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Adult rhabdomyosarcoma

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https://www.readbyqxmd.com/read/28795902/rhabdomyosarcoma-of-the-breast-report-of-a-rare-malignancy
#1
Yufen Yuan, Junna Hou, Yunbao Pan
Rhabdomyosarcoma (RMS) is an uncommon type of soft-tissue malignancy which mainly influences children. RMS rarely occurs in breast and little of the clinical behavior and treatment strategies were reported. Here, we describe a case of adult female patient with breast RMS. A 34-years-old Chinese woman visited to our hospital complaining palpable mass in her left breast. Seven months ago, the patient was diagnosed as lymphocytic mastitis and received surgical excision in the left breast. Five months later, she noticed a palpable mass again in the left breast...
August 10, 2017: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/28766068/pediatric-soft-tissue-sarcoma-of-the-limbs-clinical-outcome-of-97-patients
#2
Andrea Sambri, Giuseppe Bianchi, Ilaria Cucurnia, Marco Gambarotti, Davide Maria Donati
PURPOSE: Soft tissue sarcomas (STS) of the extremities in children are a heterogeneous group of tumors with a very different prognosis for which optimal treatment remains controversial. PATIENTS AND METHODS: We retrospectively evaluated 97 patients younger than 15 years old affected by limb soft tissue sarcomas. All cases were histologically revised, and tumor grade was assessed according to the FNLCC system. Thirty-two were rhabdomyosarcoma (RMS) and 65 non-rhabdomyosarcoma (NRMSTS); among these, 40 (61...
August 1, 2017: European Journal of Orthopaedic Surgery & Traumatology: Orthopédie Traumatologie
https://www.readbyqxmd.com/read/28762137/a-clinicopathologic-study-of-head-and-neck-malignant-peripheral-nerve-sheath-tumors
#3
Adepitan A Owosho, Cherry L Estilo, Joseph M Huryn, Ping Chi, Cristina R Antonescu
Head and neck high grade malignant peripheral nerve sheath tumors (HN-MPNSTs) are rare highly aggressive soft tissue sarcomas that show overlapping morphologic and immunophenotypic features with melanoma and other high grade sarcomas, resulting in diagnostic challenges, particularly in sporadic settings. Recent discoveries have implicated loss of function mutations in the polycomb repressive complex 2 (PRC2) components, including EED or SUZ12 genes, as one of the leading pathogenetic mechanisms in high grade MPNST...
July 31, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28756644/the-role-of-anaplastic-lymphoma-kinase-in-pediatric-cancers
#4
REVIEW
Junko Takita
The anaplastic lymphoma kinase (ALK) was initially identified as a fusion partner of the nucleophosmin gene in anaplastic large cell lymphoma (ALCL) with t(2;5)(p23;q35) translocation, and then described with different genetic abnormalities in a number of tumors. Although ALK is known to be involved in the pathogenesis of neuroblastoma through activating mutations or gene amplification, its role in the pathogenesis of other pediatric cancers is still elusive. In addition to neuroblastoma, the high-grade amplification of ALK has been described in a subset of rhabdomyosarcoma cases...
July 30, 2017: Cancer Science
https://www.readbyqxmd.com/read/28744074/multimodality-imaging-of-vaginal-rhabdomyosarcoma
#5
Richa S Chauhan, Dheeraj K Singh, Bishwarup Guha, Ishan Kumar, Ashish Verma
Rhabdomyosarcoma (RMS) is a malignant mesenchymal tumor arising from the embryonal muscle cells (rhabdomyoblasts), and is the most common soft tissue sarcoma in children and young adults accounting for 4-6% of all malignancies in this age group. Though rare overall, embryonal rhabdomyosarcoma is the most common malignancy arising in the pediatric female genitourinary tract with sarcoma botryoides being the most common variant of the tumor. In young and adolescent individuals, the cervix and uterus are affected; whereas in infants, vaginal lesions are more common...
April 2017: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/28722764/12-o-tetradecanoylphorbol-13-acetate-and-ezh2-inhibition-a-novel-approach-for-promoting-myogenic-differentiation-in-embryonal-rhabdomyosarcoma-cells
#6
Irene Marchesi, Luca Sanna, Milena Fais, Paolo Fiorentino Francesco, Antonio Giordano, Luigi Bagella
Rhabdomyosarcoma (RMS) is a soft tissue sarcoma that arises from muscle precursors affecting predominately children and young adults. It can be divided into two main classes: embryonal (eRMS) and alveolar rhabodomyosarcomas (aRMS). Despite the expression of early muscle specific genes, RMS cells fail to complete myogenesis even in differentiation conditions. We previously demonstrated that Enhancer Zeste of Homolog 2 (EZH2), the catalytic subunits of PRC2 complex, contributes to inhibit muscle differentiation in eRMS and its down-regulation causes a partial recovery of myogenesis...
July 19, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28697168/pretreatment-neutrophil-to-lymphocyte-ratio-and-lymphocyte-recovery-independent-prognostic-factors-for-survival-in-pediatric-sarcomas
#7
Liliana Vasquez, Esmeralda León, Brady Beltran, Ivan Maza, Monica Oscanoa, Jenny Geronimo
BACKGROUND: Pretreatment neutrophil-to-lymphocyte ratio (NLR) and absolute lymphocyte count (ALC) recovery have been shown to be associated with prognosis in several types of cancer in adults. However, evidence in pediatric cancer is scarce. The aim of our study was to evaluate whether pretreatment NLR and lymphocyte recovery are prognostic factors in pediatric sarcomas. MATERIALS AND METHODS: Study participants were identified from a retrospective cohort of 100 children with osteosarcoma (n=55), rhabdomyosarcoma (n=22), and Ewing sarcoma (n=23)...
July 10, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28696044/practice-patterns-of-palliative-radiation-therapy-in-pediatric-oncology-patients-in-an-international-pediatric-research-consortium
#8
Avani Dholakia Rao, Qinyu Chen, Ralph P Ermoian, Sara R Alcorn, Maria Luisa S Figueiredo, Michael J Chen, Karin Dieckmann, Shannon M MacDonald, Matthew M Ladra, Daria Kobyzeva, Alexey V Nechesnyuk, Kristina Nilsson, Eric C Ford, Brian A Winey, Rosangela C Villar, Stephanie A Terezakis
BACKGROUND/OBJECTIVES: The practice of palliative radiation therapy (RT) is based on extrapolation from adult literature. We evaluated patterns of pediatric palliative RT to describe regimens used to identify opportunity for future pediatric-specific clinical trials. DESIGN/METHODS: Six international institutions with pediatric expertise completed a 122-item survey evaluating patterns of palliative RT for patients ≤21 years old from 2010 to 2015. Two institutions use proton RT...
July 11, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28680211/an-infrequent-case-of-adult-alveolar-rhabdomyosarcoma-with-pancreatic-metastases-detected-in-f-18-fdg-pet-ct
#9
Aravintho Natarajan, Ameya Puranik, Nilendu Purandare, Archi Agrawal, Sneha Shah, Venkatesh Rangarajan
Rhabdomyosarcoma (RMS) is a rare cancer in adults, comprising about 1% of all forms of adult cancer. Common sites of metastases are lungs, skeletal system, lymph nodes, and brain. Metastases of RMS to pancreas are infrequent. We report a case of adult orbital alveolar RMS with pancreatic metastases detected in F-18-fluorodeoxyglucose-positron emission tomography/computed tomography.
July 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/28669309/outcomes-in-adult-sinonasal-rhabdomyosarcoma
#10
Katelyn Stepan, Neeraja Konuthula, Mohemmed Khan, Arjun Parasher, Anthony Del Signore, Satish Govindaraj, Eric Genden, Alfred Iloreta
Objective To describe patient demographics, histology, treatment modalities, and survival associated with adult sinonasal rhabdomyosarcoma. Study Design Retrospective review of a national database. Setting Tertiary medical center. Subjects and Methods Patient demographics, tumor histology, treatment modalities, and survival trends were examined for patients aged ≥18 years who were diagnosed with sinonasal rhabdomyosarcoma between 2004 and 2013 with the National Cancer Database. Kaplan-Meier analysis and the log-rank tests were performed to determine the unadjusted association between overall survival and various patient and disease characteristics...
July 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28643791/ewsr1-fusion-proteins-mediate-pax7-expression-in-ewing-sarcoma
#11
Gregory W Charville, Wei-Lien Wang, Davis R Ingram, Angshumoy Roy, Dafydd Thomas, Rajiv M Patel, Jason L Hornick, Matt van de Rijn, Alexander J Lazar
PAX7 is a paired-box transcription factor that is required for the developmental specification of adult skeletal muscle progenitors in mice. We previously demonstrated PAX7 expression as a marker of skeletal muscle differentiation in rhabdomyosarcoma. Here, using analyses of published whole-genome gene expression microarray data, we identify PAX7 as a gene with significantly increased expression in Ewing sarcoma in comparison to CIC-DUX4 round cell sarcoma. Analysis of PAX7 in a large cohort of 103 Ewing sarcoma cases by immunohistochemistry revealed expression in 99...
June 23, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28640941/phox2b-reliably-distinguishes-neuroblastoma-among-small-round-blue-cell-tumors
#12
Yin P Hung, John P Lee, Andrew M Bellizzi, Jason L Hornick
AIMS: Neuroblastoma shows considerable histologic overlap with other small round blue cell tumors. PHOX2B, a transcription factor essential for autonomic nervous system development, has been reported as an immunohistochemical marker for neuroblastoma. The purpose of this study was to validate the specificity and diagnostic utility of PHOX2B for peripheral neuroblastic tumors. METHODS AND RESULTS: We evaluated 240 cases (133 in whole-tissue sections; 107 in tissue microarrays), including 76 peripheral neuroblastic tumors [median age 2 years; including 4 adults] and 164 other tumors: 44 Wilms tumors; 20 Ewing sarcomas; 10 each CIC-rearranged round cell sarcomas, poorly differentiated synovial sarcomas, lymphoblastic lymphomas, alveolar rhabdomyosarcomas, embryonal rhabdomyosarcomas, mesenchymal chondrosarcomas, Merkel cell carcinomas, olfactory neuroblastomas, and melanomas; 5 each NUT midline carcinomas and desmoplastic small round cell tumors...
June 22, 2017: Histopathology
https://www.readbyqxmd.com/read/28634755/rhabdomyosarcoma-of-the-lower-female-genital-tract-an-analysis-of-144-cases
#13
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...
August 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/28616573/yap-and-the-hippo-pathway-in-pediatric-cancer
#14
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/28594087/adult-prostatic-sarcoma-a-contemporary-multicenter-rare-cancer-network-study
#15
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, Thomas Zilli, 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)...
July 2017: Prostate
https://www.readbyqxmd.com/read/28530261/a-rare-case-of-embryonal-rhabdomyosarcoma-of-the-parapharyngeal-space
#16
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/28524158/sequencing-of-dicer1-in-sarcomas-identifies-biallelic-somatic-dicer1-mutations-in-an-adult-onset-embryonal-rhabdomyosarcoma
#17
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)...
June 6, 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
#18
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
#19
Berkley E Gryder, Marielle E Yohe, Hsien-Chao Chou, Xiaohu Zhang, Joana Marques, Marco Wachtel, Beat Schaefer, Nirmalya Sen, Young 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. The mechanisms by which PAX3-FOXO1 dysregulates chromatin are unknown. We find PAX3-FOXO1 reprograms the cis-regulatory landscape by inducing de novo super enhancers. PAX3-FOXO1 uses super enhancers to set up autoregulatory loops in collaboration with the master transcription factors MYOG, MYOD, and MYCN. This myogenic super enhancer circuitry is consistent across cell lines and primary tumors...
August 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
#20
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
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