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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/29110337/pituitary-atypical-teratoid-rhabdoid-tumor-in-a-patient-with-prolactinoma-a-unique-description
#4
Valeria Barresi, Simona Lionti, Alessandro Raso, Felice Esposito, Salvatore Cannavò, Filippo F Angileri
Atypical teratoid rhabdoid tumor (ATRT) is an aggressive tumor of the CNS and characteristically occurs in the pediatric age. In adulthood, ATRT is rare and it is mainly localized in the cerebral hemispheres. Only 16 cases of ATRT have been described in the sellar region up to now. Interestingly, all sellar ATRTs occurred in adult female patients. Herein we report a novel case of sellar ATRT in a patient with previous history of lactotroph adenoma. Similar to other sellar ATRTs, this case occurred in a female adult patient...
November 6, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29093183/therapeutic-targeting-of-pgbd5-induced-dna-repair-dependency-in-pediatric-solid-tumors
#5
Anton G Henssen, Casie Reed, Eileen Jiang, Heathcliff Dorado Garcia, Jennifer von Stebut, Ian C MacArthur, Patrick Hundsdoerfer, Jun Hyun Kim, Elisa de Stanchina, Yasumichi Kuwahara, Hajime Hosoi, Neil J Ganem, Filemon Dela Cruz, Andrew L Kung, Johannes H Schulte, John H Petrini, Alex Kentsis
Despite intense efforts, the cure rates of childhood and adult solid tumors are not satisfactory. Resistance to intensive chemotherapy is common, and targets for molecular therapies are largely undefined. We have found that the majority of childhood solid tumors, including rhabdoid tumors, neuroblastoma, medulloblastoma, and Ewing sarcoma, express an active DNA transposase, PGBD5, that can promote site-specific genomic rearrangements in human cells. Using functional genetic approaches, we discovered that mouse and human cells deficient in nonhomologous end joining (NHEJ) DNA repair cannot tolerate the expression of PGBD5...
November 1, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/29076075/proximal-type-epithelioid-sarcoma-in-skull-base-a-pathological-diagnosis-challenge-with-other-intracranial-tumors
#6
Duan Zejun, Yao Kun, Lu Dehong, Qi Xueling
Proximal-type ES (PES) is a rare and aggressive sarcoma originated from soft tissues with uncertain differentiation. It mainly affects middle-aged patients and often locates in proximal extremity and deep-seated tissues. Only one case of PES located in the skull base has been reported to date. Herein, we report two cases of PES occurred in the middle cranial fossa in two middle-aged Chinese women. Microscopically, the tumors were consisted of epithelial-like cells with or without rhabdoid cells. And frequent mitotic activity and coagulation necrosis were present...
October 26, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/29050071/-poorly-differentiated-chordoma-with-ini1-loss-a-clinicopathologic-study
#7
J Y Feng, L Chen, Y Y Ma, H W Yang, L Chen
Objective: To analyze the clinicopathologic characteristics of poorly-differentiated chordoma with INI1 loss in children and to discuss the differential diagnosis. Methods: The clinical, radiological, histopathological profiles and molecular pathologic characteristics of two pediatric poorly differentiated chordoma cases with INI1 loss were reviewed. Results: The patients were a girl and a boy. Both lesions involved the slope. Both patients were presented with progressive muscle weakness or neck pain. Radiological examination showed clivus bone destruction and compression of the brain stem and cervical spinal cord...
October 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29024210/a-case-of-anaplastic-lymphoma-kinase-positive-renal-cell-carcinoma-coincident-with-hodgkin-lymphoma
#8
Yuzo Oyama, Haruto Nishida, Takahiro Kusaba, Hiroko Kadowaki, Motoki Arakane, Tsutomu Daa, Dai Watanabe, Yasuyuki Akita, Fuminori Sato, Hiromitsu Mimata, Shigeo Yokoyama
We report a case of ALK-positive renal cell carcinoma coincident with Hodgkin lymphoma. The patient was a 19 year-old-girl without sickle cell trait. The right renal tumor was discovered concomitantly with Hodgkin lymphoma (HL). After chemotherapy for HL, right nephrectomy was performed. Microscopically, the tumor showed a solid and focally pseudo-papillary growth pattern studded with tubular structures. Most tumor cells were small bland eosinophilic cells, but rhabdoid cells, vacuolated cells, pleomorphic multinucleated giant cells were also admixed...
October 12, 2017: Pathology International
https://www.readbyqxmd.com/read/28982623/robotic-radical-left-nephrectomy-with-inferior-vena-cava-level-iii-thrombectomy
#9
Ryan J Nelson, Matthew J Maurice, Jihad H Kaouk
INTRODUCTION AND OBJECTIVE: We present our robotic approach and technique to manage a large left renal tumor with inferior vena cava (IVC) Level III thrombus. The superior mesenteric artery crossing the left renal vein requires robotic docking from the left, for dissection of the left kidney followed by repositioning and re-docking the robot from the right side for dissection of the right renal vein, distal and proximal IVC, and the remaining left renal vein insertion into the IVC. METHODS: We present a 53-year-old man with a 10...
September 2017: Urology
https://www.readbyqxmd.com/read/28966010/rhabdoid-tumor-of-the-liver-report-of-6-pediatric-cases-treated-at-a-single-institute
#10
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
#11
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/28900335/rhabdoid-meningioma-of-brain-a-rare-aggressive-tumor
#12
Sajeeb Mondal, Rajashree Pradhan, Subrata Pal, Sharmistha Chatterjee, Arindam Bandyapadhyay, Debosmita Bhattacharyya
Rhabdoid meningioma is a rare aggressive variant of meningioma, regarded as WHO Grade III type. Histologically and cytologically, it is distinctive type having abundant eosinophilic cytoplasm, cytoplasmic inclusion with eccentrically placed vesicular nuclei and prominent nucleoli. High recurrence rate and poor outcome are important features. Here, we are presenting a rare case of rhabdoid meningioma found in a recurrent meningioma of the posterior fossa in a middle-aged female. We emphasized the squash cytology and histology finding of the rare neoplasm...
April 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28877071/uncommon-localization-of-extrarenal-xp11-2-translocation-associated-renal-cell-carcinoma-rcc-case-report
#13
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/28875443/sinonasal-tract-alveolar-rhabdomyosarcoma-in-adults-a-clinicopathologic-and-immunophenotypic-study-of-fifty-two-cases-with-emphasis-on-epithelial-immunoreactivity
#14
Lester D R Thompson, Vickie Y Jo, Abbas Agaimy, Antonio Llombart-Bosch, Gema Nieto Morales, Isidro Machado, Uta Flucke, Paul E Wakely, Markku Miettinen, Justin A Bishop
Sinonasal tract (SNT) alveolar rhabdomyosarcoma (ARMS) are frequently misdiagnosed, especially in adults. Fifty-two adult (≥18 years) patients with SNT ARMS were reviewed and characterized by immunohistochemistry and molecular studies. Twenty-six females and 26 males (18-72 years; mean 43.2 years), presented after a short duration (mean 2.6 months) with a large (mean 5.5 cm) destructive nasal cavity mass, involving multiple contiguous paranasal sites (n = 46) and with cervical adenopathy (n = 41)...
September 5, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28870453/spinal-cord-tumors-in-children-a-review-of-21-cases-treated-at-the-same-institution
#15
I Merlot, J Francois, J-C Marchal, A Joud, R Guerbouz, P Chastagner, O Klein
INTRODUCTION: Spinal cord tumors in children (SCTC) are rare with a frequent diagnostic delay. Its management is multidisciplinary and challenging due to functional implications. The position of surgery is now better established but the role and timing of chemotherapy (CT) and radiotherapy (RT) still remains under debate. Adverse effects of treatments are important to be taken into account, in the follow-up of these children. The aim of this paper was to present a series of 21 cases of SCTC treated at the same institution, to briefly present clinical features, treatments and outcome, with a special focus on spinal deformities in children with this condition...
September 1, 2017: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28861890/high-grade-renal-cell-carcinoma-with-emperipolesis-clinicopathological-immunohistochemical-and-molecular-genetic-analysis-of-14-cases
#16
Pavla Rotterova, Petr Martinek, Reza Alaghehbandan, Kristyna Prochazkova, Ivan Damjanov, Joanna Rogala, Saul Suster, Delia Perez-Montiel, Isabel Alvarado-Cabrero, Maris Sperga, Marian Svajdler, Kvetoslava Michalova, Kristyna Pivovarcikova, Ondrej Daum, Milan Hora, Martin Dusek, Ondrej Ondic, Adela Stehlikova, Michal Michal, Ondrej Hes
Emperipolesis has recently been described as a constant feature of "biphasic squamoid" papillary renal cell carcinoma (BPRCC). We also noticed this in some high-grade (HG) RCC, which promoted the present study to estimate the incidence of emperipolesis in RCCs and to describe them in further detail. 14 cases of HGRCC showing emperipolesis were retrieved from our registry. Microscopic examination of filed slides was supplemented with immunohistochemical and molecular-genetic analyses using paraffin embedded tissue...
September 1, 2017: Histology and Histopathology
https://www.readbyqxmd.com/read/28860959/bilateral-rhabdoid-meningioma-mimicking-glioma-an-unusual-occurrence
#17
Varsha Dalal, Fouzia Siraj, Manveen Kaur, K B Shankar, Avninder Singh
Rhabdoid meningioma is an infrequent variant of meningioma, introduced for the first time in the 2000 World Health Organization's classification of tumors of the nervous system. Owing to its aggressive clinical course and high proliferative index, it has been classified as a grade III neoplasm. We describe a fifty-year-old male with headache, weakness of limbs, and altered sensorium. CT showed hetero-dense enhancing mass lesions in both right and left parietal areas raising suspicion of high grade glioma. Histopathologic and immunohistochemical examination revealed a tumor with features of rhabdoid meningioma...
2017: German Medical Science: GMS E-journal
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
#18
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/28842319/quantitative-phosphoproteomic-analysis-of-acquired-cancer-drug-resistance-to-pazopanib-and-dasatinib
#19
Simon Vyse, Frank McCarthy, Malgorzata Broncel, Angela Paul, Jocelyn P Wong, Amandeep Bhamra, Paul H Huang
Acquired drug resistance impacts the majority of patients being treated with tyrosine kinase inhibitors (TKIs) and remains a key challenge in modern anti-cancer therapy. The lack of clinically effective therapies to overcome resistance represents an unmet need. Understanding the signalling that drives drug resistance will facilitate the development of new salvage therapies to treat patients with secondary TKI resistance. In this study, we utilise mass spectrometry to characterise the global phosphoproteomic alterations that accompany the acquisition of resistance to two FDA-approved TKIs, pazopanib and dasatinib, in the A204 rhabdoid tumour cell line...
January 6, 2018: Journal of Proteomics
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
#20
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
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