13,752 results match your criteria Rhabdomyosarcoma


Rhabdomyosarcoma disease spread evaluation on CT scans: Association with primary tumor size and Ki-67 proliferation marker.

Clin Imaging 2019 Feb 12;56:41-46. Epub 2019 Feb 12.

Tel Aviv University, Sackler Faculty of Medicine, Einstein St 68, Tel Aviv, Israel; Department of Diagnostic Imaging, the Chaim Sheba Medical Center at Tel HaShomer, Emek HaEla St 1, Ramat Gan, Israel.

Purpose: Ki-67 is a marker of cellular proliferation that is commonly used for the assessment of rhabdomyosarcoma. The aim of this study was to investigate the associations between Ki-67 expression and primary tumor diameter with CT evidence of lymph node and solid organ metastatic spread in rhabdomyosarcoma.

Materials And Methods: An institutional review board approval was granted for this study. Read More

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http://dx.doi.org/10.1016/j.clinimag.2019.02.009DOI Listing
February 2019

Immunotherapeutic Targeting of GPC3 in Pediatric Solid Embryonal Tumors.

Front Oncol 2019 26;9:108. Epub 2019 Feb 26.

Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, NY, United States.

Glypican 3 (GPC3) is a heparan sulfate proteoglycan and cell surface oncofetal protein which is highly expressed on a variety of pediatric solid embryonal tumors including the majority of hepatoblastomas, Wilms tumors, rhabdoid tumors, certain germ cell tumor subtypes, and a minority of rhabdomyosarcomas. Via both its core protein and heparan sulfate side chains, GPC3 activates the canonical Wnt/β-catenin pathway, which is frequently overexpressed in these malignancies. Loss of function mutations in lead to Simpson-Golabi-Behmel Syndrome, an X-linked overgrowth condition with a predisposition to GPC3-expressing cancers including hepatoblastoma and Wilms tumor. Read More

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http://dx.doi.org/10.3389/fonc.2019.00108DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6401603PMC
February 2019

Glypican-3 expression in malignant small round cell tumors.

Oncol Lett 2019 Mar 25;17(3):3523-3528. Epub 2019 Jan 25.

Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.

Malignant small round cell tumors usually progress rapidly and show resistance to chemotherapy, and it is often difficult to make a definitive diagnosis based on their histological morphology. Glypican-3 (GPC3) is a highly tumor-specific antigen, and the overexpression of GPC3 was reported in many pediatric and adult malignancies. In the present study, we investigated the GPC3 expression in pediatric malignant small round cell tumors to assess its role in the differential diagnosis of the tumors. Read More

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http://dx.doi.org/10.3892/ol.2019.9976DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6396161PMC

Craniofacial Approaches to Pediatric Orbital Tumors.

J Craniofac Surg 2019 Mar 6. Epub 2019 Mar 6.

Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.

Primary pediatric orbital tumors requiring surgery are uncommon and often require multidisciplinary management. Commonly used surgical approaches to the orbit include transconjunctival, transcutaneous (eyelid), transcranial, or extracranial osteotomies. This paper reviews a 10-year experience of cases that required a transcranial or extracranial surgical approach at the Birmingham Children's Hospital. Read More

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http://dx.doi.org/10.1097/SCS.0000000000005265DOI Listing

Primary alveolar rhabdomyosarcoma of retrorectal-presacral space in an adult patient: A case report of an uncommon tumor with rare presentation.

Medicine (Baltimore) 2019 Mar;98(10):e13416

Department of Radiology.

Rationale: Rhabdomyosarcoma (RMS) has known as a highly malignant soft tissue sarcoma, representing 5% to 10% of all solid tumors in childhood. Alveolar rhabdomyosarcoma (ARMS) of the retrorectal-presacral space is an extremely rare lesion for adult, no study has been reported in the English literature.

Patient Concerns: A 51-year-old male presented with abdominal pain for 1 month, significantly worse when having a bowel movement. Read More

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http://dx.doi.org/10.1097/MD.0000000000013416DOI Listing

The anti-tumor growth effect of a novel agent DMAMCL in rhabdomyosarcoma in vitro and in vivo.

J Exp Clin Cancer Res 2019 Mar 8;38(1):118. Epub 2019 Mar 8.

Liaoning Key Laboratory of Research and Application of Animal Models for Environmental and Metabolic Diseases, Medical Research Center, Shengjing Hospital of China Medical University, Shenyang, 110004, China.

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children with poor survival. New treatment approaches are urgently needed to improve treatment efficacy in RMS patients. DMAMCL is a novel agent from Asteraceae family that has been tested in phase I clinical trials in adult glioma in Australia. Read More

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http://dx.doi.org/10.1186/s13046-019-1107-1DOI Listing

Prognostic Factors and Treatment Outcomes of Adult Patients With Rhabdomyosarcoma After Multimodality Treatment.

Anticancer Res 2019 Mar;39(3):1355-1364

Division of Radiation Oncology, Department of Oncology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan, R.O.C.

Background: Adults with rhabdomyosarcoma (RMS) have a worse clinical outcome compared to pediatric cases. In the present study, the failure pattern and clinical outcome of adult patients with RMS who received multimodality treatment at our Institution was assessed.

Patients And Methods: Data were retrospectively recorded and analyzed from 20 adult patients, aged 19 years or more, who were treated for RMS at our Institution between 2004 and 2015. Read More

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http://dx.doi.org/10.21873/anticanres.13249DOI Listing

Epigenetic regulation of the PTEN-AKT-RAC1 axis by G9a is critical for tumor growth in alveolar rhabdomyosarcoma.

Cancer Res 2019 Mar 4. Epub 2019 Mar 4.

Physiology, National University of Singapore

Alveolar Rhabdomyosarcoma (ARMS) is an aggressive pediatric cancer with poor prognosis. As transient and stable modifications to chromatin have emerged as critical mechanisms in oncogenic signaling, efforts to target epigenetic modifiers as a therapeutic strategy have accelerated in recent years. To identify chromatin modifiers that sustain tumor growth, we performed an epigenetic screen and found that inhibition of lysine methyltransferase G9a significantly impacted the viability of ARMS cell lines. Read More

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http://dx.doi.org/10.1158/0008-5472.CAN-18-2676DOI Listing

Stage at diagnosis for childhood solid cancers in Australia: A population-based study.

Cancer Epidemiol 2019 Mar 1;59:208-214. Epub 2019 Mar 1.

Cancer Council Queensland, Brisbane, Australia; Menzies Health Institute Queensland, Griffith University, Gold Coast, Australia; Institute for Resilient Regions, University of Southern Queensland, Brisbane, Australia; School of Public Health, University of Queensland, Brisbane, Australia. Electronic address:

Background: Stage of cancer at diagnosis is one of the strongest predictors of survival and is essential for population cancer surveillance, comparison of cancer outcomes and to guide national cancer control strategies. Our aim was to describe, for the first time, the distribution of cases by stage at diagnosis and differences in stage-specific survival on a population basis for a range of childhood solid cancers in Australia.

Methods: The study cohort was drawn from the population-based Australian Childhood Cancer Registry and comprised children (<15 years) diagnosed with one of 12 solid malignancies between 2006 and 2014. Read More

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http://dx.doi.org/10.1016/j.canep.2019.02.013DOI Listing
March 2019
2.558 Impact Factor

Anacolosins A-F and Corymbulosins X and Y, Clerodane Diterpenes from Anacolosa clarkii Exhibiting Cytotoxicity toward Pediatric Cancer Cell Lines.

J Nat Prod 2019 Mar 4. Epub 2019 Mar 4.

Natural Products Branch, Developmental Therapeutics Program, Division of Cancer Treatment and Diagnosis , National Cancer Institute , Frederick , Maryland 21702 , United States.

An extract of the plant Anacolosa clarkii was obtained from the NCI Natural Products Repository, and it showed cytotoxic activity toward several types of pediatric solid tumor cell lines. Bioassay-guided fractionation led to the purification of eight new clerodane diterpenes [anacolosins A-F (1-6) and corymbulosins X and Y (7 and 8)] and two known compounds (9 and 10) that contained an isozuelanin skeleton. The structures of the new natural products were determined using 1D and 2D NMR and HRESIMS data, while the relative and absolute configurations of the compounds were assessed using a combination of H NMR coupling constant data, ROESY experiments, ECD (electronic circular dichroism) and VCD (vibrational circular dichroism) spectroscopy, chemical methods (including Mosher and 2-naphthacyl esterification), and chiral HPLC analyses. Read More

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http://pubs.acs.org/doi/10.1021/acs.jnatprod.8b01015
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http://dx.doi.org/10.1021/acs.jnatprod.8b01015DOI Listing
March 2019
1 Read

Sarcomas of the paranasal sinuses: An analysis of the SEER database.

Laryngoscope Investig Otolaryngol 2019 Feb 15;4(1):70-75. Epub 2019 Jan 15.

Georgetown University School of Medicine Washington DC.

Objective: To determine the demographics, treatment modalities, and overall survival of patients with sinonasal sarcoma.

Methods: All cases of primary sinonasal sarcomas diagnosed between the years of 2009 and 2014 were extracted from the National Cancer Institute's Surveillance, Epidemiology, and End Results database. Additional variables collected included age, gender, ethnicity, tumor histological subtype, tumor size, treatment modality, vitality status, and follow-up time. Read More

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http://dx.doi.org/10.1002/lio2.245DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6383302PMC
February 2019

Glutamine synthetase is necessary for sarcoma adaptation to glutamine deprivation and tumor growth.

Oncogenesis 2019 Feb 26;8(3):20. Epub 2019 Feb 26.

Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.

Despite a growing body of knowledge about the genomic landscape and molecular pathogenesis of sarcomas, translation of basic discoveries into targeted therapies and significant clinical gains has remained elusive. Renewed interest in altered metabolic properties of cancer cells has led to an exploration of targeting metabolic dependencies as a novel therapeutic strategy. In this study, we have characterized the dependency of human pediatric sarcoma cells on key metabolic substrates and identified a mechanism of adaptation to metabolic stress by examining proliferation and bioenergetic properties of rhabdomyosarcoma and Ewing sarcoma cells under varying concentrations of glucose and glutamine. Read More

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http://dx.doi.org/10.1038/s41389-019-0129-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6391386PMC
February 2019

A rare resected case of pulmonary rhabdomyosarcoma.

Gen Thorac Cardiovasc Surg 2019 Feb 26. Epub 2019 Feb 26.

Division of Chest Surgery, Hyogo Cancer Center, 13-70, Kitaoji-cho, Akashi, Japan.

Rhabdomyosarcoma is a well-known neoplasm in children that frequently occurs in the extremities, the head and neck region, and the genitourinary tract. To the best of our knowledge, pulmonary primary rhabdomyosarcoma in adults is exceedingly rare, and few resected cases have been reported. We report a case of pulmonary primary rhabdomyosarcoma that was surgically resected then treated with adjuvant chemotherapy (vincristine, actinomycin-D and cyclophosphamide). Read More

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http://dx.doi.org/10.1007/s11748-019-01086-7DOI Listing
February 2019

Aggressive embryonal rhabdomyosarcoma in a 3-month-old boy: A clinical and molecular analysis.

Pediatr Hematol Oncol 2019 Feb 26:1-8. Epub 2019 Feb 26.

a Division of Haematology/Oncology, The Hospital for Sick Children, Department of Pediatrics , University of Toronto , Toronto , ON , Canada.

Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma in the pediatric age group. While RMS has been traditionally classified on the basis of its histological appearance (with embryonal and alveolar being most common), it is now clear that the PAX-FOXO1 fusion product drives prognosis. We report here a case of pelvic embryonal RMS in a 3-month-old male who was subsequently found to have developed brain metastases during the course of chemotherapy. Read More

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http://dx.doi.org/10.1080/08880018.2019.1569185DOI Listing
February 2019
5 Reads

Embryonal rhabdomyosarcoma in a patient with a germline CBL pathogenic variant.

Cancer Genet 2019 Feb 30;231-232:62-66. Epub 2018 Dec 30.

Department of Pathology & Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA; Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.

Germline pathogenic variants in CBL are associated with an autosomal dominant RASopathy and an increased risk for malignancies, particularly juvenile myelomonocytic leukemia. Herein, we describe a patient with clinical features of a Noonan-spectrum disorder who developed embryonal rhabdomyosarcoma of the bladder at age two years. Tumor analysis using the OncoKids cancer panel revealed a CBL pathogenic variant: NM_005188. Read More

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http://dx.doi.org/10.1016/j.cancergen.2018.12.006DOI Listing
February 2019
1 Read

New human chromosomal sites with 'safe harbor' potential for targeted transgene insertion.

Hum Gene Ther 2019 Feb 22. Epub 2019 Feb 22.

University of Washington, Departments of Pathology & Genome Sciences , Box 357705 , 1959 N.E. Pacific Street Room K072C , Seattle, Washington, United States , 98195-7705 ;

We have identified 35 new sites for targeted transgene insertion that have the potential to serve as new human genomic 'safe harbor' sites. 'Safe harbor' site potential for these 35 sites, located on 16 chromosomes including both arms of the human X chromosome, and for the existing human 'safe harbor' sites AAVS1, hROSA26 and CCR5 was assessed using 8 different desirable, widely accepted criteria for 'safe harbor' sites verifiable with human genomic data. Three representative newly identified sites on human chromosomes 2 and 4 were then experimentally validated by in vitro and in vivo cleavage-sensitivity tests, and analyzed for population-level and cell line-specific sequence variants that might confound site targeting. Read More

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http://dx.doi.org/10.1089/hum.2018.169DOI Listing
February 2019
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Spindle cell liposarcoma with a TRIO-TERT fusion transcript.

Virchows Arch 2019 Feb 22. Epub 2019 Feb 22.

Massachusetts General Hospital, Department of Pathology, Harvard Medical School, Warren 2, 55 Fruit Street, Boston, MA, 02214, USA.

Conventional well-differentiated, dedifferentiated, and myxoid liposarcomas have long been known to harbor numerous typical genetic alterations that allow for diagnosis of these tumors. These include MDM2 and CDK4 amplification in well-differentiated and dedifferentiated liposarcomas as well as FUS-DDIT3 rearrangements in myxoid liposarcoma. More recently, in-frame TRIO-TERT fusion genes have been described in a subset of non-translocation-related sarcomas including myxofibrosarcoma, dedifferentiated liposarcoma, undifferentiated pleomorphic sarcoma, pleomorphic rhabdomyosarcoma, and leiomyosarcoma. Read More

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http://dx.doi.org/10.1007/s00428-019-02545-5DOI Listing
February 2019

Whole-body dose equivalent including neutrons is similar for 6 MV and 15 MV IMRT, VMAT, and 3D conformal radiotherapy.

J Appl Clin Med Phys 2019 Mar 21;20(3):56-70. Epub 2019 Feb 21.

Department of Physics, University of Zurich, Zurich, Switzerland.

Purpose: This study investigates the difference in whole-body dose equivalent between 6 and 15 MV image-guided radiotherapy (IGRT) for the treatment of a rhabdomyosarcoma in the prostate.

Methods: A previously developed model for stray radiation of the primary beam was improved and used to calculate the photon dose and photon energy in the out-of-field region for a radiotherapy patient. The dose calculated by the treatment planning system was fused with the model-calculated out-of-field dose, resulting in a whole-body photon dose distribution. Read More

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http://dx.doi.org/10.1002/acm2.12543DOI Listing

Aggressive orbital rhabdomyosarcoma in adulthood: A case report in a public hospital in Damascus, Syria.

Avicenna J Med 2019 Jan-Mar;9(1):38

Department of Pediatrics, Al-Kindy College of Medicine, University of Baghdad, Baghdad, Iraq.

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http://dx.doi.org/10.4103/ajm.AJM_137_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335876PMC
February 2019

Sinusoidal obstruction syndrome in a paediatric patient with acute lymphoblastic leukaemia after completion of reinduction therapy according to ALL Intercontinental Berlin-Frankfurt-Münster 2009.

Contemp Oncol (Pozn) 2018 31;22(4):266-269. Epub 2018 Dec 31.

Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland.

Sinusoidal obstruction syndrome (SOS), also termed veno-occlusive disease (VOD) of the liver, is a well-known complication of haematopoietic stem cell transplantation (HSCT) both in children and adults. In the medical literature there are occasional reports of SOS in patients receiving conventional chemotherapy. In children with solid tumours this entity occurs during treatment of nephroblastoma, rhabdomyosarcoma, and medulloblastoma. Read More

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http://dx.doi.org/10.5114/wo.2018.82646DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6377418PMC
December 2018
1 Read

AMORE treatment as salvage treatment in children and young adults with relapsed head-neck rhabdomyosarcoma.

Radiother Oncol 2019 Feb 17;131:21-26. Epub 2018 Dec 17.

Princess Máxima Centre for Paediatric Oncology, Utrecht, The Netherlands; Department of Paediatric Oncology, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, The Netherlands. Electronic address:

Background And Purpose: Survival after relapse of head and neck rhabdomyosarcoma (HNRMS) after prior external beam radiotherapy (EBRT) is poor, since options for adequate local treatment are often lacking. In this study we describe our experience with salvage AMORE in patients with relapsed HNRMS after prior EBRT.

Materials And Methods: Patients with relapsed HNRMS after prior EBRT in which salvage AMORE treatment was considered feasible were analysed; this includes patients with parameningeal, head and neck non-parameningeal and orbital localization. Read More

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http://dx.doi.org/10.1016/j.radonc.2018.10.036DOI Listing
February 2019

Caveolin-1 enhances metastasis formation in a human model of embryonal rhabdomyosarcoma through Erk signaling cooperation.

Cancer Lett 2019 May 13;449:135-144. Epub 2019 Feb 13.

Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy. Electronic address:

Rhabdomyosarcoma (RMS) is a pediatric soft tissue tumor classified in two major subtypes namely embryonal and alveolar, which have distinctive histopathological and genetic signatures and worse outcomes in the presence of metastases. Here, in order to evaluate the role of Caveolin-1 (Cav-1) in embryonal RMS dissemination, we employed an experimental in vivo metastasis assay using immunodeficient NOD/SCID mice. We found that the intravenous injection of human RD cells engineered for Cav-1 overexpression promoted the formation of lung metastases compared to parental cells. Read More

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http://dx.doi.org/10.1016/j.canlet.2019.02.013DOI Listing
May 2019
1 Read

Multifocal Synchronous Upper Urinary Tract Carcinosarcoma (Sarcomatoid Carcinoma) With Rhabdomyoblastic Differentiation.

Int J Surg Pathol 2019 Feb 15:1066896919828111. Epub 2019 Feb 15.

1 Department of Urology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan.

Carcinosarcoma of the upper urinary tract is very rare. In this article, we report a case of upper urinary tract carcinosarcoma with rhabdomyoblastic differentiation showing distinct transition between the epithelial and mesenchymal components confirmed by morphology and immunohistochemistry. An 81-year-old female underwent radical nephroureterectomy under the diagnosis of left ureteral urothelial carcinoma (UC). Read More

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http://dx.doi.org/10.1177/1066896919828111DOI Listing
February 2019
1 Read

Characteristics of mismatch repair deficiency in sarcomas.

Mod Pathol 2019 Feb 14. Epub 2019 Feb 14.

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

Due to the efficacy of immune checkpoint inhibitor therapy in tumors with deficient mismatch repair, there has been a surge in demand for mismatch repair deficiency testing in various tumor types. Mismatch repair deficiency is not known to play a significant role in the pathogenesis of sarcomas, and the utility of testing these tumor types is not established. This study aimed to determine the frequency, pattern, and clinicopathologic correlates of mismatch repair deficiency in sarcomas. Read More

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http://www.nature.com/articles/s41379-019-0202-3
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http://dx.doi.org/10.1038/s41379-019-0202-3DOI Listing
February 2019
2 Reads

Rhabdomyosarcoma diagnosed in the first year of life: Localized, metastatic, and relapsed disease. Outcome data from five trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS).

Pediatr Blood Cancer 2019 Feb 14:e27652. Epub 2019 Feb 14.

Department for Children and Adolescents, University Hospital of Frankfurt, Frankfurt/M., Germany.

Background: Rhabdomyosarcoma (RMS) diagnosed during the first year of life is reported to have poor outcome. Little is known about treatment and outcome data of relapsed disease (RD).

Methods: Characteristics, treatment, and outcome of 155 patients ≤ 12 months registered within the Cooperative Weichteilsarkom Studiengruppe (CWS) between 1981 and 2016 were evaluated. Read More

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http://doi.wiley.com/10.1002/pbc.27652
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http://dx.doi.org/10.1002/pbc.27652DOI Listing
February 2019
7 Reads

Getting control during follow-up visits: the views and experiences of parents on tumor surveillance after their children have completed therapy for rhabdomyosarcoma or Ewing sarcoma.

Support Care Cancer 2019 Feb 12. Epub 2019 Feb 12.

Princess Máxima Center for Paediatric Oncology, Lundlaan 6, 3584 CS, Utrecht, The Netherlands.

Purpose: Patients treated for rhabdomyosarcoma (RMS) or Ewing sarcoma (ES) are subject to extensive follow-up after completion of therapy. The aim of this follow-up is to monitor treatment side effects and to detect relapse in an early phase to improve prognosis after relapse. Little is known about parental emotional experiences during this period. Read More

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http://dx.doi.org/10.1007/s00520-019-04678-4DOI Listing
February 2019

Cervical rhabdomyosarcoma in an endocervical polyp of a 50 year old patient with intermenstrual bleeding.

Gynecol Oncol Rep 2019 May 26;28:6-8. Epub 2019 Jan 26.

Department of Obstetrics and Gynecology, State University of New York Downstate Medical Center, 450 Clarkson Ave Box 24, Brooklyn, NY 11203, United States.

•Cervical rhabdomyosarcoma does not always present as a cervical mass with vesicles.•Treatment regimens studied in the pediatric population can be used in adults with rhabdomyosarcoma.•More data is needed on outcomes of adult patients treated for rhabdomyosarcoma. Read More

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http://dx.doi.org/10.1016/j.gore.2019.01.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357501PMC

Early detection of the PAX3-FOXO1 fusion gene in circulating tumor-derived DNA in a case of alveolar rhabdomyosarcoma.

Genes Chromosomes Cancer 2019 Feb 10. Epub 2019 Feb 10.

Department of Pediatrics, Ehime University Graduate School of Medicine, Toon, Ehime, Japan.

Cell-free DNA (cfDNA), which are small DNA fragments in blood derived from dead cells including tumor cells, could serve as useful biomarkers and provide valuable genetic information about the tumors. cfDNA is now used for the genetic analysis of several types of cancers, as a surrogate for tumor biopsy, designated as "liquid biopsy." Rhabdomyosarcoma (RMS), the most frequent soft tissue tumor in childhood, can arise in any part of the body, and radiological imaging is the only available method for estimating the tumor burden, because no useful specific biological markers are present in the blood. Read More

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http://doi.wiley.com/10.1002/gcc.22734
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http://dx.doi.org/10.1002/gcc.22734DOI Listing
February 2019
7 Reads

Immune targeting of autocrine IGF2 hampers rhabdomyosarcoma growth and metastasis.

BMC Cancer 2019 Feb 7;19(1):126. Epub 2019 Feb 7.

Laboratory of Immunology and Biology of Metastasis, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Viale Filopanti 22, I-40126, Bologna, Italy.

Background: Insulin-like Growth Factor Receptor-1 (IGF1R) system sustains the genesis of rhabdomyosarcoma through IGF2 autocrine overexpression. While several IGF1R-targeted strategies have been investigated to interphere with rhabdomyosarcoma growth, no attempt to neutralize IGF2 has been reported. We therefore studied the possibility to hamper rhabdomyosarcoma growth with passive and active immune approaches targeting IGF2. Read More

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https://bmccancer.biomedcentral.com/articles/10.1186/s12885-
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http://dx.doi.org/10.1186/s12885-019-5339-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367747PMC
February 2019
4 Reads

Expanding the Spectrum of Intraosseous Rhabdomyosarcoma: Correlation Between 2 Distinct Gene Fusions and Phenotype.

Am J Surg Pathol 2019 Feb 1. Epub 2019 Feb 1.

Departments of Pathology.

Primary intraosseous rhabdomyosarcomas (RMSs) are extremely rare. Recently 2 studies reported 4 cases of primary intraosseous RMS with EWSR1/FUS-TFCP2 gene fusions, associated with somewhat conflicting histologic features, ranging from spindle to epithelioid. In this study we sought to further investigate the pathologic and molecular abnormalities of a larger group of intraosseous RMSs by a combined approach using targeted RNA sequencing analysis and fluorescence in situ hybridization (FISH). Read More

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http://dx.doi.org/10.1097/PAS.0000000000001227DOI Listing
February 2019
2 Reads
5.145 Impact Factor

[Erratum] OBP‑801, a novel histone deacetylase inhibitor, induces M‑phase arrest and apoptosis in rhabdomyosarcoma cells.

Oncol Rep 2019 Feb 1. Epub 2019 Feb 1.

Department of Pediatrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto 602‑8566, Japan.

Subsequent to the publication of the above article, the authors have realized that errors were introduced into Fig. 4 at the typesetting stage. Essentially, in Fig. Read More

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http://dx.doi.org/10.3892/or.2019.6997DOI Listing
February 2019

The Possible Role of Complete Loss of Myostatin in Limiting Excessive Proliferation of Muscle Cells (C2C12) via Activation of MicroRNAs.

Int J Mol Sci 2019 Feb 2;20(3). Epub 2019 Feb 2.

Jilin Provincial Key Laboratory of Animal Embryo Engineering, College of Animal Sciences, Jilin University, Changchun 130062, China.

Myostatin (MSTN) is a member of the TGF-β superfamily that negatively regulates skeletal muscle growth and differentiation. However, the mechanism by which complete MSTN deletion limits excessive proliferation of muscle cells remains unclear. In this study, we knocked out MSTN in mouse myoblast lines using a Clustered Regularly Interspaced Short Palindromic Repeats (CRISPR/Cas9) system and sequenced the mRNA and miRNA transcriptomes. Read More

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http://dx.doi.org/10.3390/ijms20030643DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6386905PMC
February 2019

Genetics, epigenetics and redox homeostasis in rhabdomyosarcoma: Emerging targets and therapeutics.

Redox Biol 2019 Jan 25:101124. Epub 2019 Jan 25.

Department of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 117593, Singapore. Electronic address:

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma accounting for 5-8% of malignant tumours in children and adolescents. Children with high risk disease have poor prognosis. Anti-RMS therapies include surgery, radiation and combination chemotherapy. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22132317183114
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http://dx.doi.org/10.1016/j.redox.2019.101124DOI Listing
January 2019
3 Reads

Pleomorphic Sarcomas: The State of the Art.

Surg Pathol Clin 2019 Mar 17;12(1):63-105. Epub 2018 Dec 17.

Department of Pathology, Hospital de Braga, Sete Fontes-Sao Victor, 4710-243 Braga, Portugal; University of Bordeaux, Talence, France. Electronic address:

This article focuses on pleomorphic sarcomas, which are malignant mesenchymal tumors with complex genetic background at the root of their morphologic pleomorphism. They are poorly differentiated tumors that may retain different lines of differentiation, sometimes correlating with clinicopathological or prognostic features. Accurate diagnosis in this group of tumors relies on adequate sampling due to their heterogeneity and assessment with both microscopy and large panels of immunohistochemistry. Read More

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http://dx.doi.org/10.1016/j.path.2018.10.004DOI Listing
March 2019
1 Read

Update on Myogenic Sarcomas.

Surg Pathol Clin 2019 Mar 20;12(1):51-62. Epub 2018 Dec 20.

Department of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA. Electronic address:

Myogenic sarcomas include soft tissue sarcomas that show skeletal muscle differentiation (rhabdomyosarcoma) and those with smooth muscle differentiation (leiomyosarcoma). Rhabdomyosarcomas are more common in the pediatric age group and leiomyosarcomas occur more often in the adult population. Based on the clinico-pathologic features and genetic abnormalities identified, the rhabdomyosarcomas are classified into embryonal, alveolar, spindle cell/sclerosing, and pleomorphic subtypes. Read More

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http://dx.doi.org/10.1016/j.path.2018.10.003DOI Listing

Integration of genomic copy number variations and chemotherapy-response biomarkers in pediatric sarcoma.

BMC Med Genomics 2019 Jan 31;12(Suppl 1):23. Epub 2019 Jan 31.

Herman B Wells Center for Pediatric Research, Department of Pediatrics, School of Medicine, Indiana University, Indianapolis, IN, 46202, USA.

Background: While most pediatric sarcomas respond to front-line therapy, some bone sarcomas do not show radiographic response like soft-tissue sarcomas (rhabdomyosarccomas) but do show 90% necrosis. Though, new therapies are urgently needed to improve survival and quality of life in pediatric patients with sarcomas. Complex chromosomal aberrations such as amplifications and deletions of DNA sequences are frequently observed in pediatric sarcomas. Read More

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http://dx.doi.org/10.1186/s12920-018-0456-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357363PMC
January 2019
2 Reads
2.873 Impact Factor

Indeterminate Pulmonary Nodules at Diagnosis in Rhabdomyosarcoma: Are They Clinically Significant? A Report From the European Paediatric Soft Tissue Sarcoma Study Group.

J Clin Oncol 2019 Mar 31;37(9):723-730. Epub 2019 Jan 31.

1 University of Amsterdam, Amsterdam, the Netherlands.

Purpose: To evaluate the clinical significance of indeterminate pulmonary nodules at diagnosis (defined as ≤ 4 pulmonary nodules < 5 mm or 1 nodule measuring ≥ 5 and < 10 mm) in patients with pediatric rhabdomyosarcoma (RMS).

Patients And Methods: We selected patients with supposed nonmetastatic RMS treated in large pediatric oncology centers in the United Kingdom, France, Italy, and the Netherlands, who were enrolled in the European Soft Tissue Sarcoma Study Group (E pSSG) RMS 2005 study. Patients included in the current study received a diagnosis between September 2005 and December 2013, and had chest computed tomography scans available for review that were done at time of diagnosis. Read More

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http://dx.doi.org/10.1200/JCO.18.01535DOI Listing
March 2019
18.428 Impact Factor

NRF2 orchestrates the redox regulation induced by radiation therapy, sustaining embryonal and alveolar rhabdomyosarcoma cells radioresistance.

J Cancer Res Clin Oncol 2019 Jan 30. Epub 2019 Jan 30.

Unit of Radiation Oncology, Azienda Ospedaliera Universitaria Senese, Siena, Italy.

Purpose: Tumor cells generally exhibit higher levels of reactive oxygen species (ROS), however, when stressed, tumor cells can undergo a process of 'Redox Resetting' to acquire a new redox balance with stronger antioxidant systems that enable cancer cells to become resistant to radiation therapy (RT). Here, we describe how RT affects the oxidant/antioxidant balance in human embryonal (RD) and alveolar (RH30) rhabdomyosarcoma (RMS) cell lines, investigating on the molecular mechanisms involved.

Methods: Radiations were delivered using an x-6 MV photon linear accelerator and their effects were assessed by vitality and clonogenic assays. Read More

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http://dx.doi.org/10.1007/s00432-019-02851-0DOI Listing
January 2019
1 Read

The palladacycle complex AJ-5 induces apoptotic cell death while reducing autophagic flux in rhabdomyosarcoma cells.

Cell Death Discov 2019 28;5:60. Epub 2019 Jan 28.

1Division of Cell Biology, Department of Human Biology, Faculty of Health Sciences, University of Cape Town, Cape Town, Western Cape South Africa.

Rhabdomyosarcoma (RMS) forms in skeletal muscle and is the most common soft tissue sarcoma in children and adolescents. Current treatment is associated with debilitating side effects and treatment outcomes for patients with metastatic disease are dismal. Recently, a novel binuclear palladacycle, AJ-5, was shown to exert potent cytotoxicity in melanoma and breast cancer and to present with negligible adverse effects in mice. Read More

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http://dx.doi.org/10.1038/s41420-019-0139-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6349869PMC
January 2019
1 Read

miR-545 promoted enterovirus 71 replication via directly targeting phosphatase and tensin homolog and tumor necrosis factor receptor-associated factor 6.

J Cell Physiol 2019 Jan 29. Epub 2019 Jan 29.

Basic Medicine College, Henan University of Chinese Medicine, Zhengzhou, Henan, China.

Enterovirus 71 (EV71) is a small, nonenveloped icosahedral RNA virus and is the predominant causative pathogen of hand-foot-and-mouth disease. Recently, microRNAs (miRNAs) are reported to play important roles in the pathogenesis of EV71 replication. This study investigated the role of miR-545 in the EV71 replication and explored the underlying molecular mechanisms. Read More

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http://dx.doi.org/10.1002/jcp.28222DOI Listing
January 2019
2 Reads

Aggressive recurrent orbital rhabdomyosarcoma in adulthood.

Avicenna J Med 2019 Jan-Mar;9(1):37

Department of Ophthalmology, Yusuf Al-Azma Hospital, Damascus, Syria.

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http://dx.doi.org/10.4103/ajm.AJM_149_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335881PMC
January 2019

[Transformation of teratoma in rabdomiosarcoma].

Medicina (B Aires) 2019 ;79(1):67-70

Departamento de Urología, Instituto de Oncología ángel H. Roffo, Universidad de Buenos Aires, Argentina.

Teratomas are malign germ cell tumors composed of two or more tissue layers. When there is specific organ differentiation they are called mature teratoma. They rarely grow aggressively. Read More

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January 2019
2 Reads

Dental and craniofacial alterations in long-term survivors of childhood head and neck rhabdomyosarcoma.

Oral Surg Oral Med Oral Pathol Oral Radiol 2018 Dec 21. Epub 2018 Dec 21.

Pathology Department, Universidade Federal Fluminense, Niterói (RJ), Brazil.

Objective: Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma that affects children. Treatment involves chemoradiotherapy. This study aimed at evaluating the long-term alterations to teeth and cranial bones in children, teenagers, and young adults after oncologic treatment. Read More

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http://dx.doi.org/10.1016/j.oooo.2018.12.012DOI Listing
December 2018
1 Read

Insight into the Etiology of Undifferentiated Soft Tissue Sarcomas from a Novel Mouse Model.

Mol Cancer Res 2019 Jan 25. Epub 2019 Jan 25.

Department of Cell and Developmental Biology, Vanderbilt University, Nashville, Tennessee.

Aberrant activation of the Hedgehog signaling pathway has been linked to the formation of numerous cancer types, including the myogenic soft tissue sarcoma, embryonal rhabdomyosarcoma (eRMS). Here, we report , a novel mouse model in which human GLI2A, a constitutive activator of Hedgehog signaling, induced undifferentiated sarcomas that were phenotypically divergent from eRMS. Rather, sarcomas arising in mice featured some characteristics that were reminiscent of Ewing sarcoma. Read More

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http://dx.doi.org/10.1158/1541-7786.MCR-18-0117DOI Listing
January 2019

Circ-ZNF609 regulates G1-S progression in rhabdomyosarcoma.

Oncogene 2019 Jan 22. Epub 2019 Jan 22.

Department of Biology and Biotechnology Charles Darwin, Sapienza University of Rome, Rome, Italy.

Circular RNAs (circRNAs) represent a class of covalently closed RNAs, derived from non-canonical splicing events, which are expressed in all eukaryotes and often conserved among different species. We previously showed that the circRNA originating from the ZNF609 locus (circ-ZNF609) acts as a crucial regulator of human primary myoblast growth: indeed, the downregulation of the circRNA, and not of its linear counterpart, strongly reduced the proliferation rate of in vitro cultured myoblasts. To deepen our knowledge about circ-ZNF609 role in cell cycle regulation, we studied its expression and function in rhabdomyosarcoma (RMS), a pediatric skeletal muscle malignancy. Read More

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http://dx.doi.org/10.1038/s41388-019-0699-4DOI Listing
January 2019

Dedifferentiated gastrointestinal stromal tumor: Recent advances.

Ann Diagn Pathol 2018 Dec 18. Epub 2018 Dec 18.

Department of Pathology and Genomic Medicine, Houston Methodist Hospital and Weill Medical College of Cornell University, Houston, TX 77030, USA. Electronic address:

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal lesions of the gastrointestinal tract. A small minority of GISTs exhibit morphologic and phenotypic changes and differentiate into an unusual phenotype through the process of dedifferentiation. Dedifferentiation can occur either de novo or after prolonged treatment with imatinib, a selective tyrosine kinase inhibitor. Read More

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http://dx.doi.org/10.1016/j.anndiagpath.2018.12.005DOI Listing
December 2018
5 Reads

Nutritional status and clinical outcomes in pediatric patients with solid tumors : A systematic review of the literature.

Semin Oncol 2019 Feb 21;46(1):48-56. Epub 2018 Dec 21.

Department of Pediatric Hematology, Oncology and Stem Cell Transplant, Morgan Stanley Children's Hospital, Columbia University Medical Center, New York, New York. Electronic address:

Introduction: Nutritional status (NS), defined by undernutrition (body mass index [BMI] <5th percentile) or overnutrition (BMI  ≥ 85th percentile), is a poor prognostic indicator in pediatric oncology patients. The impact of NS has been primarily studied in hematologic malignancies. This review is intended to summarize literature reporting on the association of NS and treatment-related outcomes in pediatric solid tumors. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00937754183020
Publisher Site
http://dx.doi.org/10.1053/j.seminoncol.2018.11.005DOI Listing
February 2019
2 Reads