keyword
https://read.qxmd.com/read/38630553/a-cohort-study-of-cns-tumors-in-multiple-endocrine-neoplasia-type-1
#1
JOURNAL ARTICLE
Thomas Graillon, Pauline Romanet, Clara Camilla, Camille Gelin, Romain Appay, Catherine Roche, Arnaud Lagarde, Grégory Mougel, Kaissar Farah, Maëlle Le Bras, Julien Engelhardt, Michel Kalamarides, Matthieu Peyre, Aymeric Amelot, Evelyne Emery, Elsa Magro, Helene Cebula, Rabih Aboukais, Catherine Bauters, Emmanuel Jouanneau, Moncef Berhouma, Thomas Cuny, Henry Dufour, Hugues Loiseau, Dominique Figarella-Branger, Luc Bauchet, Christine Binquet, Anne Barlier, Pierre Goudet
PURPOSE: Multiple Endocrine Neoplasia Type-1 (MEN1) is thought to increase the risk of meningioma and ependymoma. Hereby, we aimed to describe the frequency, the incidence and specific clinical and histological features of CNS tumors in the MEN1 population (except pituitary tumors). EXPERIMENTAL DESIGN: The study population included patients harboring CNS tumors diagnosed with MEN1 syndrome after 1990 and followed-up in the French MEN1 national cohort. Standardized incidence rate (SIR) was calculated based on the French Gironde CNS tumors registry...
April 17, 2024: Clinical Cancer Research
https://read.qxmd.com/read/38619273/commentary-impact-of-molecular-subgroups-on-prognosis-and-survival-outcomes-in-posterior-fossa-ependymomas-a-retrospective-study-of-412-cases
#2
JOURNAL ARTICLE
Marios Lampros, Spyridon Voulgaris, George A Alexiou
No abstract text is available yet for this article.
April 15, 2024: Neurosurgery
https://read.qxmd.com/read/38608817/predictive-factors-of-long-term-neurological-outcome-and-progression-free-survival-in-intramedullary-spinal-cord-tumors-a-10-year-single-center-cohort-study-and-review-of-the-literature
#3
JOURNAL ARTICLE
Maria Pia Tropeano, Zefferino Rossini, Andrea Franzini, Ali Baram, Donato Creatura, Luca Raspagliesi, Federico Pessina, Maurizio Fornari
BACKGROUND: Intramedullary spinal cord tumors (IMSCTs) are a rare subgroup of neoplasms, encompassing both benign, slow-growing masses and malignant lesions; radical surgical excision represents the cornerstone of treatment for such pathologies regardless of histopathology, which, on the other hand, is a known predictor of survival and neurological outcome post-surgery. The present study aims to investigate the relevance of other factors in predicting survival and long-term functional outcome...
April 10, 2024: World Neurosurgery
https://read.qxmd.com/read/38600765/a-supratentorial-mass-in-a-young-adult-with-25%C3%A2-years-of-follow-up
#4
Francesca Baciorri, Samantha Sarcognato, Francesco Di Paola, Evelina Miele, Sabrina Rossi
No abstract text is available yet for this article.
April 10, 2024: Brain Pathology
https://read.qxmd.com/read/38592530/pediatric-like-brain-tumors-in-adults
#5
REVIEW
Sandra Fernandes Dias, Oliver Richards, Martin Elliot, Paul Chumas
Pediatric brain tumors are different to those found in adults in pathological type, anatomical site, molecular signature, and probable tumor drivers. Although these tumors usually occur in childhood, they also rarely present in adult patients, either as a de novo diagnosis or as a delayed recurrence of a pediatric tumor in the setting of a patient that has transitioned into adult services.Due to the rarity of pediatric-like tumors in adults, the literature on these tumor types in adults is often limited to small case series, and treatment decisions are often based on the management plans taken from pediatric studies...
2024: Advances and Technical Standards in Neurosurgery
https://read.qxmd.com/read/38592527/ependymoma-from-benign-to-highly-aggressive-diseases-a-review
#6
REVIEW
Stephanie T Jünger, Valentina Zschernack, Martina Messing-Jünger, Beate Timmermann, Torsten Pietsch
Ependymomas comprise biologically distinct tumor types with respect to age distribution, (epi)genetics, localization, and prognosis. Multimodal risk-stratification, including histopathological and molecular features, is essential in these biologically defined tumor types. Gross total resection (GTR), achieved with intraoperative monitoring and neuronavigation, and if necessary, second-look surgery, is the most effective treatment. Adjuvant radiation therapy is mandatory in high-risk tumors and in case of residual tumor...
2024: Advances and Technical Standards in Neurosurgery
https://read.qxmd.com/read/38581034/cns-tumors-with-plagl1-fusion-beyond-zfta-and-yap1-in-the-genetic-spectrum-of-supratentorial-ependymomas
#7
JOURNAL ARTICLE
Arnault Tauziède-Espariat, Yvan Nicaise, Philipp Sievers, Felix Sahm, Andreas von Deimling, Delphine Guillemot, Gaëlle Pierron, Mathilde Duchesne, Myriam Edjlali, Volodia Dangouloff-Ros, Nathalie Boddaert, Alexandre Roux, Edouard Dezamis, Lauren Hasty, Benoît Lhermitte, Edouard Hirsch, Maria Paola Valenti Hirsch, François-Daniel Ardellier, Mélodie-Anne Karnoub, Marie Csanyi, Claude-Alain Maurage, Karima Mokhtari, Franck Bielle, Valérie Rigau, Thomas Roujeau, Marine Abad, Sébastien Klein, Michèle Bernier, Catherine Horodyckid, Clovis Adam, Petter Brandal, Pitt Niehusmann, Quentin Vannod-Michel, Corentin Provost, Nicolas Menjot de Champfleur, Lucia Nichelli, Alice Métais, Cassandra Mariet, Fabrice Chrétien, Thomas Blauwblomme, Kévin Beccaria, Johan Pallud, Stéphanie Puget, Emmanuelle Uro-Coste, Pascale Varlet
A novel methylation class, "neuroepithelial tumor, with PLAGL1 fusion" (NET-PLAGL1), has recently been described, based on epigenetic features, as a supratentorial pediatric brain tumor with recurrent histopathological features suggesting an ependymal differentiation. Because of the recent identification of this neoplastic entity, few histopathological, radiological and clinical data are available. Herein, we present a detailed series of nine cases of PLAGL1-fused supratentorial tumors, reclassified from a series of supratentorial ependymomas, non-ZFTA/non-YAP1 fusion-positive and subependymomas of the young...
April 5, 2024: Acta Neuropathologica Communications
https://read.qxmd.com/read/38579345/resection-of-intradural-spinal-lesions-with-concomitant-instrumented-fusion-in-children-a-systematic-review-and-representative-cases
#8
JOURNAL ARTICLE
Kiana Y Prather, Matthew P Baier, Nangorgo J Coulibaly, Mark E Stephens, Sixia Chen, Michael E Omini, Andrew Jea
OBJECTIVE: More than one-third of pediatric patients who undergo resection of intradural spine lesions develop progressive postoperative deformity, with as many as half of these patients subsequently requiring surgical fusion. Intradural spinal procedures with simultaneous instrumented fusion in children, however, are infrequently performed. Moreover, the rationale for patient selection, outcomes, and safety of this single-stage surgery in children has not been systematically investigated...
April 5, 2024: Journal of Neurosurgery. Pediatrics
https://read.qxmd.com/read/38555652/ependymomas-of-the-spinal-region-in-adults-clinical-and-pathological-features-and-mycn-expression-levels-in-spinal-ependymomas-and-myxopapillary-ependymomas
#9
JOURNAL ARTICLE
Fikret Dirilenoğlu, Gözde Topel, İsmail Ertan Sevin, Aslı Kahraman
BACKGROUND: Ependymomas (EPNs) of the spinal region are a heterogeneous group of tumors that account for 17.6 % in adults. Four types have been recognized: subependymoma, spinal ependymoma (Sp-EPN), myxopapillary ependymoma (MPE), and Sp-EPN-MYCN amplified, each with distinct histopathological and molecular features. METHODS: This study investigated the clinical and pathological characteristics and MYCN expression levels of 35 Sp-EPN and MPE cases diagnosed at a tertiary university hospital over a decade-long period...
March 27, 2024: Annals of Diagnostic Pathology
https://read.qxmd.com/read/38554364/intracranial-ependymoma-with-extremely-rare-extraneural-metastasis
#10
JOURNAL ARTICLE
Mehtap D Calıs
Ependymomas account for 1-8% of overall brain tumors. They are most common at the age of 3-4 years. Their metastasis is very rare, and extraneural metastasis is even more unusual. In this report, the ependymoma localized in the posterior fossa with metastasis into femoral diaphysis in a 27-year-old male patient, who was treated in 2001, is presented. As we did not have any other cases of patients having a brain and spinal tumor with extraneural metastases even after 21 years, until 2022, this case was found worthy of being presented...
January 1, 2024: Journal of Cancer Research and Therapeutics
https://read.qxmd.com/read/38549412/-supratentorial-neuroepithelial-tumor-with-plagl1-gene-fusion-a-new-type-of-morphologically-variable-pediatric-brain-neoplasm-defined-by-a-distinct-dna-methylation-class-a-case-report-and-literature-review
#11
JOURNAL ARTICLE
D N Kopachev, M V Ryzhova, A N Kislyakov, E G Shaikhaev, O G Zheludkova, E V Kumirova, S V Meshcheryakov, P A Vlasov, A M Shkatova, Zh B Semenova, A O Gushcha
BACKGROUND: Methylation analysis has become a powerful diagnostic tool in modern neurooncology. This technique is valuable to diagnose new brain tumor types. OBJECTIVE: To describe the MRI and histological pattern of neuroepithelial tumor with PLAGL1 gene fusion. MATERIAL AND METHODS: We present a 6-year-old patient with small right frontal intraaxial tumor causing drug resistant epilepsy. Despite indolent preoperative clinical course and MRI features suggesting glioneuronal tumor, histological evaluation revealed characteristics of high-grade glioma, ependymoma and neuroblastoma...
2024: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://read.qxmd.com/read/38545063/nomogram-incorporating-preoperative-mri-vasari-features-for-differentiating-intracranial-extraventricular-ependymoma-from-glioblastoma
#12
JOURNAL ARTICLE
Yao Yao, Yan Fu, Gaofeng Zhou, Xueying Wang, Liyan Li, Yipu Mao, Jing Wang, Zeming Tan, Muliang Jiang, Xiaoping Yi, Bihong T Chen
BACKGROUND: Intracranial extraventricular ependymoma (IEE) and glioblastoma (GBM) may have similar imaging findings but different prognosis. This study aimed to develop and validate a nomogram based on magnetic resonance imaging (MRI) Visually AcceSAble Rembrandt Images (VASARI) features for preoperatively differentiating IEE from GBM. METHODS: The clinical data and the MRI-VASARI features of patients with confirmed IEE (n=114) and confirmed GBM (n=258) in a multicenter cohort were retrospectively analyzed...
March 15, 2024: Quantitative Imaging in Medicine and Surgery
https://read.qxmd.com/read/38540119/comprehensive-transcriptomic-profiling-of-diverse-brain-tumor-types-uncovers-complex-structures-of-the-brain-tumor-microenvironment
#13
JOURNAL ARTICLE
Jiin Choi, Hee Jin Cho
Various types of brain tumors occur in both children and adults. These tumors manifest with different characteristics such as malignancy, cellular lineage, location of origin, and genomic profile. Recently, immunotherapy, which manipulates immune cells in the tumor microenvironment (TME) to kill tumor cells, has attracted attention as a treatment strategy for tumors. Here, we analyzed the transcriptomic architecture of the brain tumor microenvironment to provide potential guidelines to overcome the therapeutic vulnerabilities to brain tumors...
February 23, 2024: Biomedicines
https://read.qxmd.com/read/38538929/molecular-insights-and-the-role-of-18f-fdg-pet-ct-in-the-diagnosis-of-spinal-gliomas
#14
JOURNAL ARTICLE
Yoshitaka Nagashima, Yusuke Nishimura, Kaoru Eguchi, Junya Yamaguchi, Shoichi Haimoto, Fumiharu Ohka, Kazuya Motomura, Takashi Abe, Mamoru Matsuo, Eisuke Tsukamoto, Masahito Hara, Ryuta Saito
BACKGROUND: In recent years, molecular findings on spinal gliomas have become increasingly important. This study aimed to investigate the role of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) in the diagnosis of spinal glioma. METHODS: This study included patients diagnosed with spinal cord glioma who underwent 18F-FDG-PET examination at the Department of Neurosurgery, Nagoya University Hospital between January 2016 and November 2023...
March 27, 2024: Acta Neurochirurgica
https://read.qxmd.com/read/38532243/registration-incidence-patterns-and-survival-trends-of-central-nervous-system-tumors-among-children-in-germany-1980-2019-an-analysis-of-40%C3%A2-years-based-on-data-from-the-german-childhood-cancer-registry
#15
JOURNAL ARTICLE
Maike Wellbrock, Mathias Voigt, Cecile Ronckers, Desiree Grabow, Claudia Spix, Friederike Erdmann
BACKGROUND: Tumors of the central nervous system (CNS) are the second most common type of pediatric cancer in Germany. We aimed to describe registration practice, incidence, and survival patterns for childhood CNS tumors in Germany for the past 40 years. PROCEDURE: Including all CNS tumor cases in children diagnosed at ages 0-14 years registered at the German Childhood Cancer Registry (GCCR) in 1980-2019 (for survival analysis 1980-2016), we calculated age-specific and age-standardized incidence rates (ASIR) over time, average annual percentage changes (AAPC), and 1- and 5-year overall survival...
March 26, 2024: Pediatric Blood & Cancer
https://read.qxmd.com/read/38529997/impact-of-molecular-subgroups-on-prognosis-and-survival-outcomes-in-posterior-fossa-ependymomas-a-retrospective-study-of-412-cases
#16
JOURNAL ARTICLE
Bo Wang, Minjun Yan, Bo Han, Xing Liu, Pinan Liu
BACKGROUND AND OBJECTIVES: Posterior fossa ependymomas (PFEs) are rare brain tumors classified as PF-EPN-A (PFA) and PF-EPN-B (PFB) subgroups. The study aimed to evaluate the prognosis and survival outcomes in PFEs, with a focus on the impact of molecular subgroups. METHODS: A retrospective study was conducted on 412 patients with PFEs. Kaplan-Meier survival analyses were conducted to evaluate the overall survival (OS) and progression-free survival. Cox regression analyses were conducted to assess the prognostic factors...
March 26, 2024: Neurosurgery
https://read.qxmd.com/read/38527429/the-intersection-of-epigenetic-alterations-and-developmental-state-in-pediatric-ependymomas
#17
REVIEW
Alisha Simone Kardian, Stephen Mack
BACKGROUND: Ependymomas are the third most common brain cancer in children and have no targeted therapies. They are divided into at least 9 major subtypes based on molecular characteristics and major drivers and have few genetic mutations compared to the adult form of this disease, leading to investigation of other mechanisms. SUMMARY: Epigenetic alterations such as transcriptional programs activated by oncofusion proteins and alterations in histone modifications play an important role in development of this disease...
March 25, 2024: Developmental Neuroscience
https://read.qxmd.com/read/38518290/exploring-disparities-in-surgical-recommendations-for-patients-with-primary-intramedullary-spinal-cord-tumors-an-analysis-of-the-surveillance-epidemiology-and-end-results-database-from-2000-to-2019
#18
JOURNAL ARTICLE
Megan Parker, Melanie A Horowitz, Sachiv Chakravarti, Jiaqi Liu, Cathleen C Kuo, Julian Gendreau, Daniel Lubelski, Jordina Rincon-Torroella, Chetan Bettegowda, Debraj Mukherjee
OBJECTIVE: Factors that may drive recommendations for operative intervention for patients with intramedullary spinal cord tumors (ISCTs) have yet to be extensively studied. The authors investigated racial and socioeconomic disparities in the management of patients with primary spinal cord ependymomas and nonependymal gliomas, with the aim of determining the associations between socioeconomic patient characteristics, survival, and recommendations for the resection of primary ISCTs. METHODS: The Surveillance, Epidemiology, and End Results registry was queried to identify all patients > 18 years of age with ISCTs diagnosed between 2000 and 2019...
March 22, 2024: Journal of Neurosurgery. Spine
https://read.qxmd.com/read/38517184/commentary-c4-to-c7-laminoplasty-for-resection-of-an-intradural-intramedullary-ependymoma-2-dimensional-operative-video
#19
JOURNAL ARTICLE
Nasser M F El-Ghandour
No abstract text is available yet for this article.
March 22, 2024: Operative Neurosurgery (Hagerstown, Md.)
https://read.qxmd.com/read/38510015/pdp-type-brain-tumor-in-association-with-multiple-endocrine-neoplasia-type-1
#20
Halldór Bjarki Einarsson, Anja Lisbeth Frederiksen, Inge Soekilde Pedersen, Marianne Schmidt Ettrup, Martin Wirenfeldt, Henning Boldt, Nina Nguyen, Marianne Skovsager Andersen, Carsten Reidies Bjarkam, Frantz Rom Poulsen
Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant syndrome caused by inactivating pathogenic variants in the tumor suppressor gene menin 1 on chromosome 11q13 (Falchetti et al., 2009). The syndrome is characterized by neoplasia in two or more endocrine glands and has a high degree of penetrance. Pathogenic germline multiple neoplasia type 1 variants primarily result in neoplasia affecting the parathyroid glands, the pancreatic islet cells, and the anterior pituitary in combination. Primary hyperparathyroidism is the most common pathological manifestation of the syndrome, followed by pancreatic neuroendocrine tumors...
March 30, 2024: Heliyon
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