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malignant rhabdoid tumor

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https://www.readbyqxmd.com/read/29750397/fdg-pet-ct-in-the-evaluation-of-primary-and-secondary-pancreatic-malignancies
#1
Casey E Bohl, Sara M Federico, Giles W Robinson, Armita Bahrami, Barry L Shulkin
PURPOSE: Primary pancreatic carcinoma and pancreatic metastases are rare in the pediatric population. Pancreatoblastoma is the most common pancreatic malignant tumor in young children and solid-pseudopapillary tumor in teenagers. Pancreatic adenocarcinoma is extremely rare under the age of 40 and is usually associated with underlying genetic abnormalities. Secondary malignancies of the pancreas occur more frequently than primary pancreatic malignancies in children and are most commonly seen with non-Hodgkin lymphomas (NHL) and mesenchymal sarcomas...
May 11, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29742885/malignant-brain-tumours-in-children-present-and-future-perspectives
#2
REVIEW
James T Rutka
In contrast to many of the malignant tumors that occur in the central nervous system in adults, the management, responses to therapy, and future perspectives of children with malignant lesions of the brain hold considerable promise. Within the past 5 years, remarkable progress has been made with our understanding of the basic biology of the molecular genetics of several pediatric malignant brain tumors including medulloblastoma, ependymoma, atypical teratoid rhabdoid tumour, and high grade glioma/diffuse intrinsic pontine glioma...
May 2018: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/29730815/pd-1-pd-l1-and-immune-related-gene-expression-pattern-in-pediatric-malignant-brain-tumors-clinical-correlation-with-survival-data-in-korean-population
#3
Kihwan Hwang, Eun Jung Koh, Eun Jin Choi, Tae Hee Kang, Jung Ho Han, Gheeyoung Choe, Sung-Hye Park, Jennifer Holmes Yearley, Lakshmanan Annamalai, Wendy Blumenschein, Manjiri Sathe, Terri McClanahan, Hun Jung, Kyu-Chang Wang, Seung-Ki Kim, Chae-Yong Kim
BACKGROUND: PD-L1 expression has been evaluated as a predictive biomarker for immunotherapy in numerous tumor types. However, very limited data are available in pediatric brain tumors. The aim of this study was to characterize PD-1 and PD-L1 expressions of four pediatric malignant brain tumors and gene expression profile. METHODS: This study included 89 pediatric patients receiving standard treatment at Seoul National University Children's Hospital and Seoul National University Bundang Hospital between 1990 and 2014: atypical teratoid/rhabdoid tumor (AT/RT) 20; ependymoma (EPN) 20; high grade glioma (HGG) 21; and medulloblastoma (MBL) 28...
May 5, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29700418/smarca4-deficient-undifferentiated-uterine-sarcoma-malignant-rhabdoid-tumor-of-the-uterus-a-clinicopathologic-entity-distinct-from-undifferentiated-carcinoma
#4
David L Kolin, Fei Dong, Michele Baltay, Neal Lindeman, Laura MacConaill, Marisa R Nucci, Christopher P Crum, Brooke E Howitt
Small cell carcinoma of the ovary, hypercalcemic type is a rare, aggressive malignancy which usually occurs in young women and is characterized by mutations in SMARCA4, with few other alterations. We recently encountered uterine tumors with morphologic, immunohistochemical, and genetic similarities to small cell carcinoma of the ovary, hypercalcemic type. Herein we report the clinicopathologic and molecular features (using a targeted massively parallel sequencing [MPS] assay) of these tumors. The cases were diagnosed on cervical and endometrial biopsies (n = 2, 34, and 29 years) or hysterectomy and bilateral salpingo-oophorectomy (n = 3, 25, 33, and 58 years)...
April 26, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29696793/kbg-syndrome-patient-due-to-16q24-3-microdeletion-presenting-with-a-paratesticular-rhabdoid-tumor-coincidence-or-cancer-predisposition
#5
Astrid Behnert, Bernd Auber, Doris Steinemann, Michael C Frühwald, Carolin Huisinga, Kais Hussein, Christian Kratz, Tim Ripperger
KBG syndrome is a rare autosomal dominant disorder caused by constitutive haploinsufficiency of the ankyrin repeat domain-containing protein 11 (ANKRD11) being the result of either loss-of-function gene variants or 16q24.3 microdeletions. The syndrome is characterized by a variable clinical phenotype comprising a distinct facial gestalt and variable neurological involvement. ANKRD11 is frequently affected by loss of heterozygosity in cancer. It influences the ligand-dependent transcriptional activation of nuclear receptors and tumor suppressive function of tumor protein TP53...
April 25, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29693073/extracranial-congenital-malignant-rhabdoid-tumor-in-infant-with-disseminated-disease-an-uncommon-entity-and-diagnostic-challenge
#6
Emily A Dunn, Bruno P Soares, Monica S Pearl, Richard Redett, Caitlin J Alexander, Katherine B Puttgen
No abstract text is available yet for this article.
May 2018: JAAD Case Reports
https://www.readbyqxmd.com/read/29684361/high-frequency-of-ovarian-cyst-development-in-vhl-2b-snf5-mice
#7
Yasumichi Kuwahara, Leslie M Kennedy, Anthony N Karnezis, E Lorena Mora-Blanco, Arlin B Rogers, Christopher D Fletcher, David G Huntsman, Charles W M Roberts, W Kimryn Rathmell, Bernard E Weissman
The new paradigm of mutations in chromatin modifying genes as driver events in the development of cancers has proved challenging to resolve the complex influences over disease phenotypes. In particular, impaired activities of members of the SWI/SNF chromatin remodeling complex have appeared in an increasing variety of tumors. Mutations in SNF5, a member of this ubiquitously expressed complex, arise in almost all cases of malignant rhabdoid tumor in the absence of additional genetic alterations. Therefore, we studied how activation of additional oncogenic pathways might shift the phenotype of disease driven by SNF5 loss...
April 20, 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29684225/primary-intraocular-malignant-rhabdoid-tumor-without-extrascleral-compromise
#8
Eva Ayala Barroso, Alejandra Tapia Bahamondes, Juan Carlos Sánchez España, Llúcia Alós, Ramón Medel Jiménez
Primary intraocular malignant rhabdoid tumor is classified as a malignant extrarenal rhabdoid tumor. It is extremely rare, highly aggressive, and, so far, only one case (in a newborn) has been described in the medical literature. The authors report a second case of primary intraocular malignant rhabdoid tumor, this time without extrascleral involvement and in a teenager, and describe its histological, immunohistochemical, and radiological characteristics along with clinical correlations. [J Pediatr Ophthalmol...
April 23, 2018: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/29661713/review-with-novel-markers-facilitates-precise-categorization-of-41-cases-of-diagnostically-challenging-undifferentiated-small-round-cell-tumors-a-clinicopathologic-immunophenotypic-and-molecular-analysis
#9
Isidro Machado, Akihiko Yoshida, María Gema Nieto Morales, Lucas Faria Abrahão-Machado, Samuel Navarro, Julia Cruz, Javier Lavernia, Antonina Parafioriti, Piero Picci, Antonio Llombart-Bosch
BACKGROUND: Despite extensive immunohistochemical (IHC) and molecular studies combined with morphologic findings, a group of round/ovoid cell tumors histologically similar to Ewing sarcomas (ES) but lacking EWSR1-rearrangements may remain unclassifiable. DESIGN: We retrospectively analyzed 41 Ewing-like tumors (formalin-fixed, paraffin-embedded) previously determined as negative or non-informative for EWSR1-rearrangements by FISH and/or RT-PCR. A new histopathology revision and additional IHC and molecular analyses were carried out in order to investigate whether additional IHC and/or molecular testing in combination with the morphological findings may help in reaching a definitive diagnosis...
November 29, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/29661121/urothelial-carcinomas-of-the-urinary-bladder-with-plasmacytoid-or-rhabdoid-features-and-tendency-of-epithelial-mesenchymal-transition-in-3-dogs
#10
Susanne Je-Han Lin, Chi-Fei Kao, Fun-In Wang, Chian-Ren Jeng, Jih-Jong Lee, Lei-Ya Wang, Hui-Wen Chang, Yi-Jiun Chen, Chen-Hsuan Liu, Victor Fei Pang
Plasmacytoid and rhabdoid variants of urothelial carcinomas (UCs) of the urinary bladder have been described in humans with plasma cell-like or rhabdoid cellular appearance and aggressive clinical outcome. Canine UC of the bladder is generally classified as papillary/nonpapillary and infiltrating/noninfiltrating with limited information regarding other histological patterns. We report 3 cases of UC of the urinary bladder showing a unique discohesive cellular morphology with malignant behavior resembling the human plasmacytoid and rhabdoid variants of UC, which may raise some difficulties in diagnosis...
January 1, 2018: Veterinary Pathology
https://www.readbyqxmd.com/read/29625594/the-first-case-of-smarcb1-ini1-deficient-squamous-cell-carcinoma-of-the-pleura-a-case-report
#11
Kazushi Yoshida, Yutaka Fujiwara, Yasushi Goto, Takashi Kohno, Akihiko Yoshida, Koji Tsuta, Yuichiro Ohe
BACKGROUND: SMARCB1 (INI1) is a tumor-suppressor gene located at 22q11.2. Loss of SMARCB1 protein expression has been reported to be associated with atypical teratoid/rhabdoid tumors and malignant rhabdoid tumors of the kidney and extrarenal tissues. To date, however, SMARCB1-deficient carcinoma of the pleura has not been reported. We report the first case of SMARCB1- deficient squamous cell carcinoma of the pleura. CASE PRESENTATION: The case was a 33-year-old female...
April 7, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29572501/array-based-dna-methylation-profiling-in-sarcomas-with-small-blue-round-cell-histology-provides-valuable-diagnostic-information
#12
Christian Koelsche, Wolfgang Hartmann, Daniel Schrimpf, Damian Stichel, Susanne Jabar, Andreas Ranft, David E Reuss, Felix Sahm, David T W Jones, Melanie Bewerunge-Hudler, Marcel Trautmann, Thomas Klingebiel, Christian Vokuhl, Manfred Gessler, Eva Wardelmann, Iver Petersen, Daniel Baumhoer, Uta Flucke, Cristina Antonescu, Manel Esteller, Stefan Fröhling, Marcel Kool, Stefan M Pfister, Gunhild Mechtersheimer, Uta Dirksen, Andreas von Deimling
Undifferentiated solid tumors with small blue round cell histology and expression of CD99 mostly resemble Ewing sarcoma. However, they also may include other tumors such as mesenchymal chondrosarcoma, synovial sarcoma, or small cell osteosarcoma. Definitive classification usually requires detection of entity-specific mutations. While this approach identifies the majority of Ewing sarcomas, a subset of lesions remains unclassified and, therefore, has been termed "Ewing-like sarcomas" or small blue round cell tumors not otherwise specified...
March 23, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29541486/primary-intracerebral-ini1-deficient-rhabdoid-tumor-with-cd34-immunopositivity-in-a-young-adult
#13
Istvan Bodi, Anastasios Giamouriadis, Naomi Sibtain, Ross Laxton, Andrew King, Francesco Vergani
Background: Primary CNS malignant rhabdoid tumors are very rare in adults and much less is known about their biological behavior than in children. Recently, two adult cases of SMARCB1 (also known as INI1)-deficient tumor with rhabdoid cells have been described, suggesting an emerging group of primary meningeal SMARCB1-deficient tumors. We have recently encountered a case of INI1-deficient tumor with similar histology and immunophenotype to the above cases, but with a superficial cerebral, yet apparent intra-axial origin...
2018: Surgical Neurology International
https://www.readbyqxmd.com/read/29538709/in-vitro-use-of-5-ala-for-photodynamic-therapy-in-pediatric-brain-tumors
#14
Michael Schwake, Andrei Nemes, Jana Dondrop, Juliane Schroeteler, Stephanie Schipmann, Volker Senner, Walter Stummer, Christian Ewelt
BACKGROUND: Light irradiation (635 nm) of cells containing protoporphyrin IX (PPIX) after 5- aminolevulinic acid (5-ALA) pretreatment causes cell death via different pathways including apoptosis and necrosis, as previously demonstrated for malignant glioma cells. OBJECTIVE: To elucidate whether various malignant pediatric brain tumors, which have been shown to accumulate PPIX, would also be susceptible to photodynamic therapy (PDT). METHODS: Medulloblastoma (DAOY, UW228), pNET (PFSK-1), and rhabdoid tumor (BT16) cell lines were incubated with 5-ALA in variable concentrations for 4 h...
March 12, 2018: Neurosurgery
https://www.readbyqxmd.com/read/29528832/embryonal-tumors-of-the-central-nervous-system-from-the-radiologic-pathology-archives
#15
Robert Y Shih, Kelly K Koeller
Embryonal tumors of the central nervous system (CNS) are highly malignant undifferentiated or poorly differentiated tumors of neuroepithelial origin and have been defined as a category in the World Health Organization (WHO) classification since the first edition of the "Blue Book" in 1979. This category has evolved over time to reflect our ever-improving understanding of tumor biology and behavior. With the most recent update in 2016, many previous histologic diagnoses incorporate molecular parameters for the first time (genetically defined entities)...
March 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29528755/emerging-therapeutic-targets-for-the-treatment-of-malignant-rhabdoid-tumors
#16
Karolina Nemes, Michael C Frühwald
Malignant Rhabdoid Tumor (MRT) is a rare and highly aggressive malignancy primarily affecting infants and young children. The most common anatomic locations are the central nervous system (AT/RT), the kidneys (RTK) and other soft tissues (eMRT). The genetic origin of this disease is linked to mutations in SMARCB1, a gene encoding a core subunit of the SWI/SNF chromatin-remodeling complex. Areas covered: Conventional multimodal treatment may offer a significant survival benefit to certain patients. It remains to be determined, however, which patients will prove resistant to chemotherapy and need novel therapeutic approaches...
April 2018: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/29512865/malignant-progression-of-a-peripheral-nerve-sheath-tumor-in-the-setting-of-rhabdoid-tumor-predisposition-syndrome
#17
Santhosh A Upadhyaya, Rose B McGee, Breelyn A Wilky, Alberto Broniscer
Malignant progression of a benign or low-grade tumor in individuals with germline alteration of SMARCB1 gene is not well characterized. In a family in which two carrier children had germline SMARCB1 mutations and atypical teratoid rhabdoid tumor, we report malignant progression of a nerve sheath tumor over a 7-year period in an affected adult family member. Prompt identification of the germline SMARCB1 alteration and the resultant rhabdoid tumor predisposition syndrome can help guide genetic counseling and surveillance in affected family members...
March 7, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29490565/tle1-expression-in-malignant-rhabdoid-tumor-and-atypical-teratoid-rhabdoid-tumor
#18
Virginia E Duncan, Jason A Wicker, David R Kelly, Rong Li
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 MRT and ATRT...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29487714/ribavirin-as-a-potential-therapeutic-for-atypical-teratoid-rhabdoid-tumors
#19
Joshua Casaos, Sakibul Huq, Tarik Lott, Raphael Felder, John Choi, Noah Gorelick, Michael Peters, Yuanxuan Xia, Russell Maxwell, Tianna Zhao, Chenchen Ji, Thomas Simon, Julie Sesen, Sarah J Scotland, Richard E Kast, Jeffrey Rubens, Eric Raabe, Charles G Eberhart, Eric M Jackson, Henry Brem, Betty Tyler, Nicolas Skuli
Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive, malignant tumors and are the most common malignant brain tumor in children under 6 months of age. Currently, there is no standard treatment for AT/RT. Recent studies have reported potential anti-tumoral properties of ribavirin, a guanosine analog and anti-viral molecule approved by the Food and Drug Administration for treatment of hepatitis C. We previously demonstrated that ribavirin inhibited glioma cell growth in vitro and in vivo . Based on these results and the fact that no pre-clinical model of ribavirin in AT/RT exists, we decided to investigate the effect of ribavirin on several human AT/RT cell lines (BT12, BT16, and BT37) both in vitro and in vivo ...
January 30, 2018: Oncotarget
https://www.readbyqxmd.com/read/29485367/orbital-malignant-meningioma-a-unique-presentation-of-a-rare-entity
#20
Dane H Slentz, Sunil Bellur, M Reza Taheri, M Isabel Almira-Suarez, Jonathan H Sherman, Tamer N Mansour
An elderly female with progressive proptosis was found to have an aggressive retrobulbar solid orbital mass. The mass was distinct from the optic nerve sheath and intracranial meninges, and produced concave erosion of the sphenoid wing. Operative findings demonstrated an orbital mass adherent to the dura of the superior orbital fissure. The mass did not demonstrate meningeal violation, infiltrate the superior orbital fissure, or display intracranial spread. The dura remained intact after gross total resection...
February 27, 2018: Orbit
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