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Adult rhabdomyosarcoma

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https://www.readbyqxmd.com/read/28905489/consensus-and-controversies-regarding-the-treatment-of-rhabdomyosarcoma
#1
REVIEW
Scott C Borinstein, Diana Steppan, Masanori Hayashi, David M Loeb, Michael S Isakoff, Odion Binitie, Andrew S Brohl, Julia A Bridge, Mark Stavas, Eric T Shinohara, William H Meyer, Damon R Reed, Lars M Wagner
Optimal treatment of rhabdomyosarcoma (RMS) requires multidisciplinary approach, incorporating chemotherapy with local control. Although current therapies are built on cooperative group trials, a comprehensive standard of care to guide clinical decision making has been lacking, especially for relapsed patients. Therefore, we assembled a panel of pediatric and adolescent and young adult sarcoma experts to develop treatment guidelines for managing RMS and to identify areas in which further research is needed...
September 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28875443/sinonasal-tract-alveolar-rhabdomyosarcoma-in-adults-a-clinicopathologic-and-immunophenotypic-study-of-fifty-two-cases-with-emphasis-on-epithelial-immunoreactivity
#2
Lester D R Thompson, Vickie Y Jo, Abbas Agaimy, Antonio Llombart-Bosch, Gema Nieto Morales, Isidro Machado, Uta Flucke, Paul E Wakely, Markku Miettinen, Justin A Bishop
Sinonasal tract (SNT) alveolar rhabdomyosarcoma (ARMS) are frequently misdiagnosed, especially in adults. Fifty-two adult (≥18 years) patients with SNT ARMS were reviewed and characterized by immunohistochemistry and molecular studies. Twenty-six females and 26 males (18-72 years; mean 43.2 years), presented after a short duration (mean 2.6 months) with a large (mean 5.5 cm) destructive nasal cavity mass, involving multiple contiguous paranasal sites (n = 46) and with cervical adenopathy (n = 41)...
September 5, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28868225/pleomorphic-rhabdomyosarcoma-infiltrating-thoracic-spine-in-a-59-year-old-female-patient-case-report
#3
Matthias Spalteholz, Jens Gulow
Rhabdomyosarcoma (RMS) represents a malignant tumor of skeletal muscle cells arising from rhabdomyoblasts. RMS represents the most common soft tissue sarcoma in children. In adults it is uncommon and accounts for less than 1% of all malignant solid tumors. While treatment protocols are well known for children, there is no standardized regimen in adults. This is one reason, why the outcome in adults is worse than in children. We present the case of a 59-year-old female patient with pleomorphic rhabdomyosarcoma (PRMS) infiltrating the thoracic spine...
2017: GMS Interdisciplinary Plastic and Reconstructive Surgery DGPW
https://www.readbyqxmd.com/read/28863036/clinical-activity-of-eribulin-in-advanced-desmoplastic-small-round-cell-tumor
#4
Sheik Emambux, Michele Kind, Francois Le Loarer, Maud Toulmonde, Eberhard Stoeckle, Antoine Italiano
Desmoplastic small round-cell tumor is a rare but highly aggressive tumor occurring mainly in adolescents and young adults. Prolonged progression-free survival has been documented in patients who have undergone aggressive multimodality therapy - that is, multiagent intensive chemotherapy, debulking surgery, and radiation therapy. Eribulin is a microtubule-dynamics inhibitor, and it has recently been shown to be active in liposarcomas. In preclinical models, eribulin activities have also been shown to occur in Ewing's sarcoma cell lines, rhabdomyosarcomas and osteosarcomas...
August 31, 2017: Anti-cancer Drugs
https://www.readbyqxmd.com/read/28811847/a-rare-case-of-adult-rhabdomyosarcoma
#5
Benjamin Sparreboom, Brendan Litton, Julian Yaxley
BACKGROUND: Rhabdomyosarcoma is a rare, soft tissue malignancy, diagnosed particularly in adults. It commonly metastasizes to the bone marrow. We present a rare case of an adult rhabdomyosarcoma which illustrates the importance of magnetic resonance imaging in identifying early changes in the internal bone structure. CASE REPORT: A 50-year-old male presented with acute urinary retention. The patient initially had a CT scan of the lumbar spine which only revealed a protrusion of the L5-S1 intervertebral disc and no apparent cause for the patient's symptoms...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28795902/rhabdomyosarcoma-of-the-breast-report-of-a-rare-malignancy
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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...
September 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
#17
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
#18
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
#19
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
#20
MULTICENTER STUDY
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
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