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Adult rhabdomyosarcoma

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https://www.readbyqxmd.com/read/28524158/sequencing-of-dicer1-in-sarcomas-identifies-biallelic-somatic-dicer1-mutations-in-an-adult-onset-embryonal-rhabdomyosarcoma
#1
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/28466407/rhabdomyosarcoma-spindle-cell-sclerosing-variant-a-clinical-and-histopathological-examination-of-this-rare-variant-with-three-new-cases-from-the-oral-cavity
#2
Molly Housley Smith, Daniel Atherton, John D Reith, Nadim M Islam, Indraneel Bhattacharyya, Donald M Cohen
Spindle cell/sclerosing rhabdomyosarcoma (S-ScRMS) was recently recognized in 2013 by the World Health Organization (WHO) as a stand-alone entity (Parham et al., WHO classification of tumours of soft tissue and bone, IARC Press, Lyon, 2013). Historically, the spindle cell and sclerosing variants were subcategorized under embryonal type rhabdomyosarcoma. Current data supports that certain S-ScRMS cases have a more aggressive clinical course with reduction of long-term survival, and those found in the head and neck region often exhibit extensive local recurrence...
May 2, 2017: Head and Neck Pathology
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/28427078/a-case-of-adult-metastatic-rhabdomyosarcoma-of-the-prostate-cured-by-long-term-chemotherapy-with-local-radiation
#4
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/28407197/autologous-hematopoietic-stem-cell-transplantation-following-high-dose-chemotherapy-for-nonrhabdomyosarcoma-soft-tissue-sarcomas
#5
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/28388581/temozolomide-combined-with-irinotecan-caused-regression-in-an-adult-pleomorphic-rhabdomyosarcoma-patient-derived-orthotopic-xenograft-pdox-nude-mouse-model
#6
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/28367175/analysis-of-orbital-malignancies-presenting-in-a-tertiary-care-hospital-in-pakistan
#7
Asad Aslam Khan, Suhail Sarwar, Mohammad Ali A Sadiq, Imran Ahmad, Nayab Tariq, Sibghat-Ul-Noor
OBJECTIVE: To determine the frequencies of various orbital malignancies amongst orbital lesions in patients presenting in a tertiary care hospital in Pakistan. METHODS: A retrospective analysis of 666 orbital cases with an established histopathological diagnosis of malignant tumors treated in Mayo Hospital Lahore from 1996 to 2015 (20 years). RESULTS: About 66% of the malignant tumors were primary, 25% secondary, 8% haematopoietic and 1% metastatic...
January 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28356215/successful-treatment-of-adult-pleomorphic-rhabdomyosarcoma-with-bone-invasion-in-the-lower-leg-by-chemotherapy-and-biological-reconstruction-a-case-report
#8
Atsushi Tanaka, Yasuo Yoshimura, Takashige Momose, Ken'ichi Isobe, Kaoru Aoki, Munehisa Kito, Masanori Okamoto, Shuichiro Suzuki, Kenji Sano, Hiroyuki Kato
No abstract text is available yet for this article.
March 26, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28273971/clinico-pathological-spectrum-of-ophthalmic-lesions-an-experience-in-tertiary-care-hospital-of-central-india
#9
Yashita Gupta, Renuka Gahine, Nighat Hussain, Mohd Jafar Memon
INTRODUCTION: Ophthalmic lesions show varied distribution on the basis of geographical location. Eye being a unique sensory organ needs to be studied both clinically and pathologically. AIM: This study was aimed to evaluate the histomorphological and clinico-pathlological spectrum of ophthalmic lesions at a tertiary care hospital of Central India. MATERIALS AND METHODS: We reviewed all the ophthalmic biopsies performed in the Department of Pathology of our institute between January 2008 and November 2014...
January 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28271871/soft-tissue-sarcomas-in-adolescents-and-young-adults-a-comparison-with-their-paediatric-and-adult-counterparts
#10
REVIEW
Winette T A van der Graaf, Daniel Orbach, Ian R Judson, Andrea Ferrari
Survival outcomes for adolescent and young adult patients with soft tissue sarcomas lag behind those of children diagnosed with histologically similar tumours. To help understand these differences in outcomes, we discuss the following issues with regard to the management of these patients with soft tissue sarcomas: delays in diagnosis, trial availability and participation, aspects of the organisation of care (with an emphasis on age-specific needs), national centralisation of sarcoma care, international consortia, and factors related to tumour biology...
March 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28243595/cns-metastases-from-bone-and-soft-tissue-sarcomas-in-children-adolescents-and-young-adults-are-they-really-so-rare
#11
Monika Bekiesinska-Figatowska, Agnieszka Duczkowska, Marek Duczkowski, Hanna Bragoszewska, Anna Romaniuk-Doroszewska, Beata Iwanowska, Sylwia Szkudlinska-Pawlak, Jaroslaw Madzik, Katarzyna Bilska, Anna Raciborska
Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas' metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas' CNS metastases. Methods. Patients with first diagnosis in 1999-2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI)/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28222777/identification-of-somatic-and-germ-line-dicer1-mutations-in-pleuropulmonary-blastoma-cystic-nephroma-and-rhabdomyosarcoma-tumors-within-a-dicer1-syndrome-pedigree
#12
Lorena Fernández-Martínez, José Antonio Villegas, Íñigo Santamaría, Ana S Pitiot, Marta G Alvarado, Soledad Fernández, Héctor Torres, Ángeles Paredes, Pilar Blay, Milagros Balbín
BACKGROUND: DICER1 syndrome is a pediatric cancer predisposition condition causing a variety of tumor types in children and young adults. In this report we studied a family with two relatives presenting a variety of neoplastic conditions at childhood. METHODS: Germ-line mutation screening of the complete coding region of the DICER1 gene in genomic DNA from the proband was performed. The presence of somatic DICER1 mutation and further alterations in driver genes was investigated in genomic DNA obtained from available tumor samples...
February 21, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28211476/novel-roles-for-staufen1-in-embryonal-and-alveolar-rhabdomyosarcoma-via-c-myc-dependent-and-independent-events
#13
Tara E Crawford Parks, Kristen A Marcellus, Jonathan Langill, Aymeric Ravel-Chapuis, Jean Michaud, Kyle N Cowan, Jocelyn Côté, Bernard J Jasmin
Rhabdomyosarcoma is the most common soft tissue sarcoma in children and young adults. Rhabdomyosarcomas are skeletal muscle-like tumours that typically arise in muscle beds, and express key myogenic regulatory factors. However, their developmental program remains blocked in the proliferative phase with cells unable to exit the cell cycle to fuse into myotubes. Recently, we uncovered a key role for the RNA-binding protein Staufen1 during myogenic differentiation through the regulation of c-myc translation. Given the known implication of c-myc in rhabdomyosarcoma, we hypothesized in the current work that Staufen1 controls rhabdomyosarcoma tumorigenesis...
February 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28194276/a-case-report-of-concurrent-embryonal-rhabdomyosarcoma-and-diffuse-large-b-cell-lymphoma-in-an-adult-without-identifiable-cancer-predisposition
#14
M D Mathias, M V Ortiz, H Magnan, S R Ambati, E K Slotkin, A J Chou, M F Walsh, K Offit, C Moskowitz, A Kentsis, L H Wexler
BACKGROUND: Diffuse large B-cell lymphoma (DLBCL) is the most common form of non-Hodgkin lymphoma. Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood. makes up less than 1% of solid malignancies in adults with around 400 new cases each year in the United States. They have not previously been reported concurrently. CASE PRESENTATION: A 37 year old woman presented with painful enlarging leg mass. Biopsy of the mass was consistent with embryonal rhabdomyosarcoma...
2017: Biomarker Research
https://www.readbyqxmd.com/read/28129857/discovering-novel-oncogenic-pathways-and-new-therapies-using-zebrafish-models-of-sarcoma
#15
M N Hayes, D M Langenau
Sarcoma is a type of cancer affecting connective, supportive, or soft tissue of mesenchymal origin. Despite rare incidence in adults (<1%), over 15% of pediatric cancers are sarcoma. Sadly, both adults and children with relapsed or metastatic disease have devastatingly high rates of mortality. Current treatment options for sarcoma include surgery, radiation, and/or chemotherapy; however, significant limitations exist with respect to the efficacy of these strategies. Strong impetus has been placed on the development of novel therapies and preclinical models for uncovering mechanisms involved in the development, progression, and therapy resistance of sarcoma...
2017: Methods in Cell Biology
https://www.readbyqxmd.com/read/28048825/su-f-t-50-evaluation-of-monte-carlo-simulations-performance-for-pediatric-brachytherapy-dosimetry
#16
C Chatzipapas, P Papadimitroulas, G Loudos, N Papanikolaou, G Kagadis
PURPOSE: Pediatric tumors are generally treated with multi-modal procedures. Brachytherapy can be used with pediatric tumors, especially given that in this patient population low toxicity on normal tissues is critical as is the suppression of the probability for late malignancies. Our goal is to validate the GATE toolkit on realistic brachytherapy applications, and evaluate brachytherapy plans on pediatrics for accurate dosimetry on sensitive and critical organs of interest. METHODS: The GATE Monte Carlo (MC) toolkit was used...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28039349/primary-embryonal-rhabdomyosarcoma-of-the-liver
#17
Aditi Arora, Ritika Jaiswal, Nidhi Anand, Nuzhat Husain
Rhabdomyosarcomas are malignant neoplasms with striated muscle differentiation. This is the most common type of soft-tissue sarcoma in children, but occurs rarely in adults. Its occurrence in liver is infrequent. We report a case of primary hepatic embryonal rhabdomyosarcoma in a 67-year-old man. The tumour was occupying the left lobe of the liver with large component of lesion seen bulging in left subhepatic space indenting over the stomach, compressing the pancreas and gall bladder. A percutaneous biopsy was performed which revealed embryonal rhabdomyosarcoma...
December 30, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/28035748/a-phase-i-study-of-perifosine-with-temsirolimus-for-recurrent-pediatric-solid-tumors
#18
Oren J Becher, Stephen W Gilheeney, Yasmin Khakoo, David C Lyden, Sofia Haque, Kevin C De Braganca, Jill M Kolesar, Jason T Huse, Shakeel Modak, Leonard H Wexler, Kim Kramer, Ivan Spasojevic, Ira J Dunkel
BACKGROUND: The PI3K/AKT/mTOR pathway is aberrantly activated in many pediatric solid tumors including gliomas and medulloblastomas. Preclinical data in a pediatric glioma model demonstrated that the combination of perifosine (AKT inhibitor) and temsirolimus (mTOR inhibitor) is more potent at inhibiting the axis than either agent alone. We conducted this study to assess pharmacokinetics and identify the maximum tolerated dose for the combination. PROCEDURE: We performed a standard 3+3 phase I, open-label, dose-escalation study in patients with recurrent/refractory pediatric solid tumors...
December 30, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28009608/fosb-is-a-useful-diagnostic-marker-for-pseudomyogenic-hemangioendothelioma
#19
Yin P Hung, Christopher D M Fletcher, Jason L Hornick
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a distinctive vascular neoplasm of intermediate biological potential with a predilection for young adults and frequent multifocal presentation. Pseudomyogenic hemangioendothelioma is characterized by loose fascicles of plump spindled and epithelioid cells with abundant eosinophilic cytoplasm and coexpression of keratins and endothelial markers. Recently, a SERPINE1-FOSB fusion has been identified as a consistent genetic alteration in pseudomyogenic hemangioendothelioma...
May 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27998313/embryonal-rhabdomyosarcoma-of-the-perineum-in-an-adult-a-case-report
#20
Sidy Ka, Freddy Gnangnon, Mamadou Moustapha Dieng, Doudou Diouf, Jaafar Thiam, Pape Macoumba Gaye, Ahmadou Dem
BACKGROUND: We report the case of an adult patient with embryonal rhabdomyosarcoma of the perineum admitted to our practice at Joliot Curie Institute in Dakar. It is a rare tumor at this age and has a bad prognosis at this localization. CASE PRESENTATION: We describe the case of a 22-year-old African man admitted for a perineal mass that had evolved over 6 months. He complained of tenesmus, obstinate constipation, and dysuria. A clinical examination revealed perineal swelling spread over his anus, scrotum, penis, testicles, and inguinal lymph nodes...
December 20, 2016: Journal of Medical Case Reports
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