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Rhabdomyosarcoma

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https://www.readbyqxmd.com/read/28634755/rhabdomyosarcoma-of-the-lower-female-genital-tract-an-analysis-of-144-cases
#1
Dimitrios Nasioudis, Michail Alevizakos, Eloise Chapman-Davis, Steven S Witkin, Kevin Holcomb
PURPOSE: The aim of the present study was to elucidate the clinico-pathological characteristics of female patients with lower genital tract rhabdomyosarcoma (RMS) stratified by age group and investigate their prognosis, using a multi-institutional database. METHODS: The National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database was accessed (1973-2013) and a cohort of females diagnosed with RMS of the lower genital tract (vulva, vagina, cervix) was drawn...
June 20, 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/28620004/cancer-and-central-nervous-system-tumor-surveillance-in-pediatric-neurofibromatosis-1
#2
REVIEW
D Gareth R Evans, Hector Salvador, Vivian Y Chang, Ayelet Erez, Stephan D Voss, Kami Wolfe Schneider, Hamish S Scott, Sharon E Plon, Uri Tabori
Although the neurofibromatoses consist of at least three autosomal dominantly inherited disorders, neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2), and schwannomatosis, NF1 represents a multisystem pleiotropic condition very different from the other two. NF1 is a genetic syndrome first manifesting in childhood; affecting multiple organs, childhood development, and neurocognitive status; and presenting the clinician with often complex management decisions that require a multidisciplinary approach. Molecular genetic testing (see article for detailed discussion) is recommended to confirm NF1, particularly in children fulfilling only pigmentary features of the diagnostic criteria...
June 15, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28617441/concomitant-epigenetic-targeting-of-lsd1-and-hdac-synergistically-induces-mitochondrial-apoptosis-in-rhabdomyosarcoma-cells
#3
Tinka Haydn, Eric Metzger, Roland Schuele, Simone Fulda
The lysine-specific demethylase 1 (LSD1) is overexpressed in several cancers including rhabdomyosarcoma (RMS). However, little is yet known about whether or not LSD1 may serve as therapeutic target in RMS. We therefore investigated the potential of LSD1 inhibitors alone or in combination with other epigenetic modifiers such as histone deacetylase (HDAC) inhibitors. Here, we identify a synergistic interaction of LSD1 inhibitors (i.e., GSK690, Ex917) and HDAC inhibitors (i.e., JNJ-26481585, SAHA) to induce cell death in RMS cells...
June 15, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28616785/usefulness-of-nkx2-2-immunohistochemistry-for-distinguishing-ewing-sarcoma-from-other-sinonasal-small-round-blue-cell-tumors
#4
Austin McCuiston, Justin A Bishop
NKX2.2 is a new immunohistochemical marker that has been reported to be sensitive and specific for Ewing sarcoma (ES). It has not, however, been investigated specifically in the sinonasal small round blue cell tumor (SRBCT) differential diagnosis which includes many tumors specific to that site. It has also not been investigated in the newly recognized "adamantinoma-like" variant of ES. Immunohistochemistry for NKX2.2 was performed on 170 poorly differentiated sinonasal neoplasms: 73 squamous cell carcinomas (67 poorly differentiated, non-keratinizing, or basaloid types and 6 nasopharyngeal carcinomas), 46 olfactory neuroblastomas, 8 sinonasal undifferentiated carcinomas (SNUCs), 6 melanomas, 7 Ewing sarcomas, 6 SMARCB1-deficient carcinomas, 6 teratocarcinosarcomas, 5 alveolar rhabdomyosarcomas, 4 solid adenoid cystic carcinomas, 4 NK/T cell lymphomas, 3 NUT carcinomas, and 2 small cell carcinomas...
June 14, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28616573/yap-and-the-hippo-pathway-in-pediatric-cancer
#5
REVIEW
Atif A Ahmed, Abdalla D Mohamed, Melissa Gener, Weijie Li, Eugenio Taboada
The Hippo pathway is an important signaling pathway that controls cell proliferation and apoptosis. It is evolutionarily conserved in mammals and is stimulated by cell-cell contact, inhibiting cell proliferation in response to increased cell density. During early embryonic development, the Hippo signaling pathway regulates organ development and size, and its functions result in the coordinated balance between proliferation, apoptosis, and differentiation. Its principal effectors, YAP and TAZ, regulate signaling by the embryonic stem cells and determine cell fate and histogenesis...
2017: Molecular & Cellular Oncology
https://www.readbyqxmd.com/read/28616389/fibrosarcoma-of-the-eyelid-in-two-sibling-czech-wolfdogs
#6
Laura Nordio, Sabina Fattori, Chiara Giudice
Most canine tumors of the eyelid are tumors generally encountered in the skin. They are most commonly of epithelial origin and benign. In this report, we describe the cases of two sibling Czech wolfdogs presented, one year apart, with a subcutaneous mass involving the left eyelid. Both lesions were histologically consistent with a diagnosis of subcutaneous fibrosarcoma. Immunohistochemical analyses of the tumors revealed a mild positivity for vimentin and negativity for GFAP, desmin, αSMA, myoglobin, S100, PNL2 and calponin, excluding all differential diagnosis (i...
2017: Open veterinary journal
https://www.readbyqxmd.com/read/28615069/regulatory-landscape-fusion-in-rhabdomyosarcoma-through-interactions-between-the-pax3-promoter-and-foxo1-regulatory-elements
#7
Cristina Vicente-García, Barbara Villarejo-Balcells, Ibai Irastorza-Azcárate, Silvia Naranjo, Rafael D Acemel, Juan J Tena, Peter W J Rigby, Damien P Devos, Jose L Gómez-Skarmeta, Jaime J Carvajal
BACKGROUND: The organisation of vertebrate genomes into topologically associating domains (TADs) is believed to facilitate the regulation of the genes located within them. A remaining question is whether TAD organisation is achieved through the interactions of the regulatory elements within them or if these interactions are favoured by the pre-existence of TADs. If the latter is true, the fusion of two independent TADs should result in the rewiring of the transcriptional landscape and the generation of ectopic contacts...
June 14, 2017: Genome Biology
https://www.readbyqxmd.com/read/28614716/the-notch1-snail1-mef2c-pathway-regulates-growth-and-self-renewal-in-embryonal-rhabdomyosarcoma
#8
Myron S Ignatius, Madeline N Hayes, Riadh Lobbardi, Eleanor Y Chen, Karin M McCarthy, Prethish Sreenivas, Zainab Motala, Adam D Durbin, Aleksey Molodtsov, Sophia Reeder, Alexander Jin, Sivasish Sindiri, Brian C Beleyea, Deepak Bhere, Matthew S Alexander, Khalid Shah, Charles Keller, Corinne M Linardic, Petur G Nielsen, David Malkin, Javed Khan, David M Langenau
Tumor-propagating cells (TPCs) share self-renewal properties with normal stem cells and drive continued tumor growth. However, mechanisms regulating TPC self-renewal are largely unknown, especially in embryonal rhabdomyosarcoma (ERMS)-a common pediatric cancer of muscle. Here, we used a zebrafish transgenic model of ERMS to identify a role for intracellular NOTCH1 (ICN1) in increasing TPCs by 23-fold. ICN1 expanded TPCs by enabling the de-differentiation of zebrafish ERMS cells into self-renewing myf5+ TPCs, breaking the rigid differentiation hierarchies reported in normal muscle...
June 13, 2017: Cell Reports
https://www.readbyqxmd.com/read/28594087/adult-prostatic-sarcoma-a-contemporary-multicenter-rare-cancer-network-study
#9
Berardino De Bari, Bradley Stish, Mark Wayne Ball, Yacob Habboush, Paul Sargos, Marco Krengli, Alberto Bossi, Armando Stabile, Claudio Sole Pesutic, Laëtitia Lestrade, Robert Jan Smeenk, Barbara Alicja Jereczek-Fossa, Zilli Thomas, Gilles Créhange, Filippo Alongi, Nicholas Zaorsky, Mahmut Ozsahin
INTRODUCTION: Adult prostatic sarcoma (PS) is a rare disease. While surgery is considered the standard approach, the role of other therapies is not completely established. We report results of the largest multicentric contemporary cohort of PS patients. MATERIALS AND METHODS: This study included 61 adult PS patients treated in 16 American and European Institutions. Median age was 64.4 years (range: 22-87). Curative surgery was delivered in 48 patients (prostatectomy = 26, cystoprostatectomy = 22), usually with lymphadenectomy (n = 40)...
June 8, 2017: Prostate
https://www.readbyqxmd.com/read/28593107/minimally-invasive-approach-for-resection-of-parameningeal-rhabdomyosarcoma
#10
Aileen Wertz, Brittny N Tillman, Jennifer V Brinkmeier, Tiffany A Glazer, Andrew D Kroeker, Steven E Sullivan, Erin L McKean
Background  About one-third of rhabdomyosarcomas arise in the head and neck, with parameningeal primaries accounting for half of these. Principles of management involve chemotherapy, radiation, or both, in addition to surgical biopsy, debulking, and complete or near-complete resection. In the head and neck, diagnostic biopsies have historically been performed without attempt at resection due to proximity to critical structures and cosmetic considerations. Methods  Retrospective chart review of three cases of rhabdomyosarcoma at the cranial base managed through minimally invasive endoscopic surgical resection and adjuvant therapy...
June 2017: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/28590782/rhabdomyosarcoma-in-children-and-adolescents-patterns-and-risk-factors-of-distant-metastasis
#11
Jeong Rye Kim, Hee Mang Yoon, Kyung-Nam Koh, Ah Young Jung, Young Ah Cho, Jin Seong Lee
OBJECTIVE: The objective of this study is to evaluate patterns of distant metastasis and identified factors that may increase the risk of distant metastasis in pediatric patients with rhabdomyosarcoma. MATERIALS AND METHODS: This retrospective study included 69 patients (age, ≤ 20 years) who had rhabdomyosarcoma diagnosed between January 2000 and February 2016. Various imaging features, including distant metastasis, were evaluated on initial and follow-up imaging studies...
June 7, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28590507/roles-of-partitioning-defective-protein-6-par6-and-its-complexes-in-the-proliferation-migration-and-invasion-of-cancer-cells
#12
REVIEW
Lingling Ruan, Yanting Shen, Ziwen Lu, Dongsheng Shang, Zhicong Zhao, Yongjin Lu, Yanfang Wu, Yafei Zhang, Zhigang Tu, Hanqing Liu
A pivotal regulator of cell polarity and homeostasis, partitioning-defective protein 6 (Par6) forms multicomponent complexes that not only regulate cell polarity and stabilize cell morphology, but have also been demonstrated to participate in the proliferation, migration and invasion of cancer cells. The transforming growth factor (TGF)-β and extracellular signal-regulated kinase (Erk) 1/2 pathways are the most thoroughly studied pathways involving Par6 in many cancers. Aurothiomalate has been used to disrupt the interaction between Par6 and atypical protein kinase C within the multicomponent complexes, and has been shown to effectively block transformed growth and metastasis in vitro and/or in vivo in a variety of cancers, including pancreatic, prostate and lung cancers, as well as alveolar rhabdomyosarcoma...
June 7, 2017: Clinical and Experimental Pharmacology & Physiology
https://www.readbyqxmd.com/read/28589056/vaginal-ewing-sarcoma-an-uncommon-clinical-entity-in-pediatric-patients
#13
Nathan M Cross, A Luana Stanescu, Erin R Rudzinski, Douglas S Hawkins, Marguerite T Parisi
Ewing sarcoma, including classical Ewing sarcoma of the bone and primitive neuroectodermal tumors arising in bone or extraosseous primary sites, is a highly aggressive childhood neoplasm. We present two cases of Ewing sarcoma arising from the vagina in young girls. Previously reported cases in literature focused on their pathologic rather than radiographic features. We describe the spectrum of multimodality imaging appearances of Ewing sarcoma at this unusual primary site. Awareness of vaginal Ewing tumors may facilitate prompt diagnosis and lead to a different surgical approach than the more commonly encountered vaginal rhabdomyosarcoma...
2017: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/28586604/orbital-involvement-in-sinonasal-diseases
#14
Zia-Us-Salam Qazi, Sarfraz Latif, Sadia Maqsood Awan
OBJECTIVE: Orbital involvement in sinonasal diseases can present as proptosis, ophthalmoplegia or even as blindness due to optic nerve damage. There are a number of sinonasal diseases which can involve eyes. The purpose of this study was to enlist diagnoses of all the patients with sinonasal disease, in which orbit was also involved unilaterally or bilaterally and to analyse the management strategy and final outcome in all the cases. METHODS: Hundred consecutive patients having orbital symptoms along with sinonasal complaints that presented in ENT department of Shaikh Zayed federal postgraduate medical institute were included in our prospective study...
October 2016: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28583514/pediatric-sinonasal-malignancies-a-population-based-analysis
#15
Sei Y Chung, Aykut A Unsal, Suat Kılıç, Soly Baredes, James K Liu, Jean Anderson Eloy
INTRODUCTION: Pediatric Sinonasal Malignancies (PedsSNM) are rare and usually associated with a poor prognosis. We aim to investigate the epidemiology, tumor characteristics, and survival of PedsSNM using a population-based database to augment the scant literature on this topic. METHODS: The Surveillance, Epidemiology, and End Results database was queried for patients ≤18 years diagnosed with PedsSNM between the years of 1973 and 2013. Data on incidence, tumor characteristics, and survival were analyzed...
July 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28578184/rhabdomyosarcoma-of-the-vagina-in-an-adolescent-girl
#16
Geetha Narayanan, Varun Rajan, Ridu Kumar, Lali V Soman
BACKGROUND: Gynaecologic neoplasms are rare in children and represent only <5% of all childhood tumors. Rhabdomyosarcoma (RMS) of the female genital tract of children accounts for only 3.5% of the cases. CASE: A 16 year old adolescent presented with a proliferating growth and foul smelling discharge from vagina, which on biopsy was diagnosed as RMS. She received chemotherapy and radiation to the primary site. She is alive in remission at 8 years. SUMMARY: RMS of the vagina is a rare, but highly curable tumor in adolescent girls...
May 31, 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28576545/-18-f-fdg-pet-ct-in-the-staging-of-alveolar-rhabdomyosarcoma
#17
Alvaro de Bonilla-Damiá, Rosa Fernández-López, Victor M Pachón-Garrudo, Catalina Márquez-Vega, Isabel Borrego-Dorado
No abstract text is available yet for this article.
May 30, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28575066/combined-application-of-arsenic-trioxide-and-lithium-chloride-augments-viability-reduction-and-apoptosis-induction-in-human-rhabdomyosarcoma-cell-lines
#18
Sabine B Schleicher, Julian J Zaborski, Rosa Riester, Natascha Zenkner, Rupert Handgretinger, Torsten Kluba, Frank Traub, Karen A Boehme
Rhabdomyosarcomas (RMS) are the most prevalent soft tissue sarcomas affecting children and adolescents. Despite intensive treatment consisting of multimodal chemotherapy and surgery RMS patients diagnosed with metastatic disease expect long term survival rates of only 20%. Often multidrug resistance arises upon initial response emphasizing the need for new therapeutic drugs to improve treatment efficiency. Previously, we demonstrated the efficacy of the FDA approved drug arsenic trioxide (ATO) specifically inhibiting viability and clonal growth as well as inducing cell death in human RMS cell lines of different subtypes...
2017: PloS One
https://www.readbyqxmd.com/read/28566498/thermal-combination-therapies-for-local-drug-delivery-by-magnetic-resonance-guided-high-intensity-focused-ultrasound
#19
Nicole Hijnen, Esther Kneepkens, Mariska de Smet, Sander Langereis, Edwin Heijman, Holger Grüll
Several thermal-therapy strategies such as thermal ablation, hyperthermia-triggered drug delivery from temperature-sensitive liposomes (TSLs), and combinations of the above were investigated in a rhabdomyosarcoma rat tumor model (n = 113). Magnetic resonance-guided high-intensity focused ultrasound (MR-HIFU) was used as a noninvasive heating device with precise temperature control for image-guided drug delivery. For the latter, TSLs were prepared, coencapsulating doxorubicin (dox) and [Gd(HPDO3A)(H2O)], and injected in tumor-bearing rats before MR-HIFU treatment...
May 31, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28565843/inhibition-of-centrosomal-protein-164-sensitizes-rhabdomyosarcoma-cells-to-radiotherapy
#20
Jianzhou Liu, Zhiju Wang, Xiaofeng Li, Xu Zhang, Chaoji Zhang
Rhabdomyosarcoma is the second most common malignant tumor of the heart in infants and children and cannot often be resected completely. Chemotherapy and radiotherapy have a critical role in relieving symptoms and prolonging survival; therefore, enhancing the sensitivity of rhabdomyosarcoma to radiotherapy is an important area of investigation in order to improve the prognosis of patients. It has been reported that centrosomal protein 164 (CEP164) has a key role in the DNA damage-activated signaling cascade...
May 2017: Experimental and Therapeutic Medicine
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