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Rhabdomyosarcoma

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https://www.readbyqxmd.com/read/28447920/prolonged-circulation-and-increased-tumor-accumulation-of-liposomal-vincristine-in-a-mouse-model-of-rhabdomyosarcoma
#1
Maurizio Roveri, Alice Pfohl, Patricia Jaaks, Nagjie Alijaj, Jean-Christophe Leroux, Paola Luciani, Michele Bernasconi
AIM: Our goal was to improve vincristine (VCR) based rhabdomyosarcoma (RMS) therapy by encapsulating the drug into liposomes. A targeting strategy was attempted to enhance tumor accumulation. MATERIALS & METHODS: VCR was loaded in control and peptide-decorated liposomes via an active method. The interaction of an RMS-specific peptide with the presumed target furin and the cellular uptake of both liposomal groups were studied in vitro. Pharmacokinetics and biodistribution of VCR-containing liposomes were assessed in an RMS xenograft mouse model...
April 27, 2017: Nanomedicine
https://www.readbyqxmd.com/read/28447112/-imaging-diagnostics-of-breast-metastases-from-extramammary-tumors
#2
REVIEW
S Wienbeck, S Nemat, J Lotz, A Surov
Breast metastases of solid extramammary tumors are very rare in comparison to primary malignancies of the breast and account for only 0.33-6.3% of all malignant neoplasms of the breast. The most common primary tumors are malignant melanoma, distant sarcomas, lung cancer, ovarian cancer, renal cell cancer and thyroid cancer in decreasing order of frequency. This review article summarizes the clinical features and the different imaging findings of breast metastases from different extramammary solid tumors. Breast metastases are often incidental findings in computed tomography (CT) or positron emission tomography CT (PET-CT) imaging...
April 26, 2017: Der Radiologe
https://www.readbyqxmd.com/read/28446439/pax3-foxo1-establishes-myogenic-super-enhancers-and-confers-bet-bromodomain-vulnerability
#3
Berkley E Gryder, Marielle E Yohe, Hsien-Chao Chou, Xiaohu Zhang, Joana Marques, Marco Wachtel, Beat Schaefer, Nirmalya Sen, Young K Song, Alberto Gualtieri, Silvia Pomella, Rossella Rota, Abigail Cleveland, Xinyu Wen, Sivasish Sindiri, Jun S Wei, Frederic G Barr, Sudipto Das, Thorkell Andresson, Rajarshi Guha, Madhu Lal-Nag, Marc Ferrer, Jack F Shern, Keji Zhao, Craig J Thomas, Javed Khan
Alveolar rhabdomyosarcoma is a life-threatening myogenic cancer of children and adolescent young adults, driven primarily by the chimeric transcription factor PAX3-FOXO1 (P3F). The mechanisms by which P3F dysregulates chromatin are unknown. We find P3F reprograms the cis-regulatory landscape by inducing (de novo) super enhancers (SEs). P3F uses SEs to setup auto-regulatory loops in collaboration with master transcription factors MYOG, MYOD and MYCN. This myogenic SE circuitry is consistent across cell lines and primary tumors...
April 26, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/28445278/analysis-of-treatment-of-large-abdominal-malignancies-in-children-complicated-with-abdominal-compartment-syndrome-report-of-six-cases
#4
Zeng Le-Xiang, Wu Yao-Hao, Li Na, Qiu Rong-Lin, Zhou Jia-Jia, Jiang Wen-Li, Zhang Jie, Deng Xiao-Geng
To explore effective treatment of large abdominal malignancies in children complicated with abdominal compartment syndrome (ACS).Six children with large abdominal malignancies complicated with ACS were admitted to our department from January 2013 to January 2016, and the changes in their breathing, heart rate, oxygen saturation, abdominal circumference, bladder pressure, and urine output, as well as the treatment measures and outcomes, were retrospectively analyzed.The 6 children included 1 child with bilateral nephroblastoma, 1 child with abdominal alveolar rhabdomyosarcoma, 1 child with right ovarian malignant teratoma complicated with abdominal glioma, 1 child with abdominal malignant teratoma, 1 child with right nephroblastoma, and 1 child with left adrenal gland neuroblastoma...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28438533/association-of-mir-145-with-statin-induced-skeletal-muscle-toxicity-in-human-rhabdomyosarcoma-rd-cells
#5
Shun Saito, Takeo Nakanishi, Yuma Shirasaki, Miki Nakajima, Ikumi Tamai
Skeletal muscle toxicity including rhabdomyolysis in severe case is a major side effect of LDL cholesterol-lowering statin drugs. We, therefore, aimed to explore microRNA (miRNA) expression to understand molecular mechanism of statin-induced toxicity. miRNA expression profiling assay for cerivastatin (1 μM for 48 hr)-treated RD cells showed more than two-fold decrease in 26 miRNA expressions with miR-145 being down-regulated prominently. When RD cells were treated with cerivastatin at 10 μM for 36 hr, mitochondrial dysfunction was observed in 49...
April 21, 2017: Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28437010/technical-note-comparison-of-peripheral-patient-dose-from-mr-guided-60-co-therapy-and-6mv-linear-accelerator-igrt
#6
Pascal Hauri, Roger A Hälg, Uwe Schneider
PURPOSE: The use of X-ray imaging in radiation therapy can give a substantial dose to the patient. A Cobalt machine combined with an magnetic resonance imaging (MRI) was recently introduced to clinical work. One positive aspect of using non-ionizing imaging devices is the reduction of the patient exposure. The purpose of this study was to quantify the difference in out-of-field dose to the patient between the image guided radiation therapy (IGRT) treatment applied with a linear accelerator with cone beam CT (CBCT) equipment and a Cobalt machine combined with an MRI...
April 24, 2017: Medical Physics
https://www.readbyqxmd.com/read/28431807/rhabdomyosarcoma-of-the-maxillofacial-region-in-children-and-adolescents-report-of-9-cases-and-literature-review
#7
Ioannis Iatrou, Nadia Theologie-Lygidakis, Ourania Schoinohoriti, Fotis Tzermpos, Anna-Maria Vessala
OBJECTIVE: To review clinical presentation, histology, treatment and survival for pediatric maxillofacial rhabdomyosarcoma (RMS) and evaluate the role of surgical treatment. STUDY DESIGN: Retrospective analysis of medical charts. METHODS: Files of patients, treated for primary maxillofacial RMS from 1997 to 2016, were examined for clinical presentation, staging, histology, treatment protocol and complications, recurrence and final outcome...
March 23, 2017: Journal of Cranio-maxillo-facial Surgery
https://www.readbyqxmd.com/read/28427078/a-case-of-adult-metastatic-rhabdomyosarcoma-of-the-prostate-cured-by-long-term-chemotherapy-with-local-radiation
#8
Masato Konno, Koji Mitsuzuka, Shigeyuki Yamada, Shinichi Yamashita, Yasuhiro Kaiho, Akihiro Ito, Mika Watanabe, Yoichi Arai
A 32-year-old man visited our hospital due to urinary retention. Prostate-specific antigen was 13.7 ng/mL. Imaging examinations showed a large prostatic tumor and lung and internal iliac lymph node metastases. Transrectal biopsy confirmed embryonal rhabdomyosarcoma of the prostate classified as cT2bN1M1 (stage IV) by the International Rhabdomyosarcoma Study (IRS) Group staging system. Systemic chemotherapy was started according to the IRS III regimen 36, which consisted of 16 weeks of induction chemotherapy with radiation...
April 21, 2017: Urologia Internationalis
https://www.readbyqxmd.com/read/28424409/clinical-genomic-profiling-to-identify-actionable-alterations-for-investigational-therapies-in-patients-with-diverse-sarcomas
#9
Roman Groisberg, David S Hong, Vijaykumar Holla, Filip Janku, Sarina Piha-Paul, Vinod Ravi, Robert Benjamin, Shreyas Kumar Patel, Neeta Somaiah, Anthony Conley, Siraj M Ali, Alexa B Schrock, Jeffrey S Ross, Philip J Stephens, Vincent A Miller, Shiraj Sen, Cynthia Herzog, Funda Meric-Bernstam, Vivek Subbiah
BACKGROUND: There are currently no United States Food and Drug Administration approved molecularly matched therapies for sarcomas except gastrointestinal stromal tumors. Complicating this is the extreme diversity, heterogeneity, and rarity of these neoplasms. Few therapeutic options exist for relapsed and refractory sarcomas. In clinical practice many oncologists refer patients for genomic profiling hoping for guidance on treatment options after standard therapy. However, a systematic analysis of actionable mutations has yet to be completed...
April 5, 2017: Oncotarget
https://www.readbyqxmd.com/read/28423221/facial-asymmetry-in-head-and-neck-rhabdomyosarcoma-survivors
#10
Reineke A Schoot, Marinka L F Hol, Johannes H M Merks, Michael Suttie, Olga Slater, Marinde van Lennep, Saskia M J Hopman, David Dunaway, Jonathan Syme-Grant, Ludi E Smeele, Koos H Zwinderman, Huib N Caron, Peter Hammond
INTRODUCTION: Radiotherapy is essential for achieving and maintaining local control in head and neck rhabdomyosarcoma (HNRMS) patients. However, radiotherapy may cause outgrowth disturbances of facial bone and soft tissue, resulting in facial asymmetry. The aim of this study was to develop a method to visualize and measure facial asymmetry in HNRMS survivors using three-dimensional (3D) imaging techniques. METHODS: Facial deformity was evaluated in a multidisciplinary clinical assessment of 75 HNRMS survivors, treated with external beam radiotherapy (EBRT, n = 26) or Ablative surgery, MOulage brachytherapy, and REconstruction (AMORE, n = 49)...
April 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28421158/cytotoxicity-of-zardaverine-in-embryonal-rhabdomyosarcoma-from-a-costello-syndrome-patient
#11
Donna M Cartledge, Katherine M Robbins, Katherine M Drake, Rachel Sternberg, Deborah L Stabley, Karen W Gripp, E Anders Kolb, Katia Sol-Church, Andrew D Napper
Costello syndrome (CS) patients suffer from a very high 10% incidence of embryonal rhabdomyosarcoma (ERMS). As tools to discover targeted therapeutic leads, we used a CS patient-derived ERMS cell line (CS242 ERMS) harboring a homozygous p.G12A mutation in HRAS, and a control cell line derived from the same patient comprising non-malignant CS242 fibroblasts with a heterozygous p.G12A HRAS mutation. A library of 2,000 compounds with known pharmacological activities was screened for their effect on CS242 ERMS cell viability...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/28417853/the-role-of-18-f-fdg-pet-ct-in-pediatric-sarcoma
#12
REVIEW
Douglas J Harrison, Marguerite T Parisi, Barry L Shulkin
Considerable debate remains regarding how best to incorporate (18)F-FDG-PET/CT into clinical practice for pediatric sarcomas. Although there is a clear role for (18)F-FDG-PET/CT in staging pediatric sarcoma, the value of (18)F-FDG-PET/CT in prognostication for pediatric sarcomas remains unclear. In osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma, (18)F-FDG-PET/CT may be most useful in the identification of skeletal metastases, where the literature consistently suggests that it has improved sensitivity and specificity as compared to bone scintigraphy...
May 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28413550/primary-intracranial-rhabdomyosarcoma-of-the-cerebellopontine-angle-mimicking-a-vestibular-schwannoma-in-a-child
#13
Prakash Nair, Kuntal Kanti Das, Arun K Srivastava, R N Sahu, Raj Kumar, Kamlesh Yadava, Rakesh Pandey
Primary intracranial rhabdomyosarcoma (PIRMS) is a rare neoplasm, which affects infants and young children. We report a rare case of a primary embryonal rhabdomyosarcoma of the cerebellopontine angle in a 7-year-old boy with clinical and radiological features mimicking a vestibular schwannoma. The patient underwent definitive surgery and radiotherapy and is recurrence free at 6 months. PIRMS commonly occur in parameningeal locations or metastasize to the brain from an extracranial primary. Gross total tumor resection followed by adjuvant radiotherapy remains the current standard of treatment; however, chemotherapy has also been tried with favorable results...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28407197/autologous-hematopoietic-stem-cell-transplantation-following-high-dose-chemotherapy-for-nonrhabdomyosarcoma-soft-tissue-sarcomas
#14
REVIEW
Frank Peinemann, Heike Enk, Lesley A Smith
BACKGROUND: Soft tissue sarcomas (STS) are a highly heterogeneous group of rare malignant solid tumors. Nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) comprise all STS except rhabdomyosarcoma. In people with advanced local or metastatic disease, autologous hematopoietic stem cell transplantation (HSCT) applied after high-dose chemotherapy (HDCT) is a planned rescue therapy for HDCT-related severe hematologic toxicity. The rationale for this update is to determine whether any randomized controlled trials (RCTs) have been conducted and to clarify whether HDCT followed by autologous HSCT has a survival advantage...
April 13, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28400352/frequency-of-uterine-sarcomas-in-patients-admitted-for-uterine-fibroids-surgery
#15
Liselotte Mettler, Nicolai Maass, Khulkar Abdusattarova, Astrid Dempfle, Ibrakhim Alkatout
OBJECTIVE: To estimate the frequency of unsuspected uterine sarcoma identified postoperatively in women undergoing surgery for presumed benign uterine fibroids at the single-center university hospital. MATERIAL AND METHODS: This was a retrospective single-center study; the records of all 2,275 patients with uterine fibroids and uterine sarcomas from 2003 to 2015 were reviewed. Descriptive statistics were used to analyze demographic and clinical characteristics. To calculate confidence intervals (CIs), the exact Clopper-Pearson method was applied...
February 7, 2017: Journal of the Turkish German Gynecological Association
https://www.readbyqxmd.com/read/28397540/a-population-based-analysis-of-survival-for-sinonasal-rhabdomyosarcoma
#16
Aykut A Unsal, Sei Yeon Chung, Aylin B Unsal, Soly Baredes, Jean Anderson Eloy
Objectives Literature detailing factors affecting survival in sinonasal rhabdomyosarcoma (SNRMS) is limited due to its rarity. We analyze the demographics, potential prognostic factors, overall survival, and treatment efficacy of SNRMS. Study Design and Setting Administrative database study. Methods SNRMS was queried in the SEER database (1973-2013; Surveillance, Epidemiology, and End Results). Data were analyzed for demographic and clinicopathologic trends. Kaplan-Meier model was utilized for assessing survival...
April 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28388581/temozolomide-combined-with-irinotecan-caused-regression-in-an-adult-pleomorphic-rhabdomyosarcoma-patient-derived-orthotopic-xenograft-pdox-nude-mouse-model
#17
Kentaro Igarashi, Kei Kawaguchi, Tasuku Kiyuna, Takashi Murakami, Shinji Miwa, Scott D Nelson, Sarah M Dry, Yunfeng Li, Arun S Singh, Hiroaki Kimura, Katsuhiro Hayashi, Norio Yamamoto, Hiroyuki Tsuchiya, Fritz C Eilber, Robert M Hoffman
Adult pleomorphic rhabdomyosarcoma (RMS) is a rare and recalcitrant, highly-malignant mesenchymal tumor in need of improved therapeutic strategies. Our laboratory pioneered the patient-derived orthotopic xenograft (PDOX) nude mouse model with the technique of surgical orthotopic implantation (SOI). We previously described the development of a PDOX model of adult pleomorphic RMS where the tumor behaved similar to the patient donor. A high-grade pleomorphic rhabdomyosarcoma from a striated muscle was previously grown orthotopically in the right biceps-femoris muscle of nude mice to establish the PDOX model...
March 24, 2017: Oncotarget
https://www.readbyqxmd.com/read/28384876/primary-ewing-sarcoma-of-sphenoid-bone-with-intracranial-extension-a-common-tumour-at-an-uncommon-location
#18
Guddi Rani Singh, Vijayanand Choudhary, Rawi Agrawal
Primary Ewing Sarcoma of the cranial bone is rare, contributing to only 1% of all Ewing Sarcomas. Primary cranial Ewing Sarcoma occurs most commonly in temporal bone followed by parietal and occipital bones. Sphenoid bone is less commonly involved. We report a case of Ewing Sarcoma of the sphenoid bone with intra-cranial extension in a 20-month-old boy. On CT scan a provisional diagnosis of rhabdomyosarcoma was made. Fine Needle Aspiration Cytology (FNAC) and histopathological examination of core needle biopsy showed small round cell tumour...
February 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28380437/nell1-whose-high-expression-correlates-with-negative-outcomes-has-different-methylation-patterns-in-alveolar-and-embryonal-rhabdomyosarcoma
#19
Lucia Tombolan, Elena Poli, Paolo Martini, Angelica Zin, Chiara Romualdi, Gianni Bisogno, Gerolamo Lanfranchi
Rhabdomyosarcoma (RMS), which represents the most frequent soft tissue sarcoma in pediatric populations, is classified into two major subtypes: embryonal RMS (ERMS) and alveolar RMS (ARMS). ARMS subtype, which shows greater aggressiveness and proneness to metastasis with respect to ERMS, are characterized, in about 75% of cases, by specific chromosomal translocations that involve PAX and FOXO1 genes. Many findings have demonstrated that PAX/FOXO1-positive ARMS have a worse prognosis than PAX/FOXO1-negative ones and that distinct molecular features characterize RMS with different gene fusion statuses...
March 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28378394/fusion-gene-addiction-can-tumours-be-forced-to-give-up-the-habit
#20
Joanna L Selfe, Janet Shipley
Fusion of genes in tumours can have oncogenic roles in reprogramming cells through overexpression of oncogenes or the production of novel fusion proteins. A fundamental question in cancer biology is what genetic events are critical for initiation and whether these are also required for cancer progression. In recent work published in The Journal of Pathology, dependency on a fusion protein was addressed using a model of alveolar rhabdomyosarcomas - a sarcoma subtype with frequent fusion of PAX3 and FOXO1 genes that is associated with poor outcome...
April 5, 2017: Journal of Pathology
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