Natural History and Current Treatment Options for Sega in Tuberous Sclerosis Complex

Subependymal giant cell astrocytoma (SEGA) is a benign brain tumor associated with tuberous sclerosis complex (TSC). Since there were some discrepancies in SEGA classification, in 2012 a consensus statement defined SEGA as a lesion at the caudothalamic groove with either a size of more than 1 cm in any direction or a subependymal lesion at any location that has shown serial growth on consecutive imaging regardless of size. This pointed that the documented tumor growing is an important diagnostic feature.
Source: Seminars in Pediatric Neurology - Category: Neurology Authors: Source Type: research

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In this study, we chose Cytokeratin-pan, GFAP, and LCA as three immunohistochemical indicators. Intraoperative IHC was done by Novodiax ihcDirect technology combined with Leica Bond auto-staining. Compared with the manual method recommended for the reagents (Novodiax ihcDirect), the results show that auto-staining has better stability and high reproducibility in coloration, which has broad prospects for future application. PMID: 32055271 [PubMed]
Source: International Journal of Clinical and Experimental Pathology - Category: Pathology Authors: Tags: Int J Clin Exp Pathol Source Type: research
This study intends to investigate the mechanisms of ubiqutin-specific protease 22 (USP22)/B cell-specific Moloney murine leukemia virus integration site 1 (BMI1) on the biological phenotypes of glioma stem cells (GSCs) under hypoxia.Main methodsWestern blot, Cell Counting Kit-8, colony formation and flow cytometry assays were preformed to evaluate cells biological behaviors. Luciferase assay was utilized to identify the associations among USP22, HIF-1α and BMI1.Key findingsSilencing USP22 reduced the stemness and proliferation of GSCs, and increased its apoptosis in response to hypoxia. Whilst, overexpression of BMI1...
Source: Life Sciences - Category: Biology Source Type: research
The malformations of cortical development (most FCD) were the most common pathological type for children cases in China with drug ‐resistant seizures. It was speculated further that the FCD patients with shorter duration of epilepsy before surgery seem to have a higher ratio of being seizure‐free after surgery. AbstractObjectivesThe drug ‐resistant seizures are characterized by frequent and severe onset of seizures in childhood. There is only little literature had extensively explored the types of pathological brain damage in Chinese children cases. The present study aims to investigate the histopathologic findings a...
Source: Brain and Behavior - Category: Neurology Authors: Tags: ORIGINAL RESEARCH Source Type: research
Publication date: Available online 14 February 2020Source: Pharmacological ResearchAuthor(s): Qing-Yun Chong, Ze-Hui Kok, Ngoc-Linh-Chi Bui, Xiaoqiang Xiang, Andrea Li-Ann Wong, Wei Peng Yong, Gautam Sethi, Peter E. Lobie, Lingzhi Wang, Boon-Cher GohAbstractCell cycle dysregulation, characterised by aberrant activation of cyclin dependent kinases (CDKs), is a hallmark of cancer. After years of research on the first and second generations of less selective CDK inhibitors with unfavourable clinical activity and toxicity profiles, CDK4/6 inhibitors become the first and only class of highly specific CDK inhibitors being approv...
Source: Pharmacological Research - Category: Drugs & Pharmacology Source Type: research
Publication date: Available online 14 February 2020Source: Practical Radiation OncologyAuthor(s): Christopher S. Hong, Branden J. Cord, Adam J. Kundishora, Aladine A. Elsamadicy, Jason M. Beckta, Anita Huttner, Veronica L. Chiang, Charles C. MatoukAbstractRadiation necrosis (RN) is a well described complication seen after stereotactic radiosurgery (SRS) for brain arteriovenous malformations (AVM). First-line medical therapies are not always successful in alleviating symptoms and management of some cases may require open surgical resection of the lesion. Surgery is often thought of however as a last resort given its associa...
Source: Practical Radiation Oncology - Category: Cancer & Oncology Source Type: research
In conclusion, a lipophilic bismuth nanoparticle hydrogel is an innovative, low-cost alternative for the topical treatment of cervicouterine, prostate, and colon human cancers.
Source: Anti-Cancer Drugs - Category: Cancer & Oncology Tags: Preclinical Reports Source Type: research
Conclusion Several TISC-based immunotherapeutic approaches are under development in various stages of preclinical studies. As outlined in this review article, a careful and more exhaustive genetic and metabolic understanding of TISC-associated phenotypes is critical to develop novel TISC based immunotherapies. Various components within the tumor microenvironment such as tumor cells, infiltrating immune cells, and supporting stromal cells impact the TISC metabolism. This unique metabolic profile leads to upregulation of certain enzymes and proteins such as ALDH1, CEP55, IDO COA1 etc., which can be utilized for development ...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
ska M Abstract Tuberous sclerosis complex (TSC) represents a genetic condition, in which the clinical manifestations are caused by the disinhibition of the mammalian target of rapamycin (mTOR) pathway due to mutations in the TSC1 (hamartin) or TSC2 (tuberin) genes. The deregulated mTOR activity leads to multi-site tumors, including subependymal giant cell astrocytoma (SEGA). SEGA is a brain tumor that affects around 15% of TSC patients. The aim of the study was to evaluate miR-21 expression in the serum of two groups of TSC patients: with or without SEGA tumors. We found no differences in the level of mi...
Source: Folia Neuropathologica - Category: Pathology Authors: Tags: Folia Neuropathol Source Type: research
We present a 58-year-old patient with known TSC, admitted for new neurological symptoms, diagnosed with a large heterogeneous tumor involving most of the corpus callosum. Stereotactic needle brain biopsy confirmed the diagnosis to be GBM. Five previously reported similar cases are reviewed, reflecting diversity in clinical and radiological findings and indicating that a high index of clinical suspicion must be maintained in patients with TSC.
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
Abstract INTRODUCTION: Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease, characterized by the development of benign tumors in several organs. During infancy, 6 - 20% of patients develop brain tumors called subependymal giant cell astrocytoma (SEGA). CASE REPORTS: Here we present two patients with TSC, who displayed acute intracranial tumors requiring surgery. Although both tumors shared similar histological aspects with large astrocytic cells and worrisome features, immunohistochemical and genetic analysis successfully distinguished an opposite diagnosis for the two pati...
Source: Clinical Neuropathology - Category: Pathology Authors: Tags: Clin Neuropathol Source Type: research
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