13,663 results match your criteria Rhabdomyosarcoma


Worse Outcomes for Head and Neck Rhabdomyosarcoma Secondary to Reduced-dose Cyclophosphamide.

Int J Radiat Oncol Biol Phys 2018 Nov 30. Epub 2018 Nov 30.

Department of Radiation Oncology. Electronic address:

Purpose: Recent trends including the use of proton therapy and administration of reduced-doses of cyclophosphamide have been adapted in head and neck rhabdomyosarcoma (HN-RMS) to reduce late morbidity. Our primary goal was to analyze local control and survival outcomes after photon versus proton irradiation in pediatric patients with HN-RMS, with the secondary goal of analyzing the effect of cyclophosphamide dose on disease outcomes.

Methods And Materials: This single-institution cohort study was comprised of 76 pediatric HN-RMS patients treated with definitive chemoradiation from 2000 to 2018. Read More

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November 2018

A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma.

Oman J Ophthalmol 2018 Sep-Dec;11(3):284-287

Department of Ophthalmology, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.

Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. Read More

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December 2018

Combined management of perianal rhabdomyosarcoma with chemotherapy, radical surgery, and irradiation: A series of three consecutive children.

J Pediatr Surg 2018 Nov 7. Epub 2018 Nov 7.

Children's Hospital, Section of Pediatric Surgery, University of Helsinki, Finland.

Background And Aim: We describe a series of three successive patients with perianal rhabdomyosarcoma (PRMS) from 2014 to 2017 managed with combined chemotherapy, radical surgery, and radiotherapy.

Methods: Ethical consent was obtained. Data including tumor presentation, treatment, and survival was collected from hospital reports. Read More

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November 2018

Primary Meningeal Rhabdomyosarcoma of the Spinal Cord of a Young Dog with Neuromelanocytosis and Multiple Cutaneous Neurofibromas.

J Comp Pathol 2018 Nov 25;165:57-61. Epub 2018 Oct 25.

Frederick National Laboratory for Cancer Research, National Cancer Institute, Fort Detrick, Frederick, Maryland, USA.

A 7-week-old male black Labrador retriever puppy was presented for post-mortem examination following progressive hindlimb paralysis and multiple masses within the skin. A highly compressive and infiltrative intradural mass was found within the T9-T11 spinal cord. Microscopical and immunohistochemical analysis revealed features compatible with spindle cell rhabdomyosarcoma (RMS). Read More

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November 2018

Malignant Cutaneous Peripheral Nerve Sheath Tumour with Rhabdomyosarcomatous Differentiation (Triton Tumour) in a Domestic Cat.

J Comp Pathol 2018 Nov 18;165:1-5. Epub 2018 Oct 18.

Department of Pathobiology and Population Sciences, The Royal Veterinary College, Hatfield, Hertfordshire, UK.

Divergent differentiation is encountered frequently within human malignant peripheral nerve sheath tumours (MPNSTs). The new component is often a rhabdomyosarcoma, but in animals this specific form of divergent differentiation within MPNSTs has only been reported once (in a dog). Incisional wedge biopsy of a locally extensive, ventral abdominal wall mass, which extended from the dermis to the subcutis, from a 12-year-old female domestic shorthaired cat, was performed. Read More

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November 2018
1 Read

Congenital Ewing's Sarcoma, a Rare and Difficult Diagnosis: A Case Report.

Eurasian J Med 2018 Oct;50(3):202-203

Department of Gynecology and Obstetrics, Atatürk University School of Medicine, Erzurum, Turkey.

We have interestedly read the article written by Thalia Wong BS in July 2015, which is about Pediatric Blood Cancer, including clinical findings and results of infants <1 year of age with Ewing sarcoma. We report a case with congenital Ewing's sarcoma that easily interfered with rabdomyosarcoma in a pregnant woman. A 32-year-old multigravida with a big neck mass at 35 weeks was referred to our clinic. Read More

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October 2018

Minimally Invasive Surgery in Pediatric Surgical Oncology.

Children (Basel) 2018 Nov 26;5(12). Epub 2018 Nov 26.

Department of Pediatric Surgery, Vanderbilt University Medical Center, Nashville, TN 37232, USA.

The application of minimally invasive surgery (MIS) to resect pediatric solid tumors offers the potential for reduced postoperative morbidity with smaller wounds, less pain, fewer surgical site infections, decreased blood loss, shorter hospital stays, and less disruption to treatment regimens. However, significant controversy surrounds the question of whether a high-fidelity oncologic resection of childhood cancers can be achieved through MIS. This review outlines the diverse applications of MIS to treat pediatric malignancies, up to and including definitive resection. Read More

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November 2018

Rhabdomyosarcoma with FUS re-arrangement: additional case in support of a novel subtype.

Pathology 2018 Nov 23. Epub 2018 Nov 23.

Department of Anatomical Pathology, PathWest, QEII Medical Centre, Nedlands, WA, Australia.

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November 2018

HuC/D expression in small round cell tumors and neuroendocrine tumors: a useful tool for distinguishing neuroblastoma from childhood small round cell tumors.

Hum Pathol 2018 Nov 20. Epub 2018 Nov 20.

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

The RNA-binding protein HuC/D displays a neuron-specific expression and is involved in neuronal differentiation and the maintenance of the nervous system. Here we investigated the diagnostic value of HuC/D in neuroblastomas. We evaluated 85 neuroblastic tumors: 81 neuroblastomas; three ganglioneuroblastomas, intermixed; one ganglioneuroma, maturing, and 101 other tumors consisting of 34 Ewing sarcomas, 14 nephroblastomas, 11 rhabdomyosarcomas, 15 pulmonary small cell carcinomas, 18 pancreatic neuroendocrine tumors, and nine pheochromocytomas. Read More

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November 2018
1 Read

English Translation of M. Bérard: Tumeur Embryonnaire Du Muscle Strié. [Embryonal Tumor of Striated Muscle]. Lyon Med 1894; 77: 52.

Fetal Pediatr Pathol 2018 Nov 23:1-3. Epub 2018 Nov 23.

c Professor of Pathology, 4650 Sunset Boulevard #43, Children's Hospital of Los Angeles, CA, USA.

We have chosen to translate what we believe to be the first publication of a well-documented case of a young patient with embryonal rhabdomyosarcoma. The author, M. Léon Bérard, was a hospital fellow working in the department of M. Read More

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November 2018

Clinical Utility of In Situ Hybridization Assays in Head and Neck Neoplasms.

Head Neck Pathol 2018 Nov 22. Epub 2018 Nov 22.

Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Sydney, Australia.

Head and neck pathology present a unique set of challenges including the morphological diversity of the neoplasms and presentation of metastases of unknown primary origin. The detection of human papillomavirus and Epstein-Barr virus associated with squamous cell carcinoma and newer entities like HPV-related carcinoma with adenoid cystic like features have critical prognostic and management implications. In salivary gland neoplasms, differential diagnoses can be broad and include non-neoplastic conditions as well as benign and malignant neoplasms. Read More

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November 2018
3 Reads

Diagnosis of known sarcoma fusions and novel fusion partners by targeted RNA sequencing with identification of a recurrent ACTB-FOSB fusion in pseudomyogenic hemangioendothelioma.

Mod Pathol 2018 Nov 21. Epub 2018 Nov 21.

Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, 10065, USA.

Integration of morphological, immunohistochemical, and molecular methods is often necessary for the precise diagnosis and optimal clinical management of sarcomas. We have validated and implemented a clinical molecular diagnostic assay, MSK- Fusion Solid, for detection of gene fusions in solid tumors, including sarcomas. Starting with RNA extracted from formalin-fixed paraffin-embedded tumor material, this targeted RNA sequencing assay utilizes anchored multiplex PCR to detect oncogenic fusion transcripts involving 62 genes known to be recurrently rearranged in solid tumors including sarcomas without prior knowledge of fusion partners. Read More

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November 2018
4 Reads

Pannexin 1 inhibits rhabdomyosarcoma progression through a mechanism independent of its canonical channel function.

Oncogenesis 2018 Nov 21;7(11):89. Epub 2018 Nov 21.

Molecular Biomedicine Program, Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada.

Rhabdomyosarcoma (RMS) is an aggressive soft tissue sarcoma of childhood thought to arise from impaired differentiation of skeletal muscle progenitors. We have recently identified Pannexin 1 (PANX1) channels as a novel regulator of skeletal myogenesis. In the present study, we determined that PANX1 transcript and protein levels are down-regulated in embryonal (eRMS) and alveolar RMS (aRMS) patient-derived cell lines and primary tumor specimens as compared to differentiated skeletal muscle myoblasts and tissue, respectively. Read More

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November 2018
3 Reads

The HDAC3-SMARCA4-miR-27a axis promotes expression of the fusion oncogene in rhabdomyosarcoma.

Sci Signal 2018 Nov 20;11(557). Epub 2018 Nov 20.

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

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood with an unmet clinical need for decades. A single oncogenic fusion gene is associated with treatment resistance and a 40 to 45% decrease in overall survival. We previously showed that expression of this fusion oncogene in alveolar RMS (aRMS) mediates tolerance to chemotherapy and radiotherapy and that the class I-specific histone deacetylase (HDAC) inhibitor entinostat reduces PAX3:FOXO1 protein abundance. Read More

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November 2018
2 Reads

Demographics, stage distribution, and relative roles of surgery and radiotherapy on survival of persons with primary prostate sarcomas.

Cancer Med 2018 Nov 19. Epub 2018 Nov 19.

Department of Radiation Oncology, University of Utah School of Medicine, Salt Lake City, Utah.

Background: Primary prostate sarcomas (PPS) are rare. Outcomes for this cancer have not been well characterized.

Materials And Methods: Subjects with a PPS diagnosed between 1973 and 2014 were identified in the SEER database. Read More

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November 2018
3 Reads

Para testicular adult rhabdomyosarcoma: A case report and review of litterature.

Urol Case Rep 2019 Jan 22;22:60-61. Epub 2018 Sep 22.

Department of Medical Oncology, Salah Azaiez Institute, Faculty of Medicine of Tunis, Boulevard 9 Avril, Tunis El Manar University, Tunis, Tunisia.

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January 2019

Oncogenic Amplification of Zygotic Dux Factors in Regenerating p53-Deficient Muscle Stem Cells Defines a Molecular Cancer Subtype.

Cell Stem Cell 2018 Nov 1. Epub 2018 Nov 1.

Department of Cardiac Development and Remodeling, Max-Planck-Institute for Heart and Lung Research, Bad Nauheim, Germany; German Center for Cardiovascular Research (DZHK), Rhine Main, Germany. Electronic address:

The identity of tumor-initiating cells in many cancer types is unknown. Tumors often express genes associated with embryonic development, although the contributions of zygotic programs to tumor initiation and formation are poorly understood. Here, we show that regeneration-induced loss of quiescence in p53-deficient muscle stem cells (MuSCs) results in rhabdomyosarcoma formation with 100% penetrance. Read More

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November 2018

RITA downregulates Hedgehog-GLI in medulloblastoma and rhabdomyosarcoma via JNK-dependent but p53-independent mechanism.

Cancer Lett 2018 Nov 14;442:341-350. Epub 2018 Nov 14.

Department of Biosciences and Nutrition, Karolinska Institutet, Huddinge, Sweden. Electronic address:

Overactivation of the Hedgehog (HH) signaling pathway is implicated in many cancers. In this study, we demonstrate that the small molecule RITA, a p53 activator, effectively downregulates HH signaling in human medulloblastoma and rhabdomyosarcoma cells irrespective of p53. This is mediated by a ROS-independent activation of the MAP kinase JNK. Read More

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November 2018
3 Reads

Concurrent Congenital Fibrolipomatous Hamartoma and Congenital Nevus of Infancy: A Syndromic or Chance Association.

J Indian Assoc Pediatr Surg 2018 Oct-Dec;23(4):219-221

Department of Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Congenital nevi and fibrolipomatous hamartoma are benign tumors of childhood, the latter being very uncommon. Fibrous hamartoma of infancy typically occurs in <2 years of life. The concurrence of these two lesions is extremely rare. Read More

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November 2018
1 Read

Immunohistochemistry for small cell carcinoma: a potential diagnostic pitfall.

Histopathology 2018 Nov 15. Epub 2018 Nov 15.

Department of Pathology, Royal Infirmary of Edinburgh and University of Edinburgh Medical School, Edinburgh, UK.

The morphological appearances of small cell lung carcinoma (SCLC) are often characteristic however immunohistochemistry (IHC) is frequently employed to support the diagnosis, especially when there is crush artefact. In general histopathologists look for expression of cytokeratins, CD56 and TTF1 but it is well recognised that SCLC may not show typical staining reactions. While CD56 is usually expressed TTF1 may be negative and cytokeratin staining may be very focal or even absent. Read More

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November 2018
1 Read

Localized synovial sarcoma of the foot or ankle: A series of 32 Cooperative Weichteilsarkom Study Group patients.

J Surg Oncol 2018 Nov 13. Epub 2018 Nov 13.

Pediatrics 5 (Oncology, Hematology, Immunology), Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.

Background: Synovial sarcoma of the foot/ankle is rare. Mutilating surgery is often discussed.

Methods: Patients registered from 1981 to 2013 were analyzed. Read More

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November 2018
6 Reads

Evolving Role of PET/CT-MRI in Assessing Muscle Disorders.

PET Clin 2019 Jan 24;14(1):71-79. Epub 2018 Oct 24.

Department of Radiology, University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, USA. Electronic address:

2-Deoxy-2-[F]fluoroglucose (FDG) uptake in muscle is influenced by many normal physiologic processes and can also indicate pathology. Variability in physiologic uptake can be reduced with proper patient preparation, allowing for a better determination of abnormal activity. Although malignant diseases, such as rhabdomyosarcoma and skeletal muscle metastasis, are clear applications of FDG-PET/CT, there may be additional applications in infection and benign inflammatory disorders that warrant further research. Read More

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January 2019

Autophagy modulates temozolomide-induced cell death in alveolar Rhabdomyosarcoma cells.

Cell Death Discov 2018 25;4:52. Epub 2018 Oct 25.

1Department of Human Anatomy and Cell Science, Max Rady College of Medicine, Rady Faculty of Health Science, University of Manitoba, Winnipeg, Canada.

Rhabdomyosarcoma (RMS) is a muscle-derived tumor. In both pre-clinical and clinical studies Temozolomide (TMZ) has been recently tested against RMS; however, the precise mechanism of action of TMZ in RMS remains unclear. Here we demonstrate that TMZ decreases the cell viability of the RH30 RMS and C2C12 cell line, where cells display evidence of mitochondrial outer membrane permeability. Read More

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October 2018

Antitumor profile of the PI3K inhibitor ZSTK474 in human sarcoma cell lines.

Oncotarget 2018 Oct 12;9(80):35141-35161. Epub 2018 Oct 12.

Division of Molecular Pharmacology, Cancer Chemotherapy Center, Japanese Foundation for Cancer Research, Tokyo, Japan.

Treatment of patients with advanced sarcoma remains challenging due to lack of effective medicine, with the development of novel drugs being of keen interest. A pan-PI3K inhibitor, ZSTK474, has been evaluated in clinical trials against a range of advanced solid tumors, with clinical benefit shown in sarcoma patients. In the present study, we developed a panel of 14 human sarcoma cell lines and investigated the antitumor effect of 24 anticancer agents including ZSTK474, other PI3K inhibitors, and those clinically used for sarcoma treatment. Read More

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October 2018
5 Reads

Prognostic factors impacting survival in early stage uterine carcinosarcoma.

Gynecol Oncol 2018 Nov 8. Epub 2018 Nov 8.

Department of Gynecologic Oncology and Reproductive Sciences, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States of America. Electronic address:

Objective: Evaluate the impact of clinicopathologic characteristics and adjuvant treatment on survival outcomes in early stage uterine carcinosarcoma patients.

Methods: We performed a retrospective cohort study of women with stage I or II uterine carcinosarcoma at our institution between March 1990 and June 2016. All pathology had been reviewed and confirmed by gynecologic pathologists. Read More

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November 2018
2 Reads

Status of programmed death-ligand 1 expression in sarcomas.

J Transl Med 2018 Nov 6;16(1):303. Epub 2018 Nov 6.

Department of Health Sciences and Technology, SAIHST, Sungkyunkwan University, Seoul, South Korea.

Background: Sarcomas are challenging to study because of their rarity and histomorphological complexity. PD1 and PD-L1 inhibitors showed a promising anti-tumor effect in solid tumors, where a relationship between PD-L1 expression and the objective response has been evidenced.

Methods: In this study, we examined PD-L1 expression in 16 bone and soft tissue sarcoma cell lines of 11 different subtypes by means of western blot, flow cytometry and immunocytochemistry, and in 230 FFPE patient-derived tumor tissues by means of immunohistochemistry using three different antibody clones. Read More

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November 2018
5 Reads

Laparoscopic Radical Trachelectomy for Embryonal Rhabdomyosarcoma of the Cervix in a 2-Year-Old Girl.

Obstet Gynecol 2018 Dec;132(6):1486-1490

Division of Gynecologic Oncology, Department of Surgery and Surgical Oncology, University Health Network, Princess Margaret Cancer Centre, the Department of Obstetrics and Gynecology, University of Toronto, and the Section of Pediatric Gynecology, Division of Endocrinology, the Division of Pediatric Urology, Department of Surgery, and the Division of Hematology/Oncology, Hospital for Sick Children, Toronto, Ontario, Canada.

Background: Embryonal rhabdomyosarcoma of the cervix is a rare and aggressive malignancy that usually affects children and young adults.

Case: We describe a 2-year-old patient who presented with a mass protruding through the vaginal introitus. Preoperative investigations including vaginoscopy, hysteroscopy, magnetic resonance imaging, and biopsies confirmed embryonal rhabdomyosarcoma, botryoid subtype, arising from the cervix. Read More

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December 2018
5 Reads

Sentinel lymph node biopsy in head and neck rhabdomyosarcoma.

Pediatr Blood Cancer 2018 Nov 4:e27532. Epub 2018 Nov 4.

Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Head and neck rhabdomyosarcoma lymph node staging is challenging due to varied patterns of lymphatic drainage and the suboptimal predictive value of available imaging modalities. Furthermore, regional relapse rates are unacceptably high, and the toxicity of empiric radiation is undesirable in the pediatric and young adult population. In an attempt to improve locoregional control without excess morbidity, we have adopted routine sentinel lymph node biopsy in head and neck rhabdomyosarcoma, which is safe and feasible in pediatric patients. Read More

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November 2018
10 Reads

A rare pure embryonal rhabdomyosarcoma of the urinary bladder in an adult successfully managed with neoadjuvant chemotherapy and surgery: a case report.

J Med Case Rep 2018 Nov 4;12(1):329. Epub 2018 Nov 4.

Department of Pathology, University Hospital Centre Hassan II, Fez, Morocco.

Background: Rhabdomyosarcoma of the urinary bladder in adults is an extremely rare malignant neoplasm that develops from the bladder wall.

Case Presentation: We report our experience of a rare case of rhabdomyosarcoma of the bladder in a 45-year-old Moroccan man who was successfully managed with neoadjuvant chemotherapy and surgical excision of the mass; he was disease free at 24-month follow up.

Conclusions: To the best of our knowledge, this is the first reported case of a rare embryonal rhabdomyosarcoma of the bladder that was managed with neoadjuvant chemotherapy and surgery. Read More

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November 2018
4 Reads

NS-065/NCNP-01: An Antisense Oligonucleotide for Potential Treatment of Exon 53 Skipping in Duchenne Muscular Dystrophy.

Mol Ther Nucleic Acids 2018 Sep 27;13:442-449. Epub 2018 Sep 27.

Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Tokyo, Japan. Electronic address:

Duchenne muscular dystrophy (DMD), the most common lethal heritable childhood disease, is caused by mutations in the DMD gene that result in the absence of functional dystrophin protein. Exon skipping mediated by antisense oligonucleotides has recently emerged as an effective approach for the restoration of dystrophin, and skipping of exon 51 of DMD has received accelerated approval. Identifying antisense sequences that can provide the highest possible skipping efficiency is crucial for future clinical applications. Read More

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September 2018
1 Read

Extracellular multivesicular bodies in tissues affected by inflammation/repair and tumors.

Ultrastruct Pathol 2018 Sep-Oct;42(5):448-457

a Department of Basic Medical Sciences (Anatomy, Pathology and Histology) , University of La Laguna , Tenerife , Spain.

Extracellular vesicles (EVs) are a heterogeneous population involved in intercellular communication. Little attention has been paid to a peculiar EV type with the appearance of a multivesicular body: extracellular multivesicular body (EMVB), also termed matrix vesicle cluster/multivesicular cargo. The aim of this work is to assess the ultrastructural characteristics, participation, and tissue location of EMVBs in inflammation/repair and tumors (with physiopathological processes involving intense intercellular communication), for which representative specimens were used. Read More

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November 2018
1 Read

Sarcomas of the Oral and Maxillofacial Region: Analysis of 26 Cases with Emphasis on Diagnostic Challenges.

Pathol Oncol Res 2018 Oct 31. Epub 2018 Oct 31.

Department of Oral Medicine & Radiology, Maulana Azad Institute of Dental Sciences, New Delhi, India.

Sarcomas of the Oral and Maxillofacial Region (SOMR) are rare lesions which pose diagnostic and management challenges. We analyzed 26 cases of SOMR with respect to clinical presentation, histopathological subtype, treatment modalities, recurrence, and treatment outcome. In our series, Osteosarcoma (OS) was the most common type of sarcoma (7 cases), followed by 5 cases of Ewing's Sarcoma (ES), 3 cases each of Chondrosarcoma (CS) and Leiomyosarcoma (LMS), 2 cases each of Malignant Peripheral Nerve Sheath Tumor (MPNST), Pleomorphic Undifferentiated Sarcoma (PUS), Myeloid Sarcoma (MS)and Rhabdomyosarcoma (RMS). Read More

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October 2018
4 Reads

NKX2.2 immunohistochemistry in the distinction of Ewing sarcoma from cytomorphologic mimics: Diagnostic utility and pitfalls.

Cancer Cytopathol 2018 Nov 30;126(11):942-949. Epub 2018 Oct 30.

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

Background: Ewing sarcoma (ES) is a round cell sarcoma that can be challenging to diagnose on cytologic material given its significant overlap with numerous mesenchymal, epithelial, and lymphoid cytomorphologic mimics. The objective of this study was to assess the utility of a novel marker, NKX2.2, in the diagnosis of ES in cytologic material and its ability to distinguish ES from its mimics. Read More

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November 2018
3 Reads

Therapeutic Approaches Targeting PAX3-FOXO1 and Its Regulatory and Transcriptional Pathways in Rhabdomyosarcoma.

Molecules 2018 Oct 28;23(11). Epub 2018 Oct 28.

Rhabdomyosarcoma (RMS) is a family of soft tissue cancers that are related to the skeletal muscle lineage and predominantly occur in children and young adults. A specific chromosomal translocation t(2;13)(q35;q14) that gives rise to the chimeric oncogenic transcription factor PAX3-FOXO1 has been identified as a hallmark of the aggressive alveolar subtype of RMS. PAX3-FOXO1 cooperates with additional molecular changes to promote oncogenic transformation and tumorigenesis in various human and murine models. Read More

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October 2018
5 Reads
2.420 Impact Factor

What is your diagnosis? Dermal mass over the ribs with metastasis to lymph nodes in a dog.

Vet Clin Pathol 2018 Oct 29. Epub 2018 Oct 29.

College of Veterinary Medicine, University of Illinois, Urbana, Illinois.

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October 2018

Wilms Tumor Associated With the 9q22.3 Microdeletion Syndrome: 2 New Case Reports and a Review of The Literature.

J Pediatr Hematol Oncol 2018 Oct 26. Epub 2018 Oct 26.

Paediatric Urology, The Royal Children's Hospital.

Background: The 9q22.3 syndrome is an autosomal dominant microdeletion syndrome with similarities to Gorlin syndrome (GS). It encompasses the PTCH1 gene locus that harbors mutations for GS. Read More

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October 2018
6 Reads

Delayed Return of Ejaculatory Function in Adolescent Males Treated With Retroperitoneal Lymph Node Dissection and Adjuvant Therapy for Paratesticular Rhabdomyosarcoma.

Urology 2018 Oct 23. Epub 2018 Oct 23.

Department of Urology, Boston Children's Hospital, Boston, MA; Department of Surgery, Harvard Medical School, Boston, MA. Electronic address:

Due to the rarity of the disease, adverse events related to ejaculatory function following the management of paratesticular rhabdomyosarcoma with multimodal therapy in adolescents are rarely discussed. Two patients, age 14 and 15 at time of diagnosis were treated with multimodal therapy with nerve-sparing retroperitoneal lymph node dissection, chemotherapy, and radiotherapy. Each developed ejaculatory dysfunction during the treatment period, which resolved 1 year after completion of all therapies. Read More

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October 2018
1 Read

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

Oncol Rep 2019 Jan 22;41(1):643-649. Epub 2018 Oct 22.

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

Rhabdomyosarcoma (RMS) is an aggressive pediatric cancer of musculoskeletal origin. Despite multidisciplinary approaches, such as surgical resection, irradiation, and intensive chemotherapy, adopted for its treatment, the prognosis of patients with high‑risk RMS remains poor. Thus, molecularly targeted therapies are required to improve patient survival and minimize side effects. Read More

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January 2019
1 Read

Oncolytic Virus-Mediated RAS Targeting in Rhabdomyosarcoma.

Mol Ther Oncolytics 2018 Dec 15;11:52-61. Epub 2018 Sep 15.

Department of Pathology, University of Washington, Seattle, WA 98195, USA.

Aberrant activation of the receptor tyrosine kinase-mediated RAS signaling cascade is the primary driver of embryonal rhabdomyosarcoma (ERMS), a pediatric cancer characterized by a block in myogenic differentiation. To investigate the cellular function of activated RAS signaling in regulating the growth and differentiation of ERMS cells, we genetically ablated activated oncogenes with high-efficiency genome-editing technology. Knockout of in CRISPR-inducible ERMS xenograft models resulted in near-complete tumor regression through a combination of cell death and myogenic differentiation. Read More

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December 2018
4 Reads

[Neglected scrotal trauma revealing embryonic paratesticular rhabdomyosarcoma: about a case].

Pan Afr Med J 2018 11;30:107. Epub 2018 Jun 11.

Service d'Urologie, CHU Hassan II de Fès, Fès, Maroc.

Embryonic paratesticular rhabdomyosarcoma is a rare mesenchymal tumor constituting a diagnostic and therapeutic emergency. Localized forms have a favorable prognosis. Multimodal treatment is the gold standard and it is based on surgery, multidrug chemotherapy and radiotherapy, with excellent overall survival rate. Read More

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November 2018
6 Reads

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy: A safe treatment option for intraperitoneal rhabdomyosarcoma in children below 5 years of age.

Pediatr Blood Cancer 2018 Oct 26:e27517. Epub 2018 Oct 26.

Department of Pediatric Surgery, University Children's Hospital, Tuebingen, Germany.

Advanced and relapsed intraperitoneal rhabdomyosarcomas in young children represent an oncological challenge and options for local tumor control are limited. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is commonly used in advanced peritoneal tumors in adults. However, no studies are available regarding CRS and HIPEC in young children. Read More

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October 2018
3 Reads

PARP inhibitors affect growth, survival and radiation susceptibility of human alveolar and embryonal rhabdomyosarcoma cell lines.

J Cancer Res Clin Oncol 2018 Oct 24. Epub 2018 Oct 24.

Department of Paediatrics, "Sapienza" University of Rome, Viale Regina Elena 324, 00161, Rome, Italy.

Purpose: PARP inhibitors (PARPi) are used in a wide range of human solid tumours but a limited evidence is reported in rhabdomyosarcoma (RMS), the most frequent childhood soft-tissue sarcoma. The cellular and molecular effects of Olaparib, a specific PARP1/2 inhibitor, and AZD2461, a newly synthesized PARP1/2/3 inhibitor, were assessed in alveolar and embryonal RMS cells both as single-agent and in combination with ionizing radiation (IR).

Methods: Cell viability was monitored by trypan blue exclusion dye assays. Read More

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October 2018
8 Reads
3.081 Impact Factor

Analysis of the relationship between the KRAS G12V oncogene and the Hippo effector YAP1 in embryonal rhabdomyosarcoma.

Sci Rep 2018 Oct 23;8(1):15674. Epub 2018 Oct 23.

University of Aberdeen, School of Medicine, Medical Sciences and Nutrition, Foresterhill, Aberdeen, AB25 2ZD, Scotland.

Persistent hyperactivity of the Hippo effector YAP in activated satellite cells is sufficient to cause embryonal rhabdomyosarcoma (ERMS) in mice. In humans, YAP is abundant and nuclear in the majority of ERMS cases, and high YAP expression is associated with poor survival. However, YAP1 is rarely mutated in human ERMS. Read More

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October 2018

Chemoresistant pleomorphic rhabdomyosarcoma: whole exome sequencing reveals underlying cancer predisposition and therapeutic options.

J Med Genet 2018 Oct 23. Epub 2018 Oct 23.

Assistance Publique Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Génétique, Paris, France.

Background: Rhabdomyosarcoma (RMS) is rare cancer affecting children and adults. Pleomorphic RMS histology is almost exclusive to adult patients and often resistant to chemotherapy.

Objective: We report the case of a 19-year-old patient who presented with a metastatic chemoresistant pleomorphic RMS. Read More

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October 2018
2 Reads

Demographic and Treatment Variables Influencing Outcome for Localized Paratesticular Rhabdomyosarcoma: Results From a Pooled Analysis of North American and European Cooperative Groups.

J Clin Oncol 2018 Oct 23:JCO2018789388. Epub 2018 Oct 23.

David O. Walterhouse, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL; Donald A. Barkauskas, University of Southern California, Los Angeles; David Hall, Children's Oncology Group, Monrovia, CA; Andrea Ferrari, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Istituto Nazionale Tumori, Milan; Gian Luca De Salvo, Istituto Oncologico Veneto Istituto di Ricovero e Cura a Carattere Scientifico, Padua; Gianni Bisogno, University of Padua, Padova, Italy; Ewa Koscielniak, Olgahospital, Stuttgart; Guido Seitz, University Hospital, Marburg, Germany; Michael C.G. Stevens, Royal Hospital for Children, Bristol, United Kingdom; Hélène Martelli, Assistance Publique-Hôpitaux de Paris, Hopital Bicetre, Le Kremlin-Bicetre; Christophe Bergeron, Centre Léon Bérard, Lyon; Veronique Minard-Colin, Gustave Roussy, Villejuif, France; David A. Rodeberg, East Carolina University, Greenville, NC; Margarett Shnorhavorian and Douglas S. Hawkins, Seattle Children's Hospital, University of Washington; Douglas S. Hawkins, Fred Hutchinson Cancer Research Center, University of Washington, Seattle, WA; Roshni Dasgupta and John C. Breneman, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; James R. Anderson, Merck Research Laboratories, North Wales, PA; and William H. Meyer, University of Oklahoma School of Medicine, Oklahoma City, OK.

Purpose: Treatment recommendations for localized paratesticular rhabdomyosarcoma (PT RMS) differ in North America and Europe. We conducted a pooled analysis to identify demographic features and treatment choices that affect outcome.

Patients And Methods: We retrospectively analyzed the effect of nine demographic variables and four treatment choices on event-free survival (EFS) and overall survival (OS) from 12 studies conducted by five cooperative groups. Read More

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October 2018
5 Reads