Academic literature on the topic 'Rhabdoid Tumor (MRT)'

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Journal articles on the topic "Rhabdoid Tumor (MRT)"

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Duncan, Virginia E., Jason A. Wicker, David R. Kelly, and Rong Li. "TLE1 Expression in Malignant Rhabdoid Tumor and Atypical Teratoid/Rhabdoid Tumor." Pediatric and Developmental Pathology 21, no. 6 (2018): 522–27. http://dx.doi.org/10.1177/1093526618761720.

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Malignant rhabdoid tumors (MRT; atypical teratoid/rhabdoid tumor [ATRT] in the central nervous system) are aggressive tumors in infants and children which can overlap with other sarcomas, such as synovial sarcoma (SS). The gold standard for SS diagnosis is characterization of the t(X;18) chromosomal translocation. However, stratification of cases for molecular analysis is not always straightforward or feasible. Recent literature suggests transducer-like enhancer of split 1 (TLE1) protein expression may distinguish SS from certain histologic mimics; however, this has not been investigated in MR
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Fazlollahi, Ladan, Susan J. Hsiao, Manpreet Kochhar, Mahesh M. Mansukhani, Darrell J. Yamashiro, and Helen E. Remotti. "Malignant Rhabdoid Tumor, an Aggressive Tumor Often Misclassified as Small Cell Variant of Hepatoblastoma." Cancers 11, no. 12 (2019): 1992. http://dx.doi.org/10.3390/cancers11121992.

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The clinical management of pediatric liver tumors involves stratification into risk groups. One previously defined, high-risk group of hepatoblastomas is the small cell undifferentiated variant. In light of molecular studies showing SMARCB1 deletion in these tumors, it is now recognized that most small cell, undifferentiated liver tumors represent an aggressive unrelated tumor—the malignant rhabdoid tumor (MRT). SMARCB1 is a member of the chromatin remodeling SWI/SNF complex and encodes the INI1 protein. The histologic diagnosis of MRT is currently based on INI1 negative immunoreactivity and t
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Gruhle, Miriam, Karolina Nemes, Mona Steinbügl, et al. "ATRT-14. Malignant rhabdoid tumors of cranial nerves – ATRT or extracranial rhabdoid tumor?" Neuro-Oncology 24, Supplement_1 (2022): i5—i6. http://dx.doi.org/10.1093/neuonc/noac079.013.

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Abstract INTRODUCTION: Malignant rhabdoid tumors (MRT) are highly aggressive neoplasias mostly affecting young children. They are classified as rhabdoid tumors of the central nervous system (ATRT, atypical teratoid rhabdoid tumor), rhabdoid tumors of the kidney (RTK) or extracranial rhabdoid tumors arising from any soft tissue outside the central nervous system (eMRT, extracranial extrarenal MRT). We report a series of four MRTs with cranial nerve involvement. METHODS: Patients were identified from a cohort of 132 patients with MRT (2017 – 2021), as part of the European Rhabdoid Registry (EU-R
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Rahman, J., E. Wei, and K. Knowles. "Malignant Rhabdoid Tumor masquerading as Neuroblastoma: A rare Case-report." American Journal of Clinical Pathology 162, Supplement_1 (2024): S129—S130. http://dx.doi.org/10.1093/ajcp/aqae129.286.

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Abstract Introduction/Objective Malignant rhabdoid tumors (MRT) predominantly impact infants and young children, typically occurring around the age of 15 months. While they can emerge from soft tissues throughout the body, but commonly initiate in the kidneys. Neuroblastoma-like characteristics are seldom observed within the varied phenotypes of malignant rhabdoid tumors. Here, we present an exceptionally rare and distinctive subtype of malignant rhabdoid tumor exhibiting features akin to neuroblastoma localized within the unusal parapharyngeal space. Methods/Case Report A 2-month-old female p
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Guilmette, Julie, Caroline Laverdière, Denis Soulières, et al. "Malignant Rhabdoid Tumor of Soft Tissue." Pediatric and Developmental Pathology 20, no. 3 (2017): 262–66. http://dx.doi.org/10.1177/1093526617706814.

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Introduction Malignant rhabdoid tumor (MRT) is defined as a high-grade sarcoma derived from an uncertain cell of origin. Its diagnosis is associated with poor prognosis and patient’s life expectancy is greatly reduced. Material and method Here, we describe a unique case of 9-month-old boy who presented with a large MRT arising from the soft tissue of the neck. Following intensive multimodal treatment, the patient benefited from a 25 years’ remission until the discovery of multiple liver metastases. Conclusion MRT of soft tissue needs to be distinguished from other soft tissue neoplasms, as MRT
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Kenny, Colin, Elaine O’Meara, Mevlüt Ulaş, Karsten Hokamp, and Maureen J. O’Sullivan. "Global Chromatin Changes Resulting from Single-Gene Inactivation—The Role of SMARCB1 in Malignant Rhabdoid Tumor." Cancers 13, no. 11 (2021): 2561. http://dx.doi.org/10.3390/cancers13112561.

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Human cancer typically results from the stochastic accumulation of multiple oncogene-activating and tumor-suppressor gene-inactivating mutations. However, this process takes time and especially in the context of certain pediatric cancer, fewer but more ‘impactful’ mutations may in short order produce the full-blown cancer phenotype. This is well exemplified by the highly aggressive malignant rhabdoid tumor (MRT), where the only gene classically showing recurrent inactivation is SMARCB1, a subunit member of the BAF chromatin-remodeling complex. This is true of all three presentations of MRT inc
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Pawel, Bruce R. "SMARCB1-deficient Tumors of Childhood: A Practical Guide." Pediatric and Developmental Pathology 21, no. 1 (2017): 6–28. http://dx.doi.org/10.1177/1093526617749671.

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The SMARCB1 gene ( INI1, BAF47) is a member of the SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling complex, involved in the epigenetic regulation of gene transcription. SMARCB1 acts as a tumor suppressor gene, and loss of function of both alleles gives rise to SMARCB1-deficient tumors. The prototypical SMARCB1-deficient tumor is the malignant rhabdoid tumor (MRT) which was first described in the kidney but also occurs in soft tissue, viscera, and the brain (where it is referred to as atypical teratoid rhabdoid tumor or AT/RT). These are overwhelmingly tumors of the very young, an
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Attia, Adel, Moosa Suleman, and Hesham Mosleh. "Malignant Rhabdoid Tumor of the Lung in the Young Adult: A Case Report." Case Reports in Pulmonology 2011 (2011): 1–4. http://dx.doi.org/10.1155/2011/323584.

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Malignant rhabdoid tumor (MRT) is one of the most aggressive and lethal malignancies in pediatric oncology. Malignant rhabdoid tumor was initially described in 1978 as a rhabdomyosarcomatoid variant of a Wilms tumor because of its occurrence in the kidney and because of the resemblance of its cells to rhabdomyoblasts. The absence of muscular differentiation led Haas and colleagues to coin the term rhabdoid tumor of the kidney in 1981, Haas et al..
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Shirai, Ryota, Tomoo Osumi, Keita Terashima, et al. "ATRT-11. PREVALENCE OF GERMLINE VARIANTS IN SMARCB1 INCLUDING SOMATIC MOSAICISM IN AT/RT AND OTHER RHABDOID TUMORS." Neuro-Oncology 22, Supplement_3 (2020): iii277—iii278. http://dx.doi.org/10.1093/neuonc/noaa222.011.

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Abstract BACKGROUND Genetic hallmark of atypical teratoid/rhabdoid tumor (AT/RT) is loss-of-function variants or deletions in SMARCB1 gene on 22q11.2 chromosome, which is common to extracranial malignant rhabdoid tumors (MRT). Previous studies demonstrated that approximately one-thirds of AT/RT and extracranial MRT patients harbored germline SMARCB1 variants as the rhabdoid tumor predisposing syndrome. We studied herein intensive analysis of the SMARCB1 gene in AT/RT and extracranial MRT patients focusing on prevalence of germline genetic variants. PROCEDURE: In total, 16 patients were include
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Shatara, Margaret, Ajay Gupta, Mohamed H. Abu Arja, et al. "ATRT-21. RHABDOID PREDISPOSITION SYNDROME: REPORT OF MOLECULAR PROFILES AND TREATMENT APPROACH IN THREE CHILDREN WITH SYNCHRONOUS ATYPICAL TERATOID/RHABDOID TUMOR AND MALIGNANT RHABDOID TUMOR." Neuro-Oncology 22, Supplement_3 (2020): iii280. http://dx.doi.org/10.1093/neuonc/noaa222.020.

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Abstract BACKGROUND Rhabdoid predisposition syndrome is characterized by germline alterations in SMARCB1 or SMARCA4, leading to synchronous or metachronous central nervous system (CNS) and extra-CNS rhabdoid tumors. Rare survivors have been reported to date. METHODS We describe the molecular profiling and treatment regimen of three patients with synchronous atypical teratoid/rhabdoid tumor (ATRT) and malignant rhabdoid tumor of the kidney (MRT-K). All patients underwent radical nephrectomy of the kidney, and gross total resection of the primary CNS tumor was achieved for two patients. An inten
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Books on the topic "Rhabdoid Tumor (MRT)"

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Wirtz, Ralph. Interaktion der Wnt-Signalkomponente [beta] - Catenin mit dem SWI/SNF Komplex und deren Bedeutung im Krebsgeschehen rhabdoider Tumore. 2001.

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Book chapters on the topic "Rhabdoid Tumor (MRT)"

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Warmuth-Metz, Monika, and Michael Frühwald. "Pediatric Atypical Teratoid/Rhabdoid Tumors: Imaging with CT and MRI." In Pediatric Cancer. Springer Netherlands, 2012. http://dx.doi.org/10.1007/978-94-007-2957-5_4.

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Bisogno, Gianni, and Hans Merks. "Soft-Tissue Sarcomas." In Oxford Textbook of Cancer in Children. Oxford University Press, 2020. http://dx.doi.org/10.1093/med/9780198797210.003.0025.

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This chapter discusses soft-tissue sarcomas (STS) in children and adolescents, which comprise approximately 8% of all paediatric malignancies. It highlights the clinical, pathological, and biological characteristics of this heterogeneous group of tumors derived from mesenchymal cells, and it describes the current management and the treatment results. The first part of the chapter is dedicated to the most frequent paediatric STS, rhabdomyosarcomas (RMS), while the second part covers the diagnosis and treatment strategies for non-rhabdomyosarcoma STS (NRSTS) including synovial sarcomas (SS), adu
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Conference papers on the topic "Rhabdoid Tumor (MRT)"

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Brugués, M., A. Walter, A. Müller, B. Faridi, B. Strizek, and A. Geipel. "Fetaler Hydrozephalus mit postnataler Diagnose eines atypischen teratoiden, rhabdoiden infratentoriellen Tumors." In 47. Dreiländertreffen der ÖGUM/DEGUM/SGUM. Georg Thieme Verlag KG, 2024. http://dx.doi.org/10.1055/s-0044-1789072.

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Ramli, Ras A., Martina A. Finetti, Matthew P. Selby, et al. "Abstract 4875: Identification of the cellular origin and "stemness" phenotype of Malignant Rhabdoid Tumors (MRT) may represent a new therapeutic approach in paediatric oncology." In Proceedings: AACR Annual Meeting 2017; April 1-5, 2017; Washington, DC. American Association for Cancer Research, 2017. http://dx.doi.org/10.1158/1538-7445.am2017-4875.

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