, and patients can achieve long-term survival after total tumor resection. However, tumors in deep location such as brain stem and optic pathway cannot be completely removed by surgery. Chemotherapy is the first choice of adjuvant treatment when the clinical symptoms of pLGG worsen or imaging progress. In recent years, in addition to traditional chemotherapy, the development and application of targeted therapy drugs have also made great progress. This article reviews the research progress and challenges of chemotherapy and targeted therapy of pLGG, in order to provide guidance for clinical practice.
Cerebellar mutism syndrome (CMS) is a prevalent postoperative complication in children following posterior fossa tumor surgery, with a significantly variable incidence rate across different pathological types of tumors, being highest in medulloblastoma (24%-30%). The pathogenesis of CMS remains to be elucidated, but it is believed to be associated with damage to the cerebellocerebral circuits, harm to the fastigial nuclei of cerebellum and periaqueductal gray matter of the midbrain, and alterations in brain networks. Predictive models constructed based on the risk factors of CMS have not yet demonstrated the anticipated stability and have not been widely adopted in clinical settings. Pharmacological treatments are primarily based on clinical experience, yet their efficacy requires further validation, hence there is currently a lack of a clearly effective treatment method; non-pharmacological treatments, such as physical therapy, occupational therapy, and speech therapy, have shown some effect on improving the long-term quality of life. This review summarizes the pathogenesis and therapeutic advances of CMS, and future research should be dedicated to exploring the pathophysiological mechanisms of CMS, constructing more accurate predictive models, devising personalized treatment plans, and enhancing the long-term prognosis for children.
Pituitary adenoma is a benign intracranial tumor, commonly found in adults and occasionally in children. Pituitary adenoma is classified into secretory and non-secretory adenoma based on whether there is abnormal hormone secretion. The main manifestations include endocrine system abnormalities, visual disturbance and symptoms of intracranial hypertension. Although the incidence rate of pituitary adenoma in children is low, its physiological and psychological impact on children is obvious, diagnostic and therapeutic process is unique, and it has some characteristics, more complicated differential diagnosis than adults, such as more endocrine symptoms, poor paranasal sinus gasification, the impact of transnasal surgery on developing children, drug therapy and radiotherapy for children and individualization, postoperative endocrine regulation and subsequent development problems, etc.. Therefore, it is necessary to discuss pituitary adenoma in children independently from adults. This article reviews the clinical research progress of pediatric pituitary adenoma in recent years.
Objective: To investigate the imaging diagnosis and differential diagnosis methods for common tumors in the posterior fossa of children, and to propose a flow chart for the diagnosis of common tumors in the posterior fossa of children based on the limited diffusion of DWI according to the idea of localization diagnosis followed by qualitative diagnosis. Methods: A total of 118 pediatric patients with posterior fossa tumors who underwent surgical resection in Huashan Hospital, Fudan University from January 2021 to January 2024 were enrolled, and all of them underwent head CT and MRI examinations, and preoperative localization diagnosis and qualitative diagnosis were carried out accordingly. Then, based on whether DWI was diffusion limited, a flow chart for the diagnosis of common tumors in the posterior fossa of children was proposed. Results: A total of 118 children with posterior fossa tumors were localization and qualitatively diagnosed, including 41 cases of fourth ventricular tumors, including medulloblastoma (27 cases), pilocytic astrocytoma (7 cases), ependymoma (5 cases), choroid plexus papilloma (one case), and rosette-forming glioneuronal tumor (RGNT, one case). There were 38 cases of brainstem tumors, including 24 cases of diffuse midline glioma, H3 K27-altered, 5 cases of pilocytic astrocytoma, 3 cases of cavernous hemangioma, 2 cases of pediatric-type diffuse high-grade glioma (prone to diffuse midline glioma, H3 wild type), 2 cases of ganglio glioma, one case of atypical teratoid/rhabdoid tumor (AT/RT), and one case of pediatric-type diffuse low-grade glioma. There were 9 cases of cerebellopontine angle (CPA) tumors, including 3 cases of pilocytic astrocytoma, 2 cases of cholesteatoma, one case of medulloblastoma, one case of diffuse midline glioma, H3 K27-altered, one case of pilomyxoid astrocytoma, and one case of Ewing sarcoma. There were 30 cases of cerebellar tumors, including 15 cases of pilocytic astrocytoma, 7 cases of medulloblastoma, 2 cases of cavernous hemangioma, one case of ependymoma, one case of pediatric-type diffuse low- grade glioma, one case of pediatric-type diffuse high- grade glioma (prone to diffuse midline glioma, H3 wild type), one case of dysembryoplastic neuroepithelial tumor (DNT), one case of hamartoma, and one case of extrarenal rhabdomyomatoid tumor cerebellar metastases. Localization diagnosis, the accuracy of preoperative CT was 93.22% (110/118), while the accuracy of preoperative MRI was 100% (118/118). Qualitative diagnosis, 74 cases (62.71%) had accurate qualitative diagnosis, 23 cases (19.49%) had general diagnosis, and 21 cases (17.80%) had wrong diagnosis. The imaging differential diagnosis of posterior fossa tumors in children focuses on medulloblastoma, pilocytic astrocytoma, ependymoma, and diffuse midline glioma, H3 K27-altered. The incidence of limited diffusion of DWI in medulloblastoma, pilocytic astrocytoma and ependymoma outside the brainstem was 100% (35/35), 4% (1/25) and 5/6, and the difference among the three tumors was statistically significant (Z =-5.601, P = 0.000). The incidence of limited diffusion of DWI in diffuse midline glioma, H3 K27-altered and pilocytic astrocytoma in the brainstem was 79.17% (19/24) and 1/5, and the difference between the two tumors was also statistically significant (Fisher's exact possibility: P = 0.038). Conclusions: The early diagnosis of posterior fossa tumors in children is difficult, and DWI is an important basis for differential diagnosis.
Objective: To explore effective treatments and prognostic factors for diffuse intrinsic pontine glioma (DIPG). Methods: Clinical and imaging information and survival data of 14 DIPG patients, treated with radiotherapy combined with temozolomide and nitolizumab or radiotherapy combined with ACT001, were retrospectively analysed at Beijing Tiantan Hospital, Capital Medical University from April 2021 to January 2024. The median progression free survival (PFS) and overall survival (OS) were calculated using Kaplan-Meier survival curves, and multifactorial Cox regression analysis was used to investigate the effects of different factors on PFS and OS. Results: The objective response rate (ORR) was 10/14, and the median PFS and OS were 7.83 and 8.30 months, respectively. Multfactorial Cox regression analysis identified the absence of enhancement on baseline imaging as a good prognostic variable for both PFS (RR = 0.052, 95%CI: 0.006-0.416; P = 0.005) and OS (RR = 0.046, 95%CI: 0.005-0.413; P = 0.006), while male (RR = 0.085, 95%CI: 0.009-0.764; P = 0.028), older age (RR = 0.631, 95%CI: 0.423-0.942; P = 0.024), and the absence of symptoms of cranial nerve involvement at the onset (RR = 0.116, 95%CI: 0.017- 0.781; P = 0.027) were also good prognostic variables for OS. Conclusions: Female, younger age at diagnosis, cranial nerve involvement at the onset, and enhancement on baseline imaging are risk factors for the survival of children with DIPG.
Objective: To investigate the efficacy and safety of the targeted drug poly ADP-ribose polymerase (PARP) inhibitor niraparib combined with low-dose chemotherapy drugs cisplatin and etoposide in the treatment of pediatric recurrent PFA-type ependymoma. Methods: A total of 12 pediatric patients with recurrent PFA-type ependymoma, treated in Children's Hospital of Fudan University from January 2022 to January 2024, were included in the study. All patients received a treatment regimen combining niraparib with low-dose cisplatin and etoposide chemotherapy. The objective response rate (ORR) of the tumor was statistically evaluated to assess treatment efficacy, and patient tolerability and safety indicators, including liver and kidney function, cardiac function, nausea and vomiting, fatigue, bone marrow suppression, gastrointestinal reactions, and infections, were recorded. Results: Among 12 pediatric patients, 10 cases showed a tumor reduction of ≥ 30%, in which 2 cases showed a tumor reduction of 100%, while one case showed no change in tumor size, and one case showed tumor progression. The overall ORR was 10/12. Adverse reactions included 5 cases of severe vomiting, primarily occurring during chemotherapy period, and 8 cases of varying degrees of bone marrow suppression, all of which were alleviated after symptomatic treatment. Conclusions: Niraparib combined with low-dose chemotherapy drugs shows good efficacy and safety in the treatment of pediatric recurrent PFA-type ependymoma.
Objective: To compare the detection rates of epileptogenic foci in drug-refractory epilepsy children using video electroencephalography (VEEG), MRI and PET. Methods: The 24 h and 2 h VEEG, MRI and PET/CT or PET/MRI were conducted for 70 children with drug-refractory epilepsy in Children's Hospital of Nanjing Medical University from January 2020 to April 2022, and Raven's Standard Progressive Matrices (SPM) or Developmental Screening Test for Child under six (DST) based on their age (< 5 or ≥ 5 years old) were assessed for intelligence. Results: Among 70 children, 44 seizures were detected during 24 h VEEG, while 31 seizures were detected during 2 h VEEG, and there was statistically significant difference between the two methods (χ2 = 5.760, P = 0.015). The consistency of diagnosing single location/ unilateral abnormalities, bilateral abnormalities, or normal results was poor among VEEG, MRI and PET/CT or PET/MRI (κ = 0.194, P = 0.000), and the difference among the three methods was statistically significant (χ2 = 33.068, P = 0.001). The VEEG (χ2 = 25.000, P = 0.000) and PET/CT or PET/MRI (χ2 = 11.764, P = 0.002) had higher diagnostic capabilities than MRI, and PET/CT or PET/MRI had higher diagnostic capability than VEEG (χ2 = 8.048, P = 0.014). There were 41 children (58.57%) showed mental retardation, among them children with onset age ≤ 2 years had a higher rate compared to those with onset age > 2 years (χ2 = 6.193, P = 0.013), and children with abnormal VEEG background had a higher rate compared to those with normal VEEG background (χ2 = 4.868, P = 0.027). Conclusions: The detection rate of epileptiform discharges and clinical seizures can be increased by lengthening VEEG time at each time and increasing the number of times of VEEG. PET/CT or PET/MRI and VEEG can provide localization information of epileptic foci of children whose MRI results are negative. The mental retardation rate of children who have onset age ≤ 2 years and of abnormal VEEG background is higher.
Objective: To explore the application value of transnasal endoscopy combined with endovascular treatment in the operation of skull base tumors. Methods: A retrospective analysis was conducted on the clinical data of 9 patients who underwent endoscopic transnasal resection of skull base tumors and received endovascular treatment at Tianjin Huanhu Hospital from January 2017 to July 2022. To observe the tumor resection and evaluate the postoperative efficacy of nasopharyngeal carcinoma according to the World Health Organization (WHO) solid tumor measurement standards. During the follow- up period, Glasgow Outcome Scale (GOS) was used to assess postoperative neurological function recovery, and record tumor recurrence or progression. Complications related to endovascular treatment, such as pseudoaneurysm, spontaneous cerebral hemorrhage and cerebral infarction, were also recorded. Results: Among 9 patients, there were 4 patients confirmed donor artery or internal carotid artery (ICA) rupture by DSA before operation, one case underwent spring coil embolization before operation, one case had a carotid catheter sheath inserted in advance during operation, one case had successful hemostasis with spring coil embolization during operation, one case had successful hemostasis with covered stent implantation during operation, 4 cases had ICA rupture confirmed by DSA during operation, and one case had successful hemostasis with spring coil embolization after DSA confirmation of ICA rupture. The total resection rate was 8/9. During a follow-up of 32.00 (25.00, 48.50) months, all patients did not experience tumor recurrence or progression. At the last follow-up, the GOS scores were 4-5, indicating good recovery of neurological function. A patient with recurrent nasopharyngeal carcinoma who underwent immunotherapy had a recurrent pseudoaneurysm at the distal end of the ICA covered with a stent implanted in the petrous segment. Three months after the stent implantation, the patient underwent external carotid artery-radial artery-middle cerebral artery bypass surgery to successfully stop the bleeding without any postoperative complications. The remaining 8 patients did not experience complications such as spontaneous cerebral hemorrhage or pseudoaneurysm. All patients did not experience complications such as cerebral infarction. Conclusions: Evdovascular treatment is helpful in evaluating the relationship between skull base tumors and surrounding blood vessels before endoscopic transnasal resection of skull base tumors. It can effectively block the donor artery, reduce the probability of intraoperative bleeding, effectively treat the rupture of large blood vessels, and reduce the occurrence of serious complications. The efficacy and safety are both high.
Objective: To compare and analyze the clinical outcomes of endovascular treatment of anterior circulation large vessel occlusion (ac-LVO) caused by large artery atherosclerosis (LAA) and simple arterial embolization. Methods: From August 2020 to September 2022, 87 patients with ac-LVO hospitalized in The Second Affiliated Hospital of Bengbu Medical University were enrolled. All patients were treated with endovascular treatment, including intravenous thrombolysis, stent thrombectomy, aspiration thrombectomy or angioplasty (balloon dilatation or stent implantation). According to intraoperative findings (etiology), they were divided into LAA group (n = 32) and simple arterial embolization group (embolization group, n = 55). The primary outcome was the 90 d modified Rankin Scale (mRS) score, and the secondary outcomes were postoperative vascular recanalization rate [modified Thrombolysis Cerebral Infarction (mTICI)] and incidence of symptomatic cerebral hemorrhage 1 d after surgery. Results: The proportions of patients with atrial fibrillation (χ2 = 17.672, P = 0.000) and thrombus retrieval ≥ 3 times (χ2 = 10.606, P = 0.001) in the embolization group were higher than those in the LAA group, the proportion of intravenous thrombolysis in the embolization group was less than that in the LAA group (χ2 = 5.403, P = 0.020). However, the time from onset to operation (Z = 1.111, P = 0.267), the time from admission to operation (Z = 0.149, P = 0.882), preferred surgical approach for endovascular treatment (Fisher's exact probability: P = 0.153), as well as the rate of good prognosis (mRS score ≤ 2; χ2 = 0.004, P = 0.950) and mortality (χ2 = 0.035, P = 0.851) at 90 d after surgery, the rate of postoperative vascular recanalization (mTICI grade ≥ Ⅱ b; χ2 = 0.033, P = 0.856) and symptomatic cerebral hemorrhage rate 1 d after surgery (χ2 = 0.345, P = 0.557) for the secondary outcome were observed in both groups, the above differences were not statistically significant. Conclusions: The efficacy and prognosis of endovascular treatment of ac-LVO caused by LAA and simple arterial embolization has the same effect and clinical prognosis.
Glioma is the most malignant brain tumor with high mortality. Due to its complex biological characteristics such as malignant proliferation, invasion, drug resistance, and immunosuppression, the clinical treatment faces great difficulties. Although the survival rate has improved under surgery combined with radiotherapy and chemotherapy, the overall curative effect is still not obvious. As a kind of non coding RNA (ncRNA), microRNA (miRNA) which has been extensively studied in recent years is involved in the regulation of biological processes of normal tissues and various tumors (including glioma) in human. As a cancer suppressor gene, miRNA-145 plays a regulatory role in the progression of glioma through a variety of pathways, especially as a targeted drug transported to the tumor area through nanocarriers to treat tumors, becoming a current research hotspot. This article summarizes the role of miRNA-145 in pathological grading, tumor microenvironment, the regulation of glioma stem cells, drug resistance, and the prognosis of glioma, aiming to provide the reference for the diagnosis and treatment of glioma.