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Am J Clin Oncol 2020 Jun 30. Epub 2020 Jun 30.

Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL.

Objective: Surgery followed by postoperative radiation therapy (RT) is the standard of care for soft tissue sarcomas (STS) of the head and neck that are high grade or have close or positive margins.

Methods: The authors retrospectively reviewed adult patients with head and neck STS treated with RT at a single institution between 1981 and 2017. All patients who were 19 years and older with STS of the head and neck-excluding rhabdomyosarcoma, angiosarcoma, and Ewing tumors-were included in this study. Read More

Rev Esp Med Nucl Imagen Mol 2020 Jun 29. Epub 2020 Jun 29.

Servicio de Medicina Nuclear, Hospital General Universitario Gregorio Marañón, Madrid, España; Miembro del Grupo Español de Investigación en Sarcomas (GEIS).

Soft tissue sarcomas (STS) are a rare and heterogeneous group of tumors. They account for 1% of solid malignant tumors in adults and 7% in children and are responsible for 2% of cancer mortality. They require a multidisciplinary approach in centers with experience. Read More

World J Clin Cases 2020 Jun;8(12):2641-2646

Department of Urology, The Second Hospital of Anhui Medical University, Hefei 230032, Anhui Province, China.

Background: Pleomorphic rhabdomyosarcoma (RMS) of the spermatic cord is a group of rare neoplasms, and a secondary hydrocele testis occasionally occurs. The misdiagnosis of paratesticular mass may lead to a therapeutic delay.

Case Summary: A 79-year-old man presented to our clinic complaining of a 1-mo history of painless scrotal swelling. Read More

Clin Neurol Neurosurg 2020 Jun 24;196:106044. Epub 2020 Jun 24.

Department of Radiation Oncology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand 249203, India.

J Clin Immunol 2020 Jun 30. Epub 2020 Jun 30.

Department of Hematopoietic Stem Cell Transplantation, Dmitry Rogachev National Medical Center of Pediatric Hematology, Oncology and Immunology, Samory Mashela str, Moscow, Russia, 117997.

Nijmegen breakage syndrome (NBS) is a DNA repair disorder characterized by combined immunodeficiency and a high predisposition to malignancies. HSCT appears to cure immunodeficiency, but remains challenging due to limited experience in long-term risks of transplant-associated toxicity and malignancies. Twenty NBS patients received 22 allogeneic HSCTs with TCRαβ/CD19+ graft depletion with fludarabine 150 mg/m, cyclophosphamide 20-40 mg/kg and thymoglobulin 5 mg/kg based conditioning regimens (CRs). Read More

Trends Cancer 2020 Jun 26. Epub 2020 Jun 26.

Centre de Recherche en Cancérologie de Marseille Inserm U1068, Aix-Marseille University, Marseille, France; Metronomics Global Health Initiative, Marseille, France.

Maintenance therapy sometimes relies on the use of metronomic chemotherapy (MC); that is, the continuous administration of low-dose chemotherapy. Maintenance therapy has been successfully used for decades in pediatric patients with acute lymphoblastic leukemia (ALL) and recent results have demonstrated improved outcomes in patients with pediatric high-risk rhabdomyosarcoma (RMS) on maintenance therapy. Here, we review the use of metronomic maintenance therapy in pediatric cancer and discuss its mechanisms of action on the tumor microenvironment and cancer cells. Read More

Biol Pharm Bull 2020 Jun 23. Epub 2020 Jun 23.

Department of Pharmacy, Hokkaido University Hospital.

Daptomycin, a cyclic lipopeptide antibiotic, has bactericidal activity against gram-positive organisms and is especially effective against methicillin-resistant Staphylococcus aureus. Although daptomycin causes unique adverse drug reactions such as elevation of creatine phosphokinase or rhabdomyolysis, the detailed mechanisms underlying these adverse drug reactions in skeletal muscle are unclear. This study aimed to elucidate whether daptomycin causes direct skeletal muscle cell toxicity and investigate the relationship between daptomycin exposure and musculoskeletal toxicity. Read More

Ann Clin Lab Sci 2020 May;50(3):412-416

Department of Pathology, Shengli Clinical Medical College of Fujian Medical University, Fujian Provincial Hospital, Fujian, China.

Rhabdomyosarcoma (RMS) is a primitive embryonal mesenchymal neoplasm demonstrating skeletal muscle differentiation. Diagnosis of RMS remains difficult due to the diversity of clinical features, pathological forms, and lesion's locations. Immunohistochemistry and Fluorescence in Situ Hybridization are common methods used to aid RMS diagnosis. Read More

Genes Cancer 2020 ;11(1-2):53-65

Department of Pathology, University of Colorado Denver, Anschutz Medical Campus, Aurora, CO, USA.

Rhabdomyosarcoma (RMS) is the most common soft tissue malignancy of childhood. RMS exists as two major disease subtypes, with oncofusion-positive RMS (FP-RMS) typically carrying a worse prognosis than oncofusion-negative RMS (FN-RMS), in part due to higher propensity for metastasis. Epigenetic mechanisms have recently emerged as critical players in the pathogenesis of pediatric cancers, as well as potential new therapeutic vulnerabilities. Read More

Pediatr Blood Cancer 2020 Jun 23:e28479. Epub 2020 Jun 23.

Department of Paediatric Surgery, Bicêtre Hospital, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicetre, France.

Background: Paratesticular rhabdomyosarcoma (PT RMS) is rare compared to benign scrotal pathology. Inappropriate first surgery (InFS) required supplementary treatment to maintain excellent outcomes. Initial staging of regional lymph nodes is important. Read More

Dent J (Basel) 2020 Jun 18;8(2). Epub 2020 Jun 18.

Periodontology Department, Clinical Research Unit (CRU), Centro de Investigação Interdisciplinar Egas Moniz (CiiEM), Instituto Universitário Egas Moniz (IUEM), 2829-511 Caparica, Almada, Portugal.

Rhabdomyosarcoma is one of the most common soft-tissue sarcomas in children. The therapy for this condition has evolved significantly over recent decades, as has survival rates. Nevertheless, multiagent chemotherapy, radiation therapy, surgical resection or a combination of these modalities still have to be performed. Read More

JNCI Cancer Spectr 2019 Sep 20;3(3):pkz043. Epub 2019 Jun 20.

Radiation Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, MD.

Excess sarcoma risks after childhood cancer are well established, but risks among young adulthood cancer survivors are poorly understood. Using US population-based cancer registry data, we compared bone and soft-tissue sarcoma risk vs the general population among 186 351 individuals who were diagnosed with nonsarcoma first primary malignancies at ages 20-39 years from 1975 to 2014 (follow-up through 2015) and survived at least 1 year. Bone sarcomas were rare (n = 50), but risk was statistically significantly elevated overall (2. Read More

J Vet Med Sci 2020 Jun 22. Epub 2020 Jun 22.

Department of Pathogenetic and Preventive Veterinary Science, Joint Faculty of Veterinary Medicine, Kagoshima University.

Alveolar rhabdomyosarcoma (ARMS) is a rare mesenchymal tumor with differentiation toward the skeletal muscle. Although several cases of canine ARMS have been reported in veterinary medicine, only one case of abdominal ARMS has been reported in a cow. A 13-month-old, Japanese black heifer was referred for pus-like nasal discharge. Read More

Biochem Biophys Res Commun 2020 Aug 6;529(1):97-103. Epub 2020 Jun 6.

Shanghai Public Health Clinical Center & Institutes of Biomedical Sciences, Fudan University, Shanghai, China. Electronic address:

Enterovirus A71 (EVA71) and Coxsackievirus A10 (CVA10) are representative types of Enterovirus A. Dependent on the host cell types, the EVA71 entry may utilize clathrin-, caveola-, and endophilin-A2-mediated endocytosis. However, the cell-entry and intracellular trafficking pathways of CVA10, using KREMEN1 as its receptor, are unclear. Read More

Molecules 2020 Jun 17;25(12). Epub 2020 Jun 17.

Institute of Chemical Biology and Fundamental Medicine SB RAS, 630090 Novosibirsk, Russia.

Natural compounds of various origins are intensively investigated for their antitumor activity. Potential benefits of antitumor therapy can be achieved when cytotoxic agents kill cancer cells and these dying cancer cells drive adoptive immunity to the tumor. This strategy was successfully demonstrated for chemotherapeutic drugs that induce immunogenic type of cell death (ICD) with release of DAMPs (danger associated molecular patterns) and exposure of "eat me" signals. Read More

Viruses 2020 Jun 17;12(6). Epub 2020 Jun 17.

Centre for Virus and Vaccine Research, School of Science and Technology, Sunway University, Kuala Lumpur, Selangor 47500, Malaysia.

Viral plaque morphologies in human cell lines are markers for growth capability and they have been used to assess the viral fitness and selection of attenuated mutants for live-attenuated vaccine development. In this study, we investigate whether the naturally occurring plaque size variation reflects the virulence of the variants of EV-A71. Variants of two different plaque sizes (big and small) from EV-A71 sub-genotype B4 strain 41 were characterized. Read More

Br J Radiol 2020 Jun 26:20200250. Epub 2020 Jun 26.

Medical Oncology and Hematology Unit, Humanitas Clinical and Research Center, IRCCS, Rozzano, Italy.

Rhabdomyosarcoma (RMS) represents more than 50% of paediatric soft tissue tumours. Conversely, it is extremely rare among adults, where it shows peculiar biological and clinical features that are still poorly investigated. RMS patients should be referred to a Sarcoma Centre, where the contribution of experienced radiologists plays a relevant role in the diagnostic assessment of the disease, including precise localisation, staging, image-guided biopsy, response evaluation after treatment and follow-up. Read More

Pediatr Blood Cancer 2020 Jun 18:e28351. Epub 2020 Jun 18.

Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy.

Background: As dermatofibrosarcoma protuberans (DFSP) are rare with no prospective series within paediatric sarcoma trials, the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) examined the clinical data and outcomes of DFSP enrolled in a multinational study of non-rhabdomyosarcoma soft tissue sarcomas (NRSTS).

Patients And Methods: Forty-six patients with confirmed DFSP were enrolled into the EpSSG NRSTS 2005 study. All had surgical resection and none had any further therapy at diagnosis. Read More

Virchows Arch 2020 Jun 19. Epub 2020 Jun 19.

Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada.

The WHO Classification of Tumors of Soft Tissue and Bone divides rhabdomyosarcoma (RMS) into alveolar, embryonal, pleomorphic, and spindle cell/sclerosing types. Advances in molecular diagnostics have allowed for further refinement of RMS classification including the identification of new subtypes. Very rare RMS with epithelioid and spindle cell morphology, female predominance, marked osseous predilection, ALK expression, EWSR1/FUS-TFCP2 gene fusions, and highly aggressive clinical behavior have recently been recognized with only 23 cases reported in the English language literature. Read More

J Pediatr Hematol Oncol 2020 Jun 16. Epub 2020 Jun 16.

Department of Pediatrics, Mie University Graduate School of Medicine, Mie, Japan.

Li-Fraumeni syndrome (LFS) is a rare inherited disease characterized by a high and early-onset cancer risk. A cancer surveillance program is important to reduce cancer-related morbidity and mortality in individuals with LFS. We report 2 pediatric cases with LFS-related malignancy in a family. Read More

Eur J Cancer 2020 Jun 15;135:89-97. Epub 2020 Jun 15.

Gustave Roussy, Villejuif, France.

Background: The phase I component of a phase I/II study defined the recommended phase II dose and established the tolerability of nab-paclitaxel monotherapy in paediatric patients with recurrent or refractory solid tumours. The activity and safety of nab-paclitaxel monotherapy was further investigated in this phase II study.

Patients And Methods: Paediatric patients with recurrent or refractory Ewing sarcoma, neuroblastoma or rhabdomyosarcoma received 240 mg/m of nab-paclitaxel on days 1, 8 and 15 of each 28-day cycle. Read More

Indian J Otolaryngol Head Neck Surg 2020 Jun 26;72(2):214-220. Epub 2019 Nov 26.

Department of ENT, ESI PGIMSR, Basaidarapur, Guru Gobind Singh Indraprastha University, New Delhi, India.

We report four cases of embryonal rhabdomyosarcoma treated successfully with multimodality therapy which included surgery, chemotherapy and radiotherapy. Our first case was a 2 year old who admitted with the chief complaint of swelling of the upper lip. Biopsy was taken, which showed embryonal rhabdomyosarcoma. Read More

Braz J Cardiovasc Surg 2020 Jun 1;35(3):399-401. Epub 2020 Jun 1.

Yamagata University Faculty of Medicine Second Department of Surgery Yamagata Japan Second Department of Surgery, Faculty of Medicine, Yamagata University, Yamagata, Japan.

Primary malignant neoplasms of the heart are rare. Cardiac rhabdomyosarcoma is the second most common primary sarcoma. We report a rare case of a 49-year-old woman with a huge biatrial cardiac rhabdomyosarcoma treated by performing surgical resection followed by salvage chemotherapy for local recurrence. Read More

Brain Behav 2020 Jun 16:e01697. Epub 2020 Jun 16.

Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Beijing, China.

Introduction: To identify possible prognostic factors in children with head and neck rhabdomyosarcoma (RMS).

Methods: A total of 98 patients with head and neck RMS were enrolled in this retrospective study from February 2005 to September 2017. Prognostic factors were evaluated by univariate and multivariate analysis using Cox's proportional hazards model. Read More

Oncolytic Virother 2020 26;9:17-29. Epub 2020 May 26.

Department of Microbiology, Immunology, and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO, USA.

Purpose: Rhabdomyosarcomas (RMS) are difficult tumors to treat with conventional therapies. Publications indicate that oncolytic virotherapy (OV) could benefit cancer patients with tumors that are refractory to conventional treatments. It is believed that the efficacy of OV can be enhanced when used in combination with other treatments. Read More

Prostaglandins Leukot Essent Fatty Acids 2020 May 30;159:102139. Epub 2020 May 30.

Department of Pharmaceutical Sciences, Tohoku University Hospital, Sendai, Miyagi, 980-8574, Japan; Department of Pharmacy, Yamagata University Hospital, Yamagata, 990-9585, Japan. Electronic address:

ATP-binding cassette transporter C4 (ABCC4) is associated with multidrug resistance and the regulation of cell signalling. Some prostaglandins (PGs), including: PGE, PGF, PGE, and PGF are known substrates of ABCC4, and are released from some types of cells to exert their biological effects. In the present study, we demonstrate that PGD is a novel substrate of ABCC4 using a transport assay based on inside-out membrane vesicles prepared from ABCC4-overexpressing cells. Read More

J Mech Behav Biomed Mater 2020 Sep 11;109:103844. Epub 2020 May 11.

Universitat Politècnica de Catalunya, Departament of Mechanical Engineering. School of Engineering of Barcelona (ETSEIB), Av. Diagonal, 647, 08028, Barcelona, Spain.

Biliary tract rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor which is located in the biliary tract. Although this tumor represents less than 1% of the total amount of childhood cancers, when localized, a >70% overall 5-year survival rate, the resection is clinically challenging and complications might exist during the biliary obstruction. Although surgery remains a mainstay, complete tumor resection is generally difficult to achieve without mutilation and severe long-term sequelae. Read More

J Clin Res Pediatr Endocrinol 2020 Jun 16. Epub 2020 Jun 16.

Pediatrics, University of Health Sciences, Gulhane Training and Research Hospital, Ankara, Turkey.

Most cases of malignancies appear to be sporadic, but some syndromes are associated with malignancies with germline variants. Herein, a child with an unusual association of oncocytic variant adrenocortical carcinoma (ACC) and rhabdomyosarcoma was presented. An 18-month-old-boy was admitted with virilization of the genital area, penis enlargement, and erection which had begun six months ago. Read More

Cold Spring Harb Mol Case Stud 2020 Jun 12;6(3). Epub 2020 Jun 12.

Children's Cancer Therapy Development Institute, Beaverton, Oregon 97005, USA.

In this case report we evaluate the genetics of and scientific basis of therapeutic options for a 14-yr-old male patient diagnosed with metastatic PAX3-FOXO1 fusion positive alveolar rhabdomyosarcoma. A distinguishing genetic feature of this patient was a germline RET C634F mutation, which is a known driver of multiple endocrine neoplasia type 2A (MEN2A) cancer. Through sequential DNA and RNA sequencing analyses over the patient's clinical course, a set of gene mutations, amplifications, and overexpressed genes were identified and biological hypotheses generated to explore the biology of RET and coexisting signaling pathways in rhabdomyosarcoma. Read More

Laryngoscope 2020 Jun 12. Epub 2020 Jun 12.

Department of Otolaryngology - Head and Neck Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois, U.S.A.

Objective: To evaluate factors associated with overall survival (OS) of patients with non-rhabdomyosarcoma soft tissue sarcomas of the head and neck.

Study Design: Retrospective cohort study.

Methods: The National Cancer Database was queried for cases of non-rhabdomyosarcoma soft tissue sarcomas of the head and neck between 2004 and 2014. Read More

Pediatr Blood Cancer 2020 Aug 11;67(8):e28442. Epub 2020 Jun 11.

Department of Pediatrics, Nihon University Itabashi Hospital, Tokyo, Japan.

Int J Cancer 2020 Jun 11. Epub 2020 Jun 11.

Department of Surgery, East Carolina University, Greenville, North Carolina.

Paratesticular rhabdomyosarcoma (PT-RMS) carries a favorable prognosis, but questions persist regarding optimal management. Our goal was to determine the importance of primary tumor resection and surgical assessment of retroperitoneal lymph nodes during staging in patients with PT-RMS. We analyzed patients with localized PT-RMS enrolled onto one of four Children's Oncology Group studies (D9602, ARST0331, D9803 or ARST0531). Read More

Virchows Arch 2020 Jun 9. Epub 2020 Jun 9.

Department of Pathology and Laboratory Medicine, Emory University, Atlanta, GA, 30322, USA.

Histiocyte-rich rhabdomyoblastic tumor is a recently described skeletal muscle neoplasm of uncertain malignant potential, characterized by slow growth, a fibrous capsule containing peripheral lymphoid aggregates, spindle-to-epithelioid cells with a rhabdomyoblastic immunophenotype, and a dense histiocytic infiltrate. It most commonly arises within the muscles of the lower legs and trunk in young-to-middle-aged men, and initial reports suggest indolent behavior. In this paper, we present two additional cases of histiocyte-rich rhabdomyoblastic tumor with similar clinicopathologic features and discuss the differential diagnosis including its overlap with inflammatory leiomyosarcoma. Read More

Fetal Pediatr Pathol 2020 Jun 8:1-8. Epub 2020 Jun 8.

Department of Pathology, Institute of Postgraduate Medical Education and Research, Kolkata, India.

Giant cell fibroblastoma (GCF) shows a wide spectrum of morphological patterns which may lead to a misdiagnosis of sarcoma. This 14- month- old baby was referred to us for recurrent left scrotal embryonal rhabdomyosarcoma (ERMS), first diagnosed at 8 months, status post chemotherapy. Review of previous histology, cytology (with frequent multinucleated floret type giant cells but without cross striations) and immunohistochemistry resulted in the change of diagnosis to GCF. Read More

Virchows Arch 2020 Jun 7. Epub 2020 Jun 7.

Instituto de Investigação e Inovação em Saúde, Porto, Portugal.

Primary thyroid teratomas are exceedingly rare. Mature and immature variants recapitulate their gonadal counterparts (predilection for infants/children, triphasic germ layer differentiation, and favorable outcome). On the other hand, the so-called malignant teratomas affect predominantly adults and elderly, are highly aggressive, and, according to a few published cases, harbor DICER1 mutations. Read More

Head Neck Pathol 2020 Jun 5. Epub 2020 Jun 5.

Pediatric Hematology and Oncology, Children's Minnesota, Minneapolis, MN, USA.

An example of a mandibular rhabdomyosarcoma in a 15-year-old male is described featuring EWSR1-TFCP2 fusion with homolateral lymph node metastasis and apparent metastasis to the thoracic vertebra T7. This type of rhabdomyosarcoma has preference for the craniofacial skeleton. Histologically, the tumor was composed of spindle and epithelioid cells characterized by nuclear pleomorphism, cytologic atypia and brisk mitotic activity. Read More

Acta Oncol 2020 Jun 5:1-7. Epub 2020 Jun 5.

Department of Radiation Oncology, University of Maryland School of Medicine, Baltimore, MD, USA.

Pelvic target dose from intensity-modulated proton therapy (IMPT) is sensitive to patient bowel motion. Robustly optimized plans in regard to bowel filling may improve the dose coverage in the treatment course. Our purpose is to investigate the effect of air volume in large and small bowel and rectum on target dose from IMPT plans. Read More

Oncotarget 2020 May 19;11(20):1799-1815. Epub 2020 May 19.

Laboratory of Molecular Immunology and Immunotherapy, Blood Research Institute, Versiti, Milwaukee, WI, USA.

Histone deacetylase inhibitors (HDACi) are an emerging cancer therapy; however, their effect on natural killer (NK) cell-mediated anti-tumor responses remain unknown. Here, we evaluated the impact of a benzamide HDACi, entinostat, on human primary NK cells as well as tumor cell lines. Entinostat significantly upregulated the expression of NKG2D, an essential NK cell activating receptor. Read More

PET Clin 2020 Jul;15(3):333-347

Department of Radiology, Seattle Children's Hospital, M/S MA.7.220, 4800 Sand Point Way Northeast, Seattle, WA 98105, USA. Electronic address:

The role for PET with fludeoxyglucose F 18 (F-FDG PET)/computed tomography (CT) in the management of pediatric sarcomas continues to be controversial. The literature supports a role for PET/CT in the staging and surveillance of certain specific pediatric sarcoma subtypes; however, the data are less clear regarding whether PET/CT can be used as a biomarker for prognostication. Despite the interest in using this imaging modality in the management of pediatric sarcomas, most studies are limited by retrospective design and small sample size. Read More

Bol Med Hosp Infant Mex 2020 ;77(3):149-152

Servicio de Enfermedades Infecciosas, Departmento de Pediatría, Hospital Universitario Dr. José Eleuterio González, Monterrey, Nuevo León, Mexico.

Background: Weeksella virosa is one of the two species of the genus Weeksella. Clinical disease due to this bacterium in humans is rare, for which only nine cases have been reported in the literature.

Case Report: A 4-year-old male patient was diagnosed with a left orbit rhabdomyosarcoma Stage III and was admitted to a northeast third level referral center in Mexico. Read More

Pediatr Int 2020 Jun 4;62(6):742-744. Epub 2020 Jun 4.

Division of Hematology/Oncology, Tokyo Metropolitan Children Medical Center, Tokyo, Japan.

BMC Cancer 2020 Jun 3;20(1):511. Epub 2020 Jun 3.

Department of Pediatrics, Division of Hematology/Oncology, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine Chicago, Box 30, 225 East Chicago Ave., Chicago, IL, 60611, USA.

Background: The clinical significance of GLI1 expression either through canonical Hedgehog signal transduction or through non-canonical mechanisms in rhabdomyosarcoma (RMS) or Ewing sarcoma (EWS) is incompletely understood. We tested a role for Hedgehog (HH) signal transduction and GL11 expression in development of vincristine (VCR) resistance in RMS and EWS.

Methods: We characterized baseline expression and activity of HH pathway components in 5 RMS (RD, Rh18, Ruch-2, Rh30, and Rh41) and 5 EWS (CHLA9, CHLA10, TC32, CHLA258, and TC71) cell lines. Read More

Cancer Cell Int 2020 24;20:192. Epub 2020 May 24.

Department of Pediatrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Hirokoji, Kamigyo-ku, Kyoto, 602-8566 Japan.

Background: Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma. There are two subtypes, fusion gene-positive RMS (FP-RMS) and fusion gene-negative RMS (FN-RMS), depending on the presence of a fusion gene, either - or -. These fusion genes are thought to be oncogenic drivers of FP-RMS. Read More

Adv Exp Med Biol 2020 ;1257:1-10

Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Osteosarcoma was initially resistant to chemotherapy that worked for Ewing sarcoma and rhabdomyosarcoma as well as other chemotherapeutic agents available in the 1960s. In the early 1970s, responses of osteosarcoma to adriamycin were reported, and at about the same time, so were responses of osteosarcoma to high-dose methotrexate. These agents were introduced into adjuvant therapy due to the dire prognosis associated with apparently localized osteosarcoma. Read More

BMC Pediatr 2020 May 29;20(1):265. Epub 2020 May 29.

Pediatric Department of Beijing Tongren Hospital, Capital Medical University, 100730, 1# Dong Jiao Min Xiang, Dongcheng District, Beijing, China.

Background: The purpose of this study is to analyze the influence of radiation therapy on survival in a historical cohort of 56 pediatric patients with head and neck rhabdomyosarcoma.

Methods: A historical cohort of 56 pediatric patients with head and neck rhabdomyosarcoma from June 1st, 2013 to June 30th, 2019 was chosen. Clinical data and follow up results were collected including all diagnosis, treatment and prognosis information. Read More

Biomater Sci 2020 Jul 29;8(13):3678-3684. Epub 2020 May 29.

NanoMedSyn Avenue Charles Flahault, 34093, Montpellier Cedex 05, France.

Porphyrin-based periodic mesoporous organosilica nanoparticles (PMO) synthesized from a large functional octatriethoxysilylated porphyrin precursor and allowing two-photon excitation photodynamic therapy (TPE-PDT) and NIR imaging were synthesized. These PMO were grafted with polyethylene glycol (PEG) moieties and an analogue of mannose 6-phosphate functionalized at the anomeric position (AMFA). AMFAs are known to efficiently target mannose 6-phosphate receptors (M6PRs) which are over-expressed in various cancers. Read More

Environ Sci Pollut Res Int 2020 May 28. Epub 2020 May 28.

Department of Applied Sciences, University of Technology, Baghdad, Iraq.

In this work, we have prepared cerium oxide (CeO) nanoparticles (NPs) by laser ablation in water at different laser energies. The structural and optical properties of synthesized nanoparticles were characterized by X-ray diffraction (XRD), scanning electron microscope (SEM), Raman spectroscopy, energy dispersive X-ray (EDX), and UV-Vis absorption. XRD results confirmed that the synthesized cerium oxide NPs were crystalline in nature with cubic structure. Read More

J Pediatr Urol 2020 Jun 30;16(3):357-365. Epub 2020 Apr 30.

The Hospital for Sick Children, Division of Urology, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.

Introduction: Neurofibromatosis Type 1 (NF1) is an autosomal dominant, multisystem, neurocutaneous disorder. This condition has been associated with lower urinary tract dysfunction due to either direct genitourinary organ involvement or spinal cord compression. Based on current literature, there are no reviews examining the relationship between NF1 and lower urinary tract dysfunction (LUTD) in the paediatric population. Read More

Neoplasia 2020 Jul 25;22(7):274-282. Epub 2020 May 25.

Division of Hematology-Oncology, Cancer and Blood Disease Institute, Children's Hospital Los Angeles, Los Angeles, CA, USA; Keck School of Medicine, University of Southern California, Los Angeles, CA, USA. Electronic address:

Fibroblast growth factor receptor 4 (FGFR4) aberrant expression and activity have been linked to the pathogenesis of a variety of cancers including rhabdomyosarcomas (RMS). We found that treatment of alveolar rhabdomyosarcoma (aRMS) cells with Guadecitabine (SGI-110), a next-generation DNA methyltransferase inhibitor (DNMTi), resulted in a significant reduction of FGFR4 protein levels, 5 days post treatment. Chromatin immunoprecipitation-sequencing (ChIP-seq) in aRMS cells revealed attenuation of the H3K4 mono-methylation across the FGFR4 super enhancer without changes in tri-methylation of either H3K4 or H3K27. Read More

Cancer 2020 May 26. Epub 2020 May 26.

Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

Background: Approximately 50% of children with cancer in the United States who are aged <15 years receive primary treatment on a therapeutic clinical trial. To the authors' knowledge, it remains unknown whether trial enrollment has a clinical benefit compared with the best alternative standard therapy and/or off trial (ie, clinical trial effect). The authors conducted a retrospective matched cohort study to compare the morbidity and mortality of pediatric patients with cancer who are treated on a phase 3 clinical trial compared with those receiving standard therapy and/or off trial. Read More