keyword
MENU ▼
Read by QxMD icon Read
search

malignant rhabdoid tumor

keyword
https://www.readbyqxmd.com/read/29141919/rare-tumors-in-kids-may-respond-to-tazemetostat
#1
(no author information available yet)
In a phase I trial of the EZH2 inhibitor tazemetostat, children with INI1-deficient tumors-including relapsed or refractory malignant rhabdoid tumors, atypical teratoid rhabdoid tumors, epithelioid sarcomas, and poorly differentiated chordomas-responded well to treatment, with some experiencing durable responses.
November 15, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/29141209/high-throughput-drug-screening-identifies-pazopanib-and-clofilium-tosylate-as-promising-treatments-for-malignant-rhabdoid-tumors
#2
Céline Chauvin, Amaury Leruste, Arnault Tauziede-Espariat, Mamy Andrianteranagna, Didier Surdez, Aurianne Lescure, Zhi-Yan Han, Elodie Anthony, Wilfrid Richer, Sylvain Baulande, Mylène Bohec, Sakina Zaidi, Marie-Ming Aynaud, Laetitia Maillot, Julien Masliah-Planchon, Stefano Cairo, Sergio Roman-Roman, Olivier Delattre, Elaine Del Nery, Franck Bourdeaut
Rhabdoid tumors (RTs) are aggressive tumors of early childhood characterized by SMARCB1 inactivation. Their poor prognosis highlights an urgent need to develop new therapies. Here, we performed a high-throughput screening of approved drugs and identified broad inhibitors of tyrosine kinase receptors (RTKs), including pazopanib, and the potassium channel inhibitor clofilium tosylate (CfT), as SMARCB1-dependent candidates. Pazopanib targets were identified as PDGFRα/β and FGFR2, which were the most highly expressed RTKs in a set of primary tumors...
November 14, 2017: Cell Reports
https://www.readbyqxmd.com/read/29131102/a-case-of-a-4-year-old-female-with-a-primary-spinal-malignancy-presenting-with-froin-s-syndrome
#3
Andrew T Hale, Gregory P Fricker, Travis W Crook
We report the case of a 4-year-old female with a primary extradural intramedullary atypical teratoid/rhabdoid tumor (AT/RT) leading to a middle cerebral artery (MCA) infarct and Froin's syndrome. She presented with a 6-pound weight loss over the previous week, as well as a decreased urinary output and an altered mental status. She underwent a brain MRI that revealed a left MCA infarct, mild ventriculomegaly, and bilateral internal carotid artery, M1, and A1 stenosis. An external ventricular drain (EVD) was placed due to increased intracranial pressure...
November 2, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28966010/rhabdoid-tumor-of-the-liver-report-of-6-pediatric-cases-treated-at-a-single-institute
#4
Marianna Cornet, Guénolée De Lambert, Danièle Pariente, Julien Masliah Planchon, Catherine Guettier, Hélène Martelli, Florent Guérin, Sophie Branchereau
BACKGROUND: Rhabdoid tumors (RTs) of the liver are rare, aggressive and nonsecreting malignancies occurring mainly during the first year of life. Definition of RT relies on characteristic morphology and on the inactivation of the SMARCB1 tumor suppressor gene. The aim of this study was to analyze clinical data, treatments and outcomes in our patients. PATIENTS AND METHODS: 6 cases of patients treated in our institution for RT of the liver between January 2007 and January 2015 are reported...
September 14, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28945250/smarcb1-is-required-for-widespread-baf-complex-mediated-activation-of-enhancers-and-bivalent-promoters
#5
Robert T Nakayama, John L Pulice, Alfredo M Valencia, Matthew J McBride, Zachary M McKenzie, Mark A Gillespie, Wai Lim Ku, Mingxiang Teng, Kairong Cui, Robert T Williams, Seth H Cassel, He Qing, Christian J Widmer, George D Demetri, Rafael A Irizarry, Keji Zhao, Jeffrey A Ranish, Cigall Kadoch
Perturbations to mammalian SWI/SNF (mSWI/SNF or BAF) complexes contribute to more than 20% of human cancers, with driving roles first identified in malignant rhabdoid tumor, an aggressive pediatric cancer characterized by biallelic inactivation of the core BAF complex subunit SMARCB1 (BAF47). However, the mechanism by which this alteration contributes to tumorigenesis remains poorly understood. We find that BAF47 loss destabilizes BAF complexes on chromatin, absent significant changes in complex assembly or integrity...
November 2017: Nature Genetics
https://www.readbyqxmd.com/read/28877071/uncommon-localization-of-extrarenal-xp11-2-translocation-associated-renal-cell-carcinoma-rcc-case-report
#6
Jaudah Ahmed Al-Maghrabi, Mohamad Nidal Khabaz
The World Health Organization has recognized Xp11.2 translocation-associated renal cell carcinoma (RCC) as a distinct neoplasm that arises within the kidney. Although many reports of extrarenal carcinoma may be found in the literature, to the best of our knowledge, Xp11 translocation-associated RCC with intact kidneys has not been documented. This report describes a multilobulated right retroperitoneal soft tissue mass (7.9×5.3×12.6 cm) of a 37-year-old man complaining of abdominal pain in the right side...
September 4, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28843054/high-dose-treatment-for-malignant-rhabdoid-tumor-of-the-kidney-no-evidence-for-improved-survival-the-gesellschaft-f%C3%A3-r-p%C3%A3-diatrische-onkologie-und-h%C3%A3-matologie-gpoh-experience
#7
Rhoikos Furtwängler, Leo Kager, Patrick Melchior, Christian Rübe, Martin Ebinger, Nasenien Nourkami-Tutdibi, Felix Niggli, Steven Warmann, Jochen Hubertus, Gabriele Amman, Ivo Leuschner, Christian Vokuhl, Norbert Graf, Michael C Frühwald
BACKGROUND: Malignant rhabdoid tumor of the kidney (MRTK) is the most aggressive childhood renal tumor with overall survival (OS) rates ranging from 22% to 42%. Whether high-dose chemotherapy with autologous stem-cell transplantation (HDSCT) in an intensive first-line treatment offers additional benefit is an ongoing discussion. METHODS: A retrospective analysis of all 58 patients with MRTK from Austria, Switzerland, and Germany treated in the framework of consecutive, prospective renal/rhabdoid tumor studies SIOP9/GPO, SIOP93-01/GPOH (where SIOP is International Society of Pediatric Oncology and GPOH is German Society of Pediatric Oncology and Hematology), SIOP2001/GPOH, and European Rhabdoid Tumor Registry from 1991 to 2014...
August 26, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28820306/prognostic-value-of-estrogen-receptor-in-who-grade-iii-meningioma-a-long-term-follow-up-study-from-a-single-institution
#8
Lingyang Hua, Hongda Zhu, Jingrun Li, Hailiang Tang, Dapeng Kuang, Yin Wang, Feng Tang, Xiancheng Chen, Liangfu Zhou, Qing Xie, Ye Gong
OBJECTIVE Malignant meningioma is rare and classified as Grade III in the WHO classification of CNS tumors. However, the presence of estrogen receptor (ER) in WHO Grade III meningiomas and its correlation with patients' outcomes are still unclear. In this single-center cohort study, the authors analyzed clinical features, treatment, and prognosis of these malignant tumors in patients with long-term follow-up. METHODS A total of 87 patients who were pathologically diagnosed with WHO Grade III meningiomas between 2003 and 2008 were enrolled in this study and followed for at least 7 years...
August 18, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28815340/intradural-lumbosacral-malignant-extrarenal-rhabdoid-tumor-a-case-report
#9
Richard Justin Garling, Rasanjeet Singh, Carolyn Harris, Abilash Haridas
BACKGROUND: Rhabdoid tumors are highly malignant tumors predominantly affecting the pediatric population. When these tumors occur outside of the kidneys, they are referred to as malignant extrarenal rhabdoid tumors (MERT), a rare highly aggressive subtype. Less commonly, these tumors involve the neuro-axis. OBJECTIVE: Here we present a case of a 15-year-old girl with intradural MERT of the lumbosacral spine who presented with back pain, sudden worsening of lower extremity strength, and complete loss of bowel and bladder control...
August 16, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28797981/rhabdoid-meningioma-arising-concurrent-in-pulmonary-and-intracranial-with-a-rare-malignant-clinical-progression-case-report-and-literature-review
#10
Peng Zhao, Ning Li, Jinfeng Cao, Xiangtao Lin, Changhu Liang
BACKGROUND: Rhabdoid meningioma (RM) is an unusual variant of meningioma, classified as World Health Organization grade III. Although its recurrence is common, extracranial metastasis is rare and usually misdiagnosed. The transfer mechanism and pathway are ambiguous; once the metastasis occurs, the prognosis is poor, and there is no effective management. The present case is the first report of concurrent intracranial and pulmonary RM with rapid and widespread metastasis. We hope this report can serve as a helpful reference for clinicians and radiologists...
November 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28783147/marrow-ablative-chemotherapy-followed-by-tandem-autologous-hematopoietic-cell-transplantation-in-pediatric-patients-with-malignant-brain-tumors
#11
J A Guerra, G Dhall, A Marachelian, E Castillo, J Malvar, K Wong, R Sposto, J L Finlay
To improve survival in young children with malignant brain tumors, irradiation-avoiding or -minimizing marrow-ablative chemotherapy (HDCx) with autologous hematopoietic cell transplantation (AuHCT) has been investigated. We evaluated the outcome of 44 children with malignant brain tumors treated with HDCx and tandem AuHCT at Children's Hospital Los Angeles between June 1999 and July 2012. Forty-four children with malignant brain tumors were studied. Twenty-one had medulloblastoma/primitive neuro-ectodermal tumor, eight atypical teratoid/rhabdoid tumor (ATRT), five high-grade glioma, four malignant germ cell tumor, three ependymoma and three choroid plexus carcinoma...
November 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28744687/lin28a-a-sensitive-immunohistochemical-marker-for-embryonal-tumor-with-multilayered-rosettes-etmr-is-also-positive-in-a-subset-of-atypical-teratoid-rhabdoid-tumor-at-rt
#12
Shilpa Rao, R T Rajeswarie, T Chickabasaviah Yasha, Bevinahalli N Nandeesh, Arimappamagan Arivazhagan, Vani Santosh
INTRODUCTION: CNS embryonal tumors comprise a group of highly malignant neoplasms with a wide spectrum of histomorphological entities that includes Medulloblastoma (MB), Atypical Teratoid/Rhabdoid Tumor (AT/RT), Neuroblastoma (NB), Ganglioneuroblastoma (GNB), Embryonal Tumor with Multilayered Rosettes (ETMR), and the embryonal tumor-Not Otherwise Specified (NOS). The entity ETMR includes previously described histopathologic patterns-Embryonal Tumor with Abundant Neuropil and True Rosettes (ETANTR), Ependymoblastoma (EBL), and Medulloepithelioma (MEPL)...
November 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28731921/loss-of-cdkn1c-in-a-recurrent-atypical-teratoid-rhabdoid-tumor
#13
Dustin Tran, Sandra Camelo-Piragua, Avneesh Gupta, Kate Gowans, Patricia L Robertson, Rajen Mody, Carl Koschmann
Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant tumor that is commonly associated with biallelic alterations of SMARCB1. Recurrent or refractory AT/RT has not been molecularly characterized as well. We present the case of a child with recurrent AT/RT who underwent clinically integrated molecular profiling (germline DNA and tumor DNA/RNA sequencing). This demonstrated a somatic lesion in CDKN1C alongside hallmark loss of SMARCB1. This data allowed us to explore potential personalized therapies for this patient and expose a molecular driver that may be involved in similar cases...
November 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28731919/a-case-presentation-rare-occurrence-of-an-adolescent-male-presenting-with-an-atrt-and-simultaneous-low-grade-glioneuronal-tumor
#14
Matthew Cascio, Marie Rivera-Zengotita, John Fort
Atypical rhabdoid/teratoid tumor (ATRT) is an uncommon and highly malignant tumor of the central nervous system. The majority of ATRT tumors occur in infancy and young children located in the posterior fossa. The ideal treatment for cure remains controversial and prognosis is typically unfavorable. We present a case of an atypical presentation of ATRT, presenting in adolescence with an additional low-grade glioneuronal tumor discovered at diagnosis.
November 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28714904/brd9-inhibition-alone-or-in-combination-with-cytostatic-compounds-as-a-therapeutic-approach-in-rhabdoid-tumors
#15
Katja F Krämer, Natalia Moreno, Michael C Frühwald, Kornelius Kerl
Rhabdoid tumors (RT) are malignant neoplasms of early childhood. Despite intensive therapy, survival is poor and new treatment approaches are required. The only recurrent mutations in these tumors affect SMARCB1 and less commonly SMARCA4, both subunits of the chromatin remodeling complex SWItch/Sucrose Non-Fermentable (SWI/SNF). Loss of these two core subunits alters the function of the SWI/SNF complex, resulting in tumor development. We hypothesized that inhibition of aberrant SWI/SNF function by selective blockade of the BRD9 subunit of the SWI/SNF complex would reduce tumor cell proliferation...
July 16, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28683521/expression-of-cd34-and-%C3%AE-catenin-in-malignant-rhabdoid-tumor-of-the-liver-mimicking-proximal-type-epithelioid-sarcoma
#16
Woo Cheal Cho, Fabiola Balarezo
No abstract text is available yet for this article.
July 7, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28677107/a-french-retrospective-study-on-clinical-outcome-in-102-choroid-plexus-tumors-in-children
#17
A Siegfried, S Morin, C Munzer, M B Delisle, M Gambart, S Puget, C A Maurage, C Miquel, C Dufour, P Leblond, N André, D Figarella Branger, J Kanold, J-L Kemeny, C Icher, A Vital, E Uro Coste, A I Bertozzi
The aim of this study was to review and describe therapeutic approaches in children with choroid plexus tumor (CPT) based on a nationwide series. The World Health Organization classification subdivides these rare tumors into three histological subtypes corresponding to three grades of malignancy: low grade (grade I) choroid plexus papilloma (CPP), intermediate grade (grade II) atypical choroid plexus papilloma (aCPP) and high grade (grade III) choroid plexus carcinoma (CPC). This retrospective study included 102 French children younger than 18 years, treated from 2000 to 2012: 54 CPP, 26 aCPP and 22 CPC...
October 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28650886/elevated-fdg-activity-in-the-nonpneumatized-sphenoid-bone-in-an-infant
#18
Yin Jie Chen, Nelleke Tolboom, Lisa J States, Hongming Zhuang
A 7-month-old boy with malignant rhabdoid tumor of the right lateral neck, status post resection and chemotherapy, underwent FDG PET/CT for restaging. The images showed diffuse increased activity in the spleen and in the bone marrow of the appendicular bones and the spine, which is related to hematopoietin administered after chemotherapy. The images also revealed intense activity in the region of sphenoid bone, which is not a common region to have elevated FDG activity. The subsequent MRI scan showed that this activity was due to not-yet-converted red marrow in the sphenoid bone in this pediatric patient...
October 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28648942/dedifferentiated-chondrosarcoma-of-bone-with-prominent-rhabdoid-component
#19
Matthew Stemm, Chad Beck, Rajeev Mannem, John Neilson, Michael J Klein
Dedifferentiated chondrosarcomas (DDCS) are rare lesions, defined as tumors having a low-grade chondrosarcomatous component with an abrupt transition to a high-grade sarcoma. Although "malignant fibrous histiocytoma" (undifferentiated pleomorphic sarcoma) is the most common high grade saromatous component, many different types of sarcoma have been described. We present a case of dedifferentiated chondrosarcoma with rhabdomyosarcomatous differentiation harboring a prominent rhabdoid tumor component. To our knowledge, rhabdoid morphology in dedifferentiated chondrosarcoma has not been described in the English-language literature...
June 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28642448/combined-brd4-and-cdk9-inhibition-as-a-new-therapeutic-approach-in-malignant-rhabdoid-tumors
#20
Natalia Moreno, Till Holsten, Julius Mertins, Annabelle Zhogbi, Pascal Johann, Marcel Kool, Michael Meisterernst, Kornelius Kerl
Rhabdoid tumors are caused by the deletion of SMARCB1, whose protein encodes the SMARCB1 subunit of the chromatin remodeling complex SWI/SNF that is involved in global chromatin organization and gene expression control. Simultaneously inhibiting the main players involved in the deregulated transcription machinery is a promising option for preventing exaggerated tumor cell proliferation and survival as it may bypass compensatory mechanisms. In support of this hypothesis, we report efficient impairment of cellular proliferation and strong induction of cell death elicited by inhibition of bromodomain protein BRD4 and transcription kinase CDK9 using small molecular compounds...
June 21, 2017: Oncotarget
keyword
keyword
77237
1
2
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"