Read by QxMD icon Read


Karuna V Shekdar, Erin Simon Schwartz
Brain tumors can develop in the prenatal and neonatal time periods. Neuroimaging studies are crucial for the early detection of prenatal and neonatal brain tumors. Imaging allows for characterization of morphology, as well as the detection of hydrocephalus, local invasion, and distant spread. The imaging features of the more common neonatal brain tumors, including teratomas, choroid plexus tumors, ATRTs, and neoplasm mimics are described.
February 2017: Neuroimaging Clinics of North America
Benjamin W Fischer-Valuck, Ishita Chen, Amar J Srivastava, John M Floberg, Yuan James Rao, Allison A King, Eric T Shinohara, Stephanie M Perkins
BACKGROUND: Atypical teratoid rhabdoid tumors (ATRTs) are rare brain tumors that occur primarily in children under the age of 3 years. This report evaluates the treatment approach and survival outcomes in a large cohort of patients treated in the United States. METHODS: Using the National Cancer Database, the analysis included all ATRT patients aged 0 to 18 years who were diagnosed between 2004 and 2012 and had complete treatment data. RESULTS: Three hundred sixty-one ATRT patients were evaluated...
November 2, 2016: Cancer
Christine E Fuller
Over the past several decades, our understanding of malignant rhabdoid tumors (MRT) and the central nervous system equivalent atypical teratoid/rhabdoid tumor (ATRT) has undergone considerable refinement, particularly in terms of genetic characterization. MRT (both renal and extra-renal) and ATRT share phenotypic similarities and a common genetic signature, that being inactivating alterations of the SWI/SNF complex component SMARCB1 (or rarely SMARCA4). Unfortunately, a wide array of tumors bears significantly overlapping phenotypic characteristics to MRT/ATRT, posing a formidable diagnostic challenge...
August 31, 2016: Seminars in Diagnostic Pathology
Heather M Byers, Margaret P Adam, Amy LaCroix, Sarah E S Leary, Bonnie Cole, William B Dobyns, Heather C Mefford
Atypical teratoid rhabdoid tumors of the central nervous system are rare, highly malignant, embryonal tumors most often occurring in children under age 3 years. Most are due to a somatic change in tumor suppressor gene SMARCB1 followed by a second-hit, typically loss of heterozygosity, best detected on immunohistochemical staining. Despite the noteworthy genetic homogeneity of atypical teratoid rhabdoid tumors, relatively little is known about the oncogenic mechanisms that lead to biallelic inactivation of SMARCB1...
October 12, 2016: American Journal of Medical Genetics. Part A
Ming Tang, Roel Gw Verhaak
The molecular basis for the clinical heterogeneity observed in patients with malignant rhabdoid tumors is unknown. Recently, two reports revealed molecular inter-tumor heterogeneity in teratoid/rhabdoid tumors (ATRTs) and extra-cranial MRTs (ecMRTs) using genomic, transcriptomic and epigenomic profiling. Distinct molecular subgroups were identified and new therapeutic targets were revealed.
May 2016: Trends in Cancer
M Cristina Pacheco, Michelle Dolan, Anne Bendel
Ewing sarcoma (ES)/primitive neuroectodermal tumor (PNET) and atypical teratoid rhabdoid tumor (ATRT) are high-grade malignancies of childhood, each of which is associated with genetic abnormalities on chromosome 22. ES is typically characterized by rearrangement of the EWSR1 locus and ATRT by deletion of SMARCB1. We report a case with an unusual FISH signal pattern consistent with EWSR1 rearrangement that was shown to have loss of INI1 expression by immunohistochemistry due to deletion in the long arm of one chromosome 22...
July 28, 2016: Pediatric and Developmental Pathology
Stefaan W van Gool, Stefan Holm, Johannes Rachor, Lars Adamson, Antje Technau, Eberhard Maass, Michael C Frühwald, Paul G Schlegel, Matthias Eyrich
BACKGROUND AIMS: Atypical rhabdoid/teratoid tumors (AT/RT) are the most common brain tumors in infants and associated with a dismal prognosis. Although intensification of first-line therapy has resulted in improvement of overall survival, novel treatment strategies are needed. Because immunotherapy has resulted in remarkable results in several adult tumor entities, incorporation of immunotherapy into AT/RT treatment offers a novel alternative. METHODS: We retrospectively analyzed data from 7 AT/RT patients from five countries treated within the HGG-Immuno Consortium...
September 2016: Cytotherapy
Pascal D Johann, Volker Hovestadt, Christian Thomas, Astrid Jeibmann, Katharina Heß, Susanne Bens, Florian Oyen, Cynthia Hawkins, Christopher R Pierson, Kenneth Aldape, Sang Pyo Kim, Eva Widing, David Sumerauer, Péter Hauser, Frank van Landeghem, Marina Ryzhova, Andrey Korshunov, David Capper, David T W Jones, Stefan M Pfister, Reinhard Schneppenheim, Reiner Siebert, Werner Paulus, Michael C Frühwald, Marcel Kool, Martin Hasselblatt
Rhabdoid phenotype and loss of SMARCB1 expression in a brain tumor are characteristic features of atypical teratoid/rhabdoid tumors (ATRT). Rare non-rhabdoid brain tumors showing cribriform growth pattern and SMARCB1 loss have been designated cribriform neuroepithelial tumor (CRINET). Small case series suggest that CRINETs may have a relatively favorable prognosis. However, the long-term outcome is unclear and it remains uncertain whether CRINET represents a distinct entity or a variant of ATRT. Therefore, 10 CRINETs were clinically and molecularly characterized and compared with 10 ATRTs of each of three recently described molecular subgroups (i...
July 6, 2016: Brain Pathology
Rintaro Hashizume, Ali Zhang, Sabine Mueller, Michael D Prados, Rishi R Lulla, Stewart Goldman, Amanda M Saratsis, Andrew P Mazar, Alexander H Stegh, Shi-Yuan Cheng, Craig Horbinski, Daphne A Haas-Kogan, Jann N Sarkaria, Todd Waldman, C David James
BACKGROUND: Radiation therapy is the most commonly used postsurgical treatment for primary malignant brain tumors. Consequently, investigating the efficacy of chemotherapeutics combined with radiation for treating malignant brain tumors is of high clinical relevance. In this study, we examined the cyclin-dependent kinase 4/6 inhibitor palbociclib, when used in combination with radiation for treating human atypical teratoid rhabdoid tumor (ATRT) as well as glioblastoma (GBM). METHODS: Evaluation of treatment antitumor activity in vitro was based upon results from cell proliferation assays, clonogenicity assays, flow cytometry, and immunocytochemistry for DNA double-strand break repair...
November 2016: Neuro-oncology
Satyashiva Munjal, Uttara Chatterjee, Matthieu Vinchon, Sandip Chatterjee
INTRODUCTION: Infantile brain tumours (age < 1 year) are increasingly being diagnosed due to advances in prenatal and perinatal diagnostic imaging. We present here our retrospective study of 64 infant brain tumours that brings to the fore the epidemiology, clinical presentation, pathology and outcome of this unique subset of paediatric brain tumours presenting to two tertiary referral centres in Kolkata in India and Lille in France between the years 1999 and 2014. METHODS: Data was retrospectively collected from Kolkata (n = 30) and Lille (n = 34) for patients presenting with infant brain tumours and analysed for factors such as age at presentation, clinical features, gender, location of tumour, pathology, management and outcome...
September 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Khaled Elsayad, Jan Kriz, Laith Samhouri, Uwe Haverkamp, Ronald Straeter, Walter Stummer, Hans Theodor Eich
BACKGROUND: Atypical teratoid rhabdoid tumor (ATRT) is a highly aggressive disease of embryonic origin accounting for <5% of all pediatric central nervous system (CNS) tumors. PATIENTS AND METHODS: We describe a series of five cases of CNS ATRT. The first three patients underwent subtotal tumor resection. Gross total resection of the tumor was achieved in the fourth and fifth patients. Only 4 patients received chemotherapy, whereas all 5 patients received additive radiotherapy (RT)...
August 2016: Strahlentherapie und Onkologie: Organ der Deutschen Röntgengesellschaft ... [et Al]
Ki Woong Sung, Do Hoon Lim, Eun Sang Yi, Young Bae Choi, Ji Won Lee, Keon Hee Yoo, Hong Hoe Koo, Ji Hye Kim, Yeon-Lim Suh, Yoo Sook Joung, Hyung Jin Shin
Purpose: We prospectively evaluated the effectiveness of tandem high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT) in improving the survival of patients with atypical teratoid/rhabdoid tumors (ATRTs) while reducing the risks of late adverse effects from radiotherapy (RT). Materials and Methods: For young children (< 3 years old), tandem HDCT/auto-SCT was administered after six cycles of induction chemotherapy. RT was deferred until after 3 years of age unless the tumor showed relapse or progression...
April 1, 2016: Cancer Research and Treatment: Official Journal of Korean Cancer Association
Peter Sidaway
No abstract text is available yet for this article.
May 2016: Nature Reviews. Clinical Oncology
Yan Jiang, Lin Zhang
OBJECTIVE: To explore the mechanism of all-transretinoic acid (ATRA) increasing retinoblastoma (RB) sensitivity to vincristine, and the inhibiting effect of vincristine combined with ATRA treatment on the SO-RB50 cell proliferation. METHODS: SO-RB50 cells were cultivated by routine culture method. Different concentrations of vincristine or ATRA were added into culture solution. After 48 h, cell counting kit-8 was used to detect the median inhibitory concentration (IC50) of vincristine combined with ATRT treatment to SO-RB50 cells...
March 2016: Asian Pacific Journal of Tropical Medicine
(no author information available yet)
ATRTs are comprised of three epigenetically distinct but genetically similar subgroups.
April 2016: Cancer Discovery
Pascal D Johann, Serap Erkek, Marc Zapatka, Kornelius Kerl, Ivo Buchhalter, Volker Hovestadt, David T W Jones, Dominik Sturm, Carl Hermann, Maia Segura Wang, Andrey Korshunov, Marina Rhyzova, Susanne Gröbner, Sebastian Brabetz, Lukas Chavez, Susanne Bens, Stefan Gröschel, Fabian Kratochwil, Andrea Wittmann, Laura Sieber, Christina Geörg, Stefan Wolf, Katja Beck, Florian Oyen, David Capper, Peter van Sluis, Richard Volckmann, Jan Koster, Rogier Versteeg, Andreas von Deimling, Till Milde, Olaf Witt, Andreas E Kulozik, Martin Ebinger, Tarek Shalaby, Michael Grotzer, David Sumerauer, Josef Zamecnik, Jaume Mora, Nada Jabado, Michael D Taylor, Annie Huang, Eleonora Aronica, Anna Bertoni, Bernhard Radlwimmer, Torsten Pietsch, Ulrich Schüller, Reinhard Schneppenheim, Paul A Northcott, Jan O Korbel, Reiner Siebert, Michael C Frühwald, Peter Lichter, Roland Eils, Amar Gajjar, Martin Hasselblatt, Stefan M Pfister, Marcel Kool
Atypical teratoid/rhabdoid tumor (ATRT) is one of the most common brain tumors in infants. Although the prognosis of ATRT patients is poor, some patients respond favorably to current treatments, suggesting molecular inter-tumor heterogeneity. To investigate this further, we genetically and epigenetically analyzed 192 ATRTs. Three distinct molecular subgroups of ATRTs, associated with differences in demographics, tumor location, and type of SMARCB1 alterations, were identified. Whole-genome DNA and RNA sequencing found no recurrent mutations in addition to SMARCB1 that would explain the differences between subgroups...
March 14, 2016: Cancer Cell
Andrea Muscat, Dean Popovski, W Samantha N Jayasekara, Fernando J Rossello, Melissa Ferguson, Kieren D Marini, Muhammad Alamgeer, Elizabeth M Algar, Peter Downie, D Neil Watkins, Jason E Cain, David M Ashley
PURPOSE: Malignant rhabdoid tumor (MRT) and atypical teratoid rhabdoid tumors (ATRT) are rare aggressive undifferentiated tumors primarily affecting the kidney and CNS of infants and young children. MRT are almost exclusively characterized by homozygous deletion or inactivation of the chromatin remodeling gene SMARCB1 SMARCB1 protein loss leads to direct impairment of chromatin remodeling and we have previously reported a role for this protein in histone acetylation. This provided the rationale for investigating the therapeutic potential of histone deactylase inhibitors (HDACi) in MRT...
July 15, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Somayyeh Fahiminiya, Leora Witkowski, Javad Nadaf, Jian Carrot-Zhang, Catherine Goudie, Martin Hasselblatt, Pascal Johann, Marcel Kool, Ryan S Lee, Tenzin Gayden, Charles W M Roberts, Jaclyn A Biegel, Nada Jabado, Jacek Majewski, William D Foulkes
Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is the most common undifferentiated ovarian malignancy diagnosed in women under age 40. We and others recently determined that germline and/or somatic deleterious mutations in SMARCA4 characterize SCCOHT. Alterations in this gene, or the related SWI/SNF chromatin remodeling gene SMARCB1, have been previously reported in atypical teratoid/rhabdoid tumors (ATRTs) and malignant rhabdoid tumors (MRTs). To further describe the somatic landscape of SCCOHT, we performed whole exome sequencing on 14 tumors and their matched normal tissues and compared their genomic alterations with those in ATRT and ovarian high grade serous carcinoma (HGSC)...
January 12, 2016: Oncotarget
Mehmet Öztürk, Ahmet Siğirci, Neşe Karadağ
Atypical teratoid rhabdoid tumour (ATRT) is a rare malignant tumour of the central nervous system with embryonal roots. The majority are seen in early childhood and location is often in the posterior fossa. Surgery, radiotherapy and chemotherapy are used in treatment. Knowledge of the localisation of the mass preoperatively is necessary for direction of the chemoradiotherapy and sufficient resection in surgery. Differentiation from other brain tumours is important because of poor prognosis and differences in treatment...
2015: SpringerPlus
D Schrey, F Carceller Lechón, G Malietzis, L Moreno, C Dufour, S Chi, L Lafay-Cousin, K von Hoff, T Athanasiou, L V Marshall, S Zacharoulis
Atypical teratoid rhabdoid tumour (ATRT) is a malignant tumour of the central nervous system with a dismal prognosis. There is no consensus on optimal treatment and different multimodal strategies are currently being used in an attempt to improve outcomes. To evaluate the impact of high-dose chemotherapy followed by autologous stem-cell rescue (HD48 SCR), radiotherapy (RT) at first line, intrathecal chemotherapy (IT) and extent of surgical resection upon recurrence-free survival (RFS) and overall survival (OS)...
January 2016: Journal of Neuro-oncology
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"