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Rhabdomyosarcoma

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https://www.readbyqxmd.com/read/28548706/45-gy-is-not-sufficient-radiotherapy-dose-for-group-iii-orbital-embryonal-rhabdomyosarcoma-after-less-than-complete-response-to-12-weeks-of-arst0331-chemotherapy-a-report-from-the-soft-tissue-sarcoma-committee-of-the-children-s-oncology-group
#1
Ralph P Ermoian, John Breneman, David O Walterhouse, Yueh-Yun Chi, Jane Meza, James Anderson, Douglas S Hawkins, Andrea A Hayes-Jordan, David M Parham, Torunn I Yock, Sarah S Donaldson, Suzanne L Wolden
BACKGROUND: Recent Children's Oncology Group (COG) trials tested the efficacy of reduced therapy in an effort to lessen late effects compared to the Intergroup Rhabdomyosarcoma Study (IRS) IV regimen with associated hematologic and hepatic toxicity, and infertility. Here, we analyze the efficacy of 45 Gray (Gy) local radiotherapy (RT) in patients with Group III orbital embryonal rhabdomyosarcoma (ERMS) enrolled on the COG low-risk study ARST0331. PROCEDURE: Sixty-two patients with Group III orbital ERMS were treated on ARST0331 with four cycles of vincristine (VCR), dactinomycin (DACT), and cyclophosphamide (CPM; VAC, total cumulative CPM dose 4...
May 26, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28546788/dmd-transcripts-in-crl-2061-rhabdomyosarcoma-cells-show-high-levels-of-intron-retention-by-intron-specific-pcr-amplification
#2
Emma Tabe Eko Niba, Ryo Yamanaka, Abdul Qawee Mahyoob Rani, Hiroyuki Awano, Masaaki Matsumoto, Hisahide Nishio, Masafumi Matsuo
BACKGROUND: The DMD gene encoding dystrophin is mutated in Duchenne muscular dystrophy, a fatal progressive muscle wasting disease. DMD has also been shown to act as a tumor suppressor gene. Rhabdomyosarcoma (RMS) is a mesodermal sarcoma that shares characteristics of skeletal muscle precursors. Products of the DMD gene in RMS have not yet been fully clarified. Here, DMD products were analyzed in CRL-2061 cells established from alveolar RMS. METHODS: The 14-kb long DMD cDNA was PCR amplified as 20 separated fragments, as were nine short intron regions...
2017: Cancer Cell International
https://www.readbyqxmd.com/read/28544597/metastasis-of-soft-tissue-sarcomas-in-lymph-node-a-cytomorphological-study
#3
Jahnavi Gandhi, Shailee Mehta, Trupti Patel, Amisha Gami, Majal Shah, Dhaval Jetly
BACKGROUND: Soft-tissue sarcoma (STS) rarely metastasizes to lymph node compared to carcinoma. Fine-needle aspiration cytology (FNAC) carries a pivotal role in diagnosis of metastatic tumor to lymph node. This study highlights the role of FNAC in diagnosis of STS metastasis to lymph node. METHOD: A retrospective study over a period by 4 years carried out. FNAC of enlarged lymph node was performed in patients with STS. Cytology smears were examined in conjunction with clinical details...
May 24, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28540560/alveolar-rhabdomyosarcoma-decellularization
#4
Michela Pozzobon, Mattia Saggioro, Stefania D'Agostino, Gianni Bisogno, Maurizio Muraca, Piergiorgio Gamba
In cancer research, it is an urgent need in the obtainment of a simple and reproducible model that mimics in all the complexity the pathological microenvironment. Specifically, the will to improve the overall survival of young patients affected by rhabdomyosarcoma compels researchers to develop new models resembling the multifaceted environment of the pathology to deeply study the disease under novel and different aspects. To this end, we developed a decellularization protocol for alveolar rhabdomyosarcoma (ARMS) able to maintain the three-dimensional structure...
May 25, 2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28539588/cooperation-of-oncolytic-herpes-virotherapy-and-pd-1-blockade-in-murine-rhabdomyosarcoma-models
#5
Chun-Yu Chen, Pin-Yi Wang, Brian Hutzen, Les Sprague, Hayley M Swain, Julia K Love, Joseph R Stanek, Louis Boon, Joe Conner, Timothy P Cripe
Oncolytic virotherapy is an effective immunotherapeutic approach for cancer treatment via a multistep process including direct tumor cell lysis, induction of cytotoxic or apoptosis-sensitizing cytokines and promotion of antitumor T cell responses. Solid tumors limit the effectiveness of immunotherapeutics in diverse ways such as secretion of immunosuppressive cytokines and expression of immune inhibitory ligands to inhibit antitumor T cell function. Blocking programmed cell death protein (PD)-1 signaling, which mediates T cell suppression via engagement of its inhibitory ligands, PD-L1 or PD-L2, is of particular interest due to recent successes in many types of cancer...
May 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28534646/pulmonary-rhabdomyosarcoma-associated-with-check-valve-mechanism
#6
Udit Chaddha, Jeffrey A Hagen, Bassam Yaghmour
No abstract text is available yet for this article.
May 23, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28530261/a-rare-case-of-embryonal-rhabdomyosarcoma-of-the-parapharyngeal-space
#7
G Iannella, M De Vincentiis, A Corsi, A Greco, G Magliulo
A 24-year-old man was admitted to our Otolaryngology Department following a head and neck CT scan performed for cranial trauma that showed a bulky neoformation in the right parapharyngeal space. Magnetic resonance imaging confirmed the presence of an oval formation with sharp margins and colliquative areas of necrosis involving the right parapharyngeal space. The mass was completely excised by a latero-cervical approach. Based on histological features and immunohistochemical analysis, a diagnosis of embryonal rhabdomyosarcoma of the parapharyngeal space was made...
May 22, 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28529244/identification-of-tumor-initiating-cells-derived-from-two-canine-rhabdomyosarcoma-cell-lines
#8
Takuya Evan Kishimoto, Shoko Yashima, Rei Nakahira, Eri Onozawa, Daigo Azakami, Makoto Ujike, Kazuhiko Ochiai, Toshiyuki Ishiwata, Kimimasa Takahashi, Masaki Michishita
Cancer stem cells or tumor-initiating cells (TICs) are a small subpopulation of cells that have the capacity to self-renew, differentiate and initiate tumors. These cells may function in tumor initiation, aggression and recurrence. Whether spheres derived from canine rhabdomyosarcoma cells have stem cell-like properties is unclear. We induced sphere formation in the canine rhabdomyosarcoma cell lines, CMS-C and CMS-J, and characterized the spheres in vitro and in vivo. Sphere-forming cells were more resistant to vincristine, mitoxantrone and doxorubicin than adherent cells...
May 21, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28528067/transesophageal-echocardiograms-of-a-left-atrial-rhabdomyosarcoma
#9
Takahiro Tamura
No abstract text is available yet for this article.
June 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28524158/sequencing-of-dicer1-in-sarcomas-identifies-biallelic-somatic-dicer1-mutations-in-an-adult-onset-embryonal-rhabdomyosarcoma
#10
Leanne de Kock, Barbara Rivera, Timothée Revil, Paul Thorner, Catherine Goudie, Dorothée Bouron-Dal Soglio, Catherine S Choong, John R Priest, Paul J van Diest, Jantima Tanboon, Anja Wagner, Jiannis Ragoussis, Peter Fm Choong, William D Foulkes
BACKGROUND: Sarcomas are rare and heterogeneous cancers. We assessed the contribution of DICER1 mutations to sarcoma development. METHODS: The coding region of DICER1 was sequenced in 67 sarcomas using a custom Fluidigm Access Array. The RNase III domains were Sanger sequenced in six additional sarcomas to identify hotspot DICER1 variants. RESULTS: The median age of sarcoma diagnosis was 45.7 years (range: 3 months to 87.4 years). A recurrent embryonal rhabdomyosarcoma (ERMS) of the broad ligament, first diagnosed at age 23 years, harboured biallelic pathogenic somatic DICER1 variants (1 truncating and 1 RNase IIIb missense)...
May 18, 2017: British Journal of Cancer
https://www.readbyqxmd.com/read/28523882/phacomatosis-pigmentokeratotica-a-mosaic-rasopathy-with-malignant-potential
#11
Amit Om, Sara S Cathey, Robert M Gathings, Michelle Hudspeth, Jennifer A Lee, Sean Marzolf, Lara Wine Lee
Phacomatosis pigmentokeratotica (PPK) is a rare epidermal nevus syndrome characterized by the co-occurrence of a nevus sebaceous arranged along the lines of Blaschko with a speckled lentiginous nevus (SLN). We report a novel KRAS mutation in a patient with a large nevus sebaceous and an SLN who subsequently developed a vaginal botryoid rhabdomyosarcoma, an association not previously reported in the literature. This case expands our knowledge of the genetic basis for phacomatosis, in which mutations in HRAS have been previously described, although this report provides evidence that activating mutations in KRAS or HRAS may cause PPK...
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28521631/immunohistochemical-profile-of-myc-protein-in-pediatric-small-round-blue-cell-tumors
#12
Karen M Chisholm, Chandra Krishnan, Amy Heerema-McKenney, Yasodha Natkunam
Deregulation of MYC oncoprotein in cancers can result from multiple oncogenic mechanisms. Although MYC translocations define Burkitt lymphoma and MYC protein expression is a poor prognostic factor in undifferentiated neuroblastomas, the distribution of MYC protein (c-MYC) across other pediatric small round blue cell tumors (SRBCT) has not been well characterized. We undertook this study to assess MYC protein expression in a large cohort of pediatric lymphomas, sarcomas, and other SRBCT. Tissue microarrays containing 302 SRBCT were successfully evaluated by immunohistochemistry using anti-MYC clone Y69, with nuclear positivity scored as 0%, 1%-25%, 26%-50%, 51%-75%, or 76%-100%...
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28521080/histology-fusion-status-and-outcome-in-metastatic-rhabdomyosarcoma-a-report-from-the-children-s-oncology-group
#13
Erin R Rudzinski, James R Anderson, Yueh-Yun Chi, Julie M Gastier-Foster, Caroline Astbury, Frederic G Barr, Stephen X Skapek, Douglas S Hawkins, Brenda J Weigel, Alberto Pappo, William H Meyer, Michael A Arnold, Lisa A Teot, David M Parham
BACKGROUND: Distinguishing alveolar rhabdomyosarcoma (ARMS) from embryonal rhabdomyosarcoma (ERMS) has historically been of prognostic and therapeutic importance. However, classification has been complicated by shifting histologic criteria required for an ARMS diagnosis. Children's Oncology Group (COG) studies after IRS-IV, which included the height of this diagnostic shift, showed both an increased number of ARMS and an increase in the proportion of fusion-negative ARMS. Following diagnostic standardization and histologic re-review of ARMS cases enrolled during this era, analysis of low-risk (D9602) and intermediate-risk (D9803) rhabdomyosarcoma (RMS) studies showed that fusion status rather than histology best predicts prognosis for patients with RMS...
May 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28515009/transfer-learning-on-fused-multiparametric-mr-images-for-classifying-histopathological-subtypes-of-rhabdomyosarcoma
#14
Imon Banerjee, Alexis Crawley, Mythili Bhethanabotla, Heike E Daldrup-Link, Daniel L Rubin
This paper presents a deep-learning-based CADx for the differential diagnosis of embryonal (ERMS) and alveolar (ARMS) subtypes of rhabdomysarcoma (RMS) solely by analyzing multiparametric MR images. We formulated an automated pipeline that creates a comprehensive representation of tumor by performing a fusion of diffusion-weighted MR scans (DWI) and gadolinium chelate-enhanced T1-weighted MR scans (MRI). Finally, we adapted transfer learning approach where a pre-trained deep convolutional neural network has been fine-tuned based on the fused images for performing classification of the two RMS subtypes...
May 5, 2017: Computerized Medical Imaging and Graphics: the Official Journal of the Computerized Medical Imaging Society
https://www.readbyqxmd.com/read/28510278/tfg-met-fusion-in-an-infantile-spindle-cell-sarcoma-with-neural-features
#15
Uta Flucke, Max M van Noesel, Marc Wijnen, Lei Zhang, Chun-Liang Chen, Yun-Shao Sung, Cristina R Antonescu
An increasing number of congenital and infantile sarcomas displaying a primitive, monomorphic spindle cell phenotype have been characterized to harbor recurrent gene fusions, including infantile fibrosarcoma and congenital spindle cell rhabdomyosarcoma. Here we report an unusual spindle cell sarcoma presenting as a large and infiltrative pelvic soft tissue mass in a 4-month-old girl, which revealed a novel TFG-MET gene fusion by whole transcriptome RNA sequencing. The tumor resembled the morphology of an infantile fibrosarcoma with both fascicular and patternless growth, however, it expressed strong S100 protein immunoreactivity, while lacking SOX10 staining and retaining H3K27me3 expression...
May 16, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28507282/p53-suppresses-ribonucleotide-reductase-via-inhibiting-mtorc1
#16
Zhengfu He, Xing Hu, Weijin Liu, Adrienne Dorrance, Ramiro Garzon, Peter J Houghton, Changxian Shen
Balanced deoxyribonucleotides pools are essential for cell survival and genome stability. Ribonucleotide reductase is the rate-limiting enzyme for the production of deoxyribonucleotides. We report here that p53 suppresses ribonucleotide reductase subunit 1 (RRM1) and 2 (RRM2) via inhibiting mammalian target of rapamycin complex 1 (mTORC1). In vitro, cancer cell lines and mouse embryonic fibroblast cells were treated with different concentrations of pharmacological inhibitors for different times. In vivo, rhabdomyosarcoma Rh30 cell tumor-bearing mice were treated with rapamycin or AZD8055...
April 26, 2017: Oncotarget
https://www.readbyqxmd.com/read/28506520/the-evolving-role-of-proton-beam-therapy-for-sarcomas
#17
S Frisch, B Timmermann
As an alternative to conventional photon-based radiotherapy, radiation with protons is recognised to offer considerable advantages. Today, central nervous system tumours, various sarcomatous tumours, childhood cancer and head and neck tumours are commonly treated with proton therapy. This review evaluates current data from clinical and dosimetric trials on the treatment of selected sarcomatous tumours like rhabdomyosarcoma, osteosarcoma, chordoma, chondrosarcoma and Ewing sarcoma. Special considerations for paediatric tumours and future prospects of proton therapy are outlined...
May 12, 2017: Clinical Oncology: a Journal of the Royal College of Radiologists
https://www.readbyqxmd.com/read/28502472/a-novel-concept-for-tumour-targeting-with-radiation-inverse-dose-painting-or-targeting-the-low-drug-uptake-volume
#18
Ala Yaromina, Marlies Granzier, Rianne Biemans, Natasja Lieuwes, Wouter van Elmpt, Georgy Shakirin, Ludwig Dubois, Philippe Lambin
BACKGROUND AND PURPOSE: We tested a novel treatment approach combining (1) targeting radioresistant hypoxic tumour cells with the hypoxia-activated prodrug TH-302 and (2) inverse radiation dose-painting to boost selectively non-hypoxic tumour sub-volumes having no/low drug uptake. MATERIAL AND METHODS: (18)F-HX4 hypoxia tracer uptake measured with a clinical PET/CT scanner was used as a surrogate of TH-302 activity in rhabdomyosarcomas growing in immunocompetent rats...
May 11, 2017: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/28501984/pediatric-gynecologic-cancers
#19
REVIEW
Lauren Pommert, William Bradley
PURPOSE OF REVIEW: Three primary categories of gynecologic cancer are found in pediatric and adolescent patients: stromal carcinomas including juvenile granulosa cell tumors and Sertoli-Leydig cell tumors, rhabdomyosarcomas arising from the vagina and cervix (sarcoma botryoides), and ovarian germ cell tumors which comprise a wide range of histologies. These entities are rare and treatment approaches have focused on decreasing late effects of chemotherapy treatment. Here, we review presentation, histologic classifications, diagnosis, and treatment recommendations for pediatric gynecologic cancers...
July 2017: Current Oncology Reports
https://www.readbyqxmd.com/read/28501810/alveolar-rhabdomyosarcoma-in-a-6-month-old-boy
#20
Andrea Bassi, Barbara Zamma Gallarati, Iacopo Sardi, Milena Guidi, Chiara Caporalini, Paola Pelosi, Maurizio de Martino
No abstract text is available yet for this article.
May 13, 2017: Archives of Disease in Childhood
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