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Childs Nerv Syst 2022 Jun 27. Epub 2022 Jun 27.

Department of Neurosurgery, Copenhagen University Hospital Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen E, Denmark.

Purpose: Cerebellar mutism syndrome (CMS) is a severe neurological complication of posterior fossa tumour surgery in children, and postoperative speech impairment (POSI) is the main component. Left-handedness was previously suggested as a strong risk factor for POSI. The aim of this study was to investigate the relationship between handedness and the risk of POSI. Read More

Ochsner J 2022 ;22(2):176-181

Division of Neurosurgery, SBH Health System, Bronx, NY.

Medulloblastoma of the posterior fossa is commonly encountered in pediatric populations but rarely reported in adults. Adult cases of medulloblastoma typically occur in younger patients, tend to arise intra-axially within the cerebellar hemisphere, and usually exhibit classic histopathologic features. A 54-year-old male presented with headaches, dizziness, gait instability, and frequent falls that had worsened during the prior 3 months. Read More

Neurohospitalist 2022 Jul 22;12(3):544-549. Epub 2022 Apr 22.

Jefferson Headache Center, Thomas Jefferson University, Philadelphia, PA, USA.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are part of the trigeminal autonomic cephalalgia (TAC) group of headache disorders. Attacks present with repeated, severe, sharp, stabbing, or throbbing pain. Patients may experience a single attack, recurrent attacks with pain-free interictal periods, or a sawtooth pattern background pain with superimposed stabs. Read More

Int J Environ Res Public Health 2022 Jun 9;19(12). Epub 2022 Jun 9.

Physical and Rehabilitation Medicine, Ghent University Hospital, 9000 Ghent, Belgium.

Background: Short- and long-term consequences after treatment for childhood fossa posterior tumors are extensively reported in the literature; however, papers highlighting physical function throughout rehabilitation and its correlation with Intelligence Quotient (IQ) are sparse. This study aims to describe the physical functioning and IQ of these survivors, their progression during rehabilitation, and the association with histopathological tumor classification. Additionally, the correlation between gross motor functioning and cognitive functioning was investigated. Read More

Children (Basel) 2022 Jun 16;9(6). Epub 2022 Jun 16.

Pediatric Neurosurgery Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy.

This narrative review highlights the latest achievements in the field of post-surgical rehabilitation of posterior fossa tumors. Studies investigating the effects of cognitive rehabilitation programs have been considered, following a comprehensive literature search in the scientific electronic databases: Pubmed, Scopus, Plos One, and ScienceDirect. This review investigates the effects of cognitive remediation, with specific highlights for single cognitive domains. Read More

Front Surg 2022 6;9:915253. Epub 2022 Jun 6.

Department of Neurooncology, N. N. Burdenko National Medical Research Center of Neurosurgery, Ministry of Health of the Russian Federation, Moscow, Russia.

Tumors of the IV ventricle represent 1-5% of all intracranial lesions; they are implicated in 2/3 of the tumors of the ventricular system. According to modern standards, the first treatment stage for this pathology is microsurgical removal. Currently, for the removal of neoplasms of the IV ventricle and brainstem, the median suboccipital approach is widely used, followed by one of the microapproaches. Read More

Oper Neurosurg (Hagerstown) 2022 Jul 11;23(1):e58. Epub 2022 May 11.

Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School & University Hospital, New Brunswick, New Jersey, USA.

J Neuroinflammation 2022 Jun 20;19(1):159. Epub 2022 Jun 20.

Laboratory of Molecular Neuropathology, Pediatric Cancer Center, College of Pharmaceutical Sciences, Soochow University, Suzhou, China.

Background: Medulloblastoma (MB) is the most common malignant brain tumor in children. Approximately one-third of MB patients remain incurable. Understanding the molecular mechanism of MB tumorigenesis is, therefore, critical for developing specific and effective treatment strategies. Read More

Front Surg 2022 1;9:886438. Epub 2022 Jun 1.

Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan, China.

Objective: This study aims to identify the predictors of postoperative hydrocephalus in patients with posterior fossa tumors (PFTs) and guide the management of perioperative hydrocephalus.

Methods: We performed a single-institution, retrospective analysis of patients who underwent resection of PFTs in our department over a 10-year period (2011-2021). A total of 682 adult patients met the inclusion criteria and were divided into either a prophylactic external ventricular drainage (EVD) group or a nonprophylactic-EVD group. Read More

Cureus 2022 May 17;14(5):e25066. Epub 2022 May 17.

Department of Neurology, Mount Auburn Hospital, Harvard Medical School, Boston, USA.

Cranial dermoid cysts are rare, embryologic tumors containing fat, hair, and other ectodermal elements. They occur most frequently in the posterior fossa and are typically diagnosed as incidental findings on brain imaging done for an unrelated reason. Traumatic rupture of a previously unidentified intracranial dermoid cyst can mimic symptoms of post-concussion syndrome and should be ruled out with magnetic resonance imaging (MRI). Read More

Front Public Health 2022 2;10:914536. Epub 2022 Jun 2.

Department of Integrated Care Management Center, West China Hospital, Sichuan University, Chengdu, China.

Background: is the most common malignant brain tumor of childhood, accounting for 6 to 7 percent of all childhood CNS tumors. The purpose of this study was to evaluate the economic efficacy of a bevacizumab combined with + irinotecan regimen for the treatment of recurrent pediatric in China.

Methods: The data analyzed were from a randomized phase II screening trial that showed an improved survival benefit in child patients with recurrent treated with a T+I+B combination regimen. Read More

Genes Chromosomes Cancer 2022 Jun 18. Epub 2022 Jun 18.

The Division of Hematology, Oncology, Blood and Marrow Transplant, Nationwide Children's Hospital and The Ohio State University, 700 Children's Drive, Columbus, OH, USA.

Ependymal tumors are the third most common brain tumor under 14 years old. Even though metastatic disease is a rare event, it affects mostly young children and carries an adverse prognosis. The factors associated with dissemination and the best treatment approach have not yet been established and there is limited published data on how to manage metastatic disease, especially in patients under 3 years of age. Read More

J Immunother Cancer 2022 Jun;10(6)

Melanoma, Cancer Immunotherapy and Innovative Therapy, Istituto Nazionale Tumori IRCCS Fondazione G. Pascale, Napoli, Italy

The Sonic hedgehog (Shh) signaling pathway is an essential pathway in the human body that plays an important role in embryogenesis and tissue homeostasis. Aberrant activation of this pathway has been linked to the development of different diseases, ranging from cancer to immune dysregulation and infections.Uncontrolled activation of the pathway through sporadic mutations or other mechanisms is associated with cancer development and progression in various malignancies, such as basal cell carcinoma, medulloblastoma, pancreatic cancer, breast cancer and small-cell lung carcinoma. Read More

Child Care Health Dev 2022 Jun 16. Epub 2022 Jun 16.

Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.

Background: This study investigates the severeness of participation barriers in patients surviving paediatric posterior fossa tumours (PFT) many years after treatment. In the past, aetiological studies on adverse outcome have primarily focused on pathobiological risk factors. The current analysis aims to investigate the importance of environmental variables. Read More

J Clin Invest 2022 Jun 15;132(12). Epub 2022 Jun 15.

Neurooncol Adv 2022 Jan-Dec;4(1):vdac065. Epub 2022 May 12.

Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.

Background: The aim of this case-control study was to investigate the severity of the cerebellar cognitive affective syndrome (CCAS) in patients treated for pediatric posterior fossa tumors (PFT) and evaluate its diagnostic validity and predictive value for long-term effects.

Methods: Using neuropsychological test data from 56 patients with PFT (average age: 14 years), the severity of deficits in the CCAS core areas (executive functions, verbal functions, visuospatial abilities and emotions/behaviour) was examined. Neuropsychological and academic long-term outcomes of patients with CCAS were compared to two control groups of PFT patients (treated with either surgery or surgery followed by radio-/chemotherapy) without the syndrome. Read More

Pediatr Blood Cancer 2022 Jun 10:e29830. Epub 2022 Jun 10.

The Division of Hematology and Oncology, St. Louis Children's Hospital, Washington University, School of Medicine in St. Louis, Washington University, St. Louis, Missouri, USA.

Purpose: Primary germ cell tumors (GCTs) are the most common central nervous system (CNS) neoplasm in patients with Down syndrome (DS). However, a standard of care has not been established due to paucity of data.

Methods: A retrospective multi-institutional analysis was conducted, in addition to a comprehensive review of the literature. Read More

Commun Biol 2022 Jun 9;5(1):569. Epub 2022 Jun 9.

Donald K. Johnson Eye Institute, Krembil Research Institute, University Health Network, Toronto, ON, M5T 2S8, Canada.

Spontaneous mouse models of medulloblastoma (MB) offer a tractable system to study malignant progression in the brain. Mouse Sonic Hedgehog (Shh)-MB tumours first appear at postnatal stages as preneoplastic changes on the surface of the cerebellum, the external granule layer (EGL). Here we compared traditional histology and 3DISCO tissue clearing in combination with light sheet fluorescence microscopy (LSFM) to identify and quantify preneoplastic changes induced by disrupting stromal Norrin/Frizzled 4 (Fzd4) signalling, a potent tumour inhibitory signal in two mouse models of spontaneous Shh-MB. Read More

Genes Dev 2022 May;36(9-10):514-532

Tumor Initiation and Maintenance Program, National Cancer Institute-Designated Cancer Center, Sanford Burnham Prebys Medical Discovery Institute, La Jolla, California 92037, USA.

Medulloblastoma is an aggressive brain tumor that occurs predominantly in children. Despite intensive therapy, many patients die of the disease, and novel therapies are desperately needed. Although immunotherapy has shown promise in many cancers, the low mutational burden, limited infiltration of immune effector cells, and immune-suppressive microenvironment of medulloblastoma have led to the assumption that it is unlikely to respond to immunotherapy. Read More

Clin Neuroradiol 2022 Jun 8. Epub 2022 Jun 8.

Department of Radiology, Division of Neuroradiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Purpose: This study aimed to evaluate the application of apparent diffusion coefficient (ADC) histogram analysis to differentiate posterior fossa tumors (PFTs) in children.

Methods: A total of 175 pediatric patients with PFT, including 75 pilocytic astrocytomas (PA), 59 medulloblastomas, 16 ependymomas, and 13 atypical teratoid rhabdoid tumors (ATRT), were analyzed. Tumors were visually assessed using DWI trace and conventional MRI images and manually segmented and post-processed using parametric software (pMRI). Read More

Neurochirurgie 2022 Jun 3. Epub 2022 Jun 3.

Institute of Cancer & Genomic Science, University of Birmingham, B152TT, Birmingham, United Kingdom.

Background: Differential diagnosis between medulloblastoma (MB), ependymoma (EP) and astrocytoma (PA) is important due to differing medical treatment strategies and predicted survival. The aim of this study was to investigate non-invasive MRI-based radiomic analysis of whole tumors to classify the histologic tumor types of pediatric posterior fossa brain tumor and improve the accuracy of discrimination, using a random forest classifier.

Methods: MRI images of 99 patients, with 59 MBs, 13 EPs and 27 PAs histologically confirmed by surgery and pathology before treatment, were included in this retrospective study. Read More

Curr Med Imaging 2022 Jun 2. Epub 2022 Jun 2.

Department of Medical Oncology, Çukurova University School of Medicine, Adana, Turkey.

Introduction: Paragangliomas are tumors of neuroendocrine origin, may appear in different localizations, and are related to the autonomic nervous system. Paragangliomas are generally asymptomatic and may rarely appear with adrenergic symptoms, and clinical findings depend on the catecholamines they secrete. Extra-adrenal paragangliomas are mostly benign like all paragangliomas. Read More

ESMO Open 2022 May 30;7(3):100495. Epub 2022 May 30.

Department of Gynecology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. Electronic address:

Background: Up to 40% of patients with metastatic human epidermal growth factor receptor 2 (HER2)-positive breast cancer develop brain metastases (BMs). Understanding of clinical features of these patients with HER2-positive breast cancer and BMs is vital.

Patients And Methods: A total of 2948 patients from the Brain Metastases in Breast Cancer registry were available for this analysis, of whom 1311 had primary tumors with the HER2-positive subtype. Read More

Lancet 2022 05;399(10340):2065

Division of Neurosurgery, University of Connecticut Health Center, UConn Health, Farmington, CT, USA. Electronic address:

Anticancer Res 2022 Jun;42(6):3203-3207

Department of Neurological Surgery, Hospital Padilla, Tucumán, Argentina.

Background/aim: Extraventricular supratentorial ependymomas are rare entities. Most ependymomas are located at the infratentorial and intraventricular level, and only in a small group of cases they do not present continuity with the ventricular system. This is a case report of a patient with an atypical location of a cerebral ependymoma, which required the implementation of a complex and infrequent approach for its complete microsurgical removal. Read More

Anticancer Res 2022 Jun;42(6):3217-3230

Division of Clinical Chemistry and Biochemistry, and University Children's Hospital Zurich, Zurich, Switzerland;

Background: Eight human catalytic phosphoinositide 3-kinase (PI3K) isoforms exist which are subdivided into three classes. While class I isoforms have been well-studied in cancer, little is known about the functions of class II PI3Ks.

Materials And Methods: The expression pattern and functions of the class II PI3KC2β isoform were investigated in a panel of tumour samples and cell lines. Read More

Radiother Oncol 2022 May 28. Epub 2022 May 28.

Toulouse NeuroImaging Center (ToNIC), INSERM University of Toulouse Paul Sabatier, Toulouse, France; Radiation Oncology Department, Institut Claudius Regaud- Institut Universitaire du Cancer de Toulouse-Oncopole, Toulouse, France.

Introduction: In recent years, progress in pediatric posterior fossa tumor (PFT) treatments has improved survival rates. However, the majority of survivors present neurocognitive sequelae that impact academic achievement.

Methods: This review examines the literature from 2000 to 2020 on long-term outcomes in different memory systems for survivors of pediatric PFT, considering the impact of radiotherapy which is a well-known prognostic factor for global neurocognitive function. Read More

BMC Biol 2022 05 30;20(1):124. Epub 2022 May 30.

Department of Developmental Biology, Washington University School of Medicine, St Louis, MO, USA.

Background: Neurodevelopmental disorders increase brain tumor risk, suggesting that normal brain development may have protective properties. Mutations in epigenetic regulators are common in pediatric brain tumors, highlighting a potentially central role for disrupted epigenetic regulation of normal brain development in tumorigenesis. For example, lysine 27 to methionine mutation (H3K27M) in the H3F3A gene occurs frequently in Diffuse Intrinsic Pontine Gliomas (DIPGs), the most aggressive pediatric glioma. Read More

Front Oncol 2021 9;11:608129. Epub 2022 May 9.

School of Computer Science and Engineering, University of Electronic Science and Technology of China, Chengdu, China.

Aim: Multifocal desmoplastic infantile ganglioglioma/astrocytoma (DIA/DIG) has rarely been reported. Here, two cases have been presented, reviewing the literature and proposed treatment algorithms for this rare tumor.

Patients And Methods: We report two patients diagnosed with multifocal DIA/DIGs in West China Hospital. Read More

Clin Neurol Neurosurg 2022 Jul 18;218:107303. Epub 2022 May 18.

Department of Neurosurgery, Wessex Neurological Centre, Southampton General Hospital, Southampton, UK. Electronic address:

Tanycytic ependymomas are a rare spinal cord tumour arising from tanycyte cells lining the ventricle or spinal central canal. This is the first report of familial spinal tanycytic ependymoma occurring in two first degree relatives. Both patients underwent surgical resection of the intra-medullary tumours with good overall recovery. Read More