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https://www.readbyqxmd.com/read/29346182/atypical-teratoid-rhabdoid-tumor-of-the-cauda-equina-in-a-child-report-of-a-very-unusual-case
#1
James M Shiflett, Betty L Herrington, David A Joyner, Ali G Saad
Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive malignant primitive neoplasms that commonly occur in children younger than 2 years of age. The prognosis is generally dismal with a median survival time of <1 year. The majority of AT/RT occur in the posterior fossa and less frequently the supratentorium. Primary pediatric spinal AT/RT are exceedingly rare and only 15 cases have been reported to date. Here we report a very unusual case of primary spinal AT/RT extensively involving the spinal cord from T11 down to the cauda equina...
January 16, 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/29344246/silibinin-inhibits-migration-and-invasion-of-the-rhabdoid-tumor-g401-cell-line-via-inactivation-of-the-pi3k-akt-signaling-pathway
#2
Yumei Li, Chunmei Zhang, Danfeng Cai, Congde Chen, Dongmei Mu
Rhabdoid tumors, which tend to occur prior to the age of 2 years, are one of the most aggressive malignancies and have a poor prognosis due to the frequency of metastasis. Silibinin, a natural extract, has been approved as a potential tumor suppressor in various studies, however, whether or not it also exerts its antitumor capacity in rhabdoid tumors, particularly with regards to tumor migration and invasion, is unclear. The rhabdoid tumor G401 cell line was used in the present in vitro study. An MTT assay was used to assess the cytotoxicity of silibinin on G401 cells, cell migration was studied using a wound healing assay and a Transwell migration assay, and cell invasion was determined using a Transwell invasion assay...
December 2017: Oncology Letters
https://www.readbyqxmd.com/read/29340047/inhibition-of-polo-like-kinase-4-plk4-a-new-therapeutic-option-for-rhabdoid-tumors-and-pediatric-medulloblastoma
#3
Simone Treiger Sredni, Anders W Bailey, Amreena Suri, Rintaro Hashizume, Xingyao He, Nundia Louis, Tufan Gokirmak, David R Piper, Daniel M Watterson, Tadanori Tomita
Rhabdoid tumors (RT) are highly aggressive and vastly unresponsive embryonal tumors. They are the most common malignant CNS tumors in infants below 6 months of age. Medulloblastomas (MB) are embryonal tumors that arise in the cerebellum and are the most frequent pediatric malignant brain tumors. Despite the advances in recent years, especially for the most favorable molecular subtypes of MB, the prognosis of patients with embryonal tumors remains modest with treatment related toxicity dreadfully high. Therefore, new targeted therapies are needed...
December 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/29339179/primary-diffuse-leptomeningeal-atypical-teratoid-rhabdoid-tumor-diagnosed-by-cerebrospinal-fluid-cytology-case-report-with-molecular-genetic-analysis
#4
Ran Tomomasa, Satoshi Nakata, Sumihito Nobusawa, Keishi Horiguchi, Nozomi Matsumura, Hayato Ikota, Junko Hirato, Yuhei Yoshimoto, Hideaki Yokoo
Atypical teratoid/rhabdoid tumors (AT/RT) are rare malignant neoplasms that mainly affect infants and young children, and are typically located in the cerebellar hemispheres. These tumors are histologically characterized by varying proportions of rhabdoid cells, and nuclear INI1 immunonegativity. Here, we report a case of a 15-year-old male with primary diffuse leptomeningeal AT/RT. The patient had symptoms similar to those of meningitis. Magnetic resonance imaging revealed leptomeningeal thickening. Cytological examination using cerebrospinal fluid was repeatedly performed and revealed rhabdoid cells with loss of INI1 reactivity, and shortly after, the diagnosis of AT/RT was confirmed by tissue biopsy...
January 12, 2018: Human Pathology
https://www.readbyqxmd.com/read/29324471/sellar-region-atypical-teratoid-rhabdoid-tumors-atrt-in-adults-display-dna-methylation-profiles-of-the-atrt-myc-subgroup
#5
Pascal D Johann, Susanne Bens, Florian Oyen, Rabea Wagener, Caterina Giannini, Arie Perry, Jack M Raisanen, Gerald F Reis, Sumihito Nobusawa, Kazunori Arita, Jörg Felsberg, Guido Reifenberger, Abbas Agaimy, Rolf Buslei, David Capper, Stefan M Pfister, Reinhard Schneppenheim, Reiner Siebert, Michael C Frühwald, Werner Paulus, Marcel Kool, Martin Hasselblatt
Atypical teratoid/rhabdoid tumor (ATRT) is a highly malignant brain tumor predominantly encountered in infants. Mutations of the SMARCB1 gene are the characteristic genetic lesion. A small group of ATRT stands out clinically, because these tumors are located in the sellar region of adults. To investigate if sellar region ATRT in adults represents a molecular distinct entity, we characterized molecular alterations in 7 sellar region ATRTs in adults as compared with 150 pediatric ATRTs and 47 pituitary adenomas using SMARCB1 sequencing, multiplex ligation-dependent probe amplification and fluorescence in situ hybridization as well as DNA methylation profiling...
January 10, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29316066/smarcb1-ini1-baf47-deficient-pleural-malignant-mesothelioma-with-rhabdoid-features
#6
Noriko Kimura, Masaru Hasegawa, Kenzo Hiroshima
Malignant mesothelioma (MM) with rhabdoid features is an MM variant. Fifteen cases have been reported previously, all of which were combined with other types of MM. Herein, we report an autopsy case of pleural MM with monomorphic rhabdoid features. The patient was a 62-year-old male without a history of asbestos exposure. An autopsy revealed a soft, granular tumor that replaced the entire left pleura and had invaded to the diaphragm and lower lobe of the lung. The tumor cells, which had eosinophilic plump cytoplasm and eccentric nuclei, were loosely cohesive...
January 5, 2018: Pathology International
https://www.readbyqxmd.com/read/29315108/review-of-molecular-classification-and-treatment-implications-of-pediatric-brain-tumors
#7
Ana S Guerreiro Stucklin, Vijay Ramaswamy, Craig Daniels, Michael D Taylor
PURPOSE OF REVIEW: Brain tumors are the most common solid tumors and leading cause of cancer-related death in children. The advent of large-scale genomics has resulted in a plethora of profiling studies that have mapped the genetic and epigenetic landscapes of pediatric brain tumors, ringing in a new era of precision diagnostics and targeted therapies. In this review, we highlight the most recent findings, focusing on studies published after 2015, and discuss how new evidence is changing the care of children with brain tumors...
February 2018: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/29307627/dedifferentiated-liposarcoma-composed-predominantly-of-rhabdoid-epithelioid-cells-a-frequently-misdiagnosed-highly-aggressive-variant
#8
Abbas Agaimy, Michael Michal, Ladislav Hadravsky, Michal Michal
Dedifferentiated liposarcoma is one of the most common sarcoma types in adults with a predilection for the retroperitoneum. We have recently encountered 6 cases of DDL composed predominantly of rounded, rhabdoid or epithelioid cells mimicking rhabdoid melanoma, epithelioid rhabdomyosarcoma or undifferentiated carcinoma. Patients were 5 males and one female aged 64 to 81 years (median, 68). Tumors originated in the retroperitoneum (n=5; 3 in the psoas muscle) and deep soft tissue of the thigh (n=1). All 3 patients with follow-up died of metastatic disease within 4 to 8 months...
January 4, 2018: Human Pathology
https://www.readbyqxmd.com/read/29288786/an-unusual-presentation-of-metastatic-urothelial-bladder-carcinoma-with-rhabdoid-features-presenting-as-obstructive-uropathy
#9
A Abello, J C Delto, A A Wagner, A K Das
No abstract text is available yet for this article.
December 27, 2017: Urology
https://www.readbyqxmd.com/read/29280680/smarcb1-deficient-tumors-of-childhood-a-practical-guide
#10
Bruce R Pawel
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)...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29258531/malignant-rhabdoid-tumor-in-the-renal-allograft-of-an-adult-transplant-recipient-a-unique-case-of-a-rare-tumor
#11
Jing Xiong, Tiefen Su, Pengcheng Zhu, Qilin Ao, Qiurong Ruan, Guoping Wang
BACKGROUND: Renal transplant recipients have increased risk for developing malignant diseases because of immunosuppression or donor-to-recipient transmission. Malignant rhabdoid tumor (MRT) is a rare, highly aggressive and lethal tumor primarily affecting the kidney of infants and young children. MRT has not been reported in the renal allograft of an adult recipient after kidney transplantation. CASE PRESENTATION: In this report, a 47-year-old woman who received a kidney transplantation from an infant donor and developed a mass in the transplanted kidney is presented...
December 19, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/29248378/atypical-teratoid-rhabdoid-tumor-of-the-sellar-region-in-adult-women-is-it-a-sex-related-disease
#12
REVIEW
Akihiro Nishikawa, Toshihiro Ogiwara, Alhusain Nagm, Kenji San, Maki Okada, Akihiro Chiba, Masahiro Agata, Tomoki Kaneko, Hisashi Tamada, Takeshi Uehara, Kazuhiro Hongo
Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal tumor in children and a rare entity. Although adult AT/RT is extremely rare, some cases of adult-onset AT/RT in the sellar region have been described. Here, we report an adult patient with AT/RT of the sellar region in whom it was difficult to make a definitive diagnosis and the clinical course was aggressive. This is the first report of autopsy findings that could confirm the clinical characteristics of this rare unresolved pathology, and will contribute to the improvement of prognosis...
December 13, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29237968/atypical-teratoid-rhabdoid-tumor-of-the-central-nervous-system-case-series-from-a-regional-tertiary-care-cancer-centre-in-south-india
#13
L Appaji, B S Aruna Kumari, K Govind Babu, C S Premalata, K P R Pramod, Gita R Bhat, Chinchu Prem
BACKGROUND: Atypical teratoid rhabdoid tumors (AT/RT) constitute a rare group of pediatric brain tumors. AIM: To study the clinical, histopathological, and immunohistochemical (IHC) profile, management and outcome of children with AT/RT of the central nervous system who presented between the years 2007 and 2015 in a regional tertiary care center in South India. MATERIALS AND METHODS: This was a retrospective study. Demographic and clinical data were obtained from the clinical case files...
October 2017: Journal of Cancer Research and Therapeutics
https://www.readbyqxmd.com/read/29230040/congenital-embryonal-rhabdomyosarcoma-caused-by-heterozygous-concomitant-ptch1-and-ptch2-germline-mutations
#14
Julia Taeubner, Triantafyllia Brozou, Nan Qin, Jasmin Bartl, Sebastian Ginzel, Joerg Schaper, Joerg Felsberg, Simone Fulda, Christian Vokuhl, Arndt Borkhardt, Michaela Kuhlen
The sonic hedgehog (SHH) signaling pathway has been shown to play important roles in embryogenesis, cell proliferation as well as in cell differentiation. It is aberrantly activated in various common cancers in adults, but also in pediatric neoplasms, such as rhabdomyosarcoma (RMS) and atypical teratoid/rhabdoid tumors (AT/RTs). Dysregulation and germline mutation in PATCHED1 (PTCH1), a receptor for SHH, is responsible for the Gorlin Syndrome, a familial cancer predisposing syndrome including RMS. Here, we report a newborn diagnosed with congenital embryonal RMS...
December 11, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29228610/targeting-polo-like-kinase-1-in-smarcb1-deleted-atypical-teratoid-rhabdoid-tumor
#15
Irina Alimova, Angela M Pierce, Peter Harris, Andrew Donson, Diane K Birks, Eric Prince, Ilango Balakrishnan, Nicholas K Foreman, Marcel Kool, Lindsey Hoffman, Sujatha Venkataraman, Rajeev Vibhakar
Atypical teratoid rhabdoid tumor (ATRT) is an aggressive and malignant pediatric brain tumor. Polo-like kinase 1 (PLK1) is highly expressed in many cancers and essential for mitosis. Overexpression of PLK1 promotes chromosome instability and aneuploidy by overriding the G2-M DNA damage and spindle checkpoints. Recent studies suggest that targeting PLK1 by small molecule inhibitors is a promising approach to tumor therapy. We investigated the effect of PLK1 inhibition in ATRT. Gene expression analysis showed that PLK1 was overexpressed in ATRT patient samples and tumor cell lines...
November 14, 2017: Oncotarget
https://www.readbyqxmd.com/read/29223518/synchronous-central-nervous-system-atypical-teratoid-rhabdoid-tumor-and-malignant-rhabdoid-tumor-of-the-kidney-case-report-of-a-long-term-survivor-and-review-of-the-literature
#16
Mohammad H Abu Arja, Priyal Patel, Summit H Shah, Jeffery J Auletta, Erin K Meyer, Suzanne E Conley, Jennifer H Aldrink, Jonathan A Pindrik, Mohamed S AbdelBaki
BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) with synchronous or metachronous extra-CNS disease is a rare childhood malignancy with a dismal prognosis. CASE DESCRIPTION: We report a seven-week-old female with metastatic AT/RT and synchronous malignant rhabdoid tumor of the kidney who received an intensive multimodal approach combining surgical resection, intrathecal chemotherapy, and high-dose chemotherapy with autologous peripheral blood stem cell transplant (PBSCT)...
December 6, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29218405/multimodal-treatments-combined-with-gamma-knife-surgery-for-primary-atypical-teratoid-rhabdoid-tumor-of-the-central-nervous-system-a-single-institute-experience-of-18-patients
#17
Yan-Ming Ren, Xia Wu, Chao You, Yue-Kang Zhang, Qiang Li, Yan Ju
OBJECTIVES: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, highly malignant tumor of the central nervous system with poor prognosis. Nowadays, multimodal management, including surgery, chemotherapy (CMT), and radiation therapy (RT), is advocated. However, AT/RT treatment with gamma knife surgery (GKS) was rarely reported. The aim of this study was to assess the efficacy and safety of GKS for the treatment of AT/RT. PATIENTS AND METHODS: Medical records of AT/RT patients who underwent surgery from 2007 to 2014 at the West China Hospital were retrospectively reviewed and statistically analyzed...
December 7, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29218250/evaluation-of-the-role-of-downregulation-of-snf5-ini1-core-subunit-of-swi-snf-complex-in-clear-cell-renal-cell-carcinoma-development
#18
Elzbieta Sarnowska, Michal Szymanski, Nataliia Rusetska, Marcin Ligaj, Iga Jancewicz, Pawel Cwiek, Marta Skrodzka, Marcin Leszczynski, Joanna Szarkowska, Alicja Chrzan, Malgorzata Stachowiak, Jaroslaw Steciuk, Anna Maassen, Lech Galek, Tomasz Demkow, Janusz A Siedlecki, Tomasz J Sarnowski
Clear cell renal cell carcinoma (ccRCC) is characterized by stabilization of hypoxia-inducible factor (HIF1), and mutations in von Hippel-Lindau (VHL) gene. Additionally, in about 40% of ccRCC cases the mutation in PBRM1 (POLYBROMO1) gene coding for a non-core subunit of SWI/SNF chromatin remodeling complex was found suggesting potential impairment of this complex function in ccRCC. In this study we assessed the extent to which the core SWI/SNF complex subunit - INI1 (hSNF5/SMARCB1) is affected in ccRCC and whether it has any consequences on the development of this type of cancer...
2017: American Journal of Cancer Research
https://www.readbyqxmd.com/read/29204511/smarca4-germline-gene-mutation-in-a-patient-with-epithelial-ovarian-a-case-report
#19
Reshma Muppala, Talia Donenberg, Marilyn S Huang, Matthew P Schlumbrecht
Background: SMARCA4 is gene whose protein product participates in chromatin remodeling. Somatic mutations in this gene are associated with non-small cell lung cancer and malignant rhabdoid tumors, and both germline and somatic mutations are seen with small cell carcinoma of the ovary, hypercalcemic type. To date, there are no data identifying an association with more common epithelial carcinomas of the ovary. Case: The patient is a 57-year-old female without any significant family history of cancer, diagnosed with high-grade serous carcinoma of the ovary...
November 2017: Gynecologic Oncology Reports
https://www.readbyqxmd.com/read/29200698/extrarenal-extracranial-rhabdoid-tumor-of-the-pelvis-in-a-young-adult-management-of-a-challenging-case
#20
Bharti Devnani, Ahitagni Biswas, Sameer Bakhshi, Seema Kaushal, Tripti Nakra
Rhabdoid tumor commonly occurs in the kidney and has an aggressive clinical course with high mortality. Central nervous system is the most common extrarenal site. Extrarenal extracranial rhabdoid tumor (EERT) is rare. EERT usually presents in childhood, and presentation in adulthood is extremely rare. This tumor is often difficult to diagnose, and there is no established standard of care due to the paucity of cases. We herein report a case of extrarenal rhabdoid tumor of the pelvis in a young adult and discuss the presentation and possible treatment options of this rare tumor...
July 2017: Indian Journal of Medical and Paediatric Oncology
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