Ultra‐rare sarcomas: A consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities

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Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology

Margaret von Mehren, R. Randall, Robert Benjamin … (2018)

Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis, staging, and treatment of STS of the extremities, superficial trunk, or head and neck; outlines treatment recommendations by disease stage; and reviews the evidence to support the guidelines recommendations.

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Bone sarcomas: ESMO–PaedCan–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up†

P G Casali, S. Bielack, N Abecassis … (2018)

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Pexidartinib versus placebo for advanced tenosynovial giant cell tumour (ENLIVEN): a randomised phase 3 trial

William D. Tap, Hans Gelderblom, Emanuela Palmerini … (2019)

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Author and article information

Contributors

Silvia Stacchiotti: (View ORCID Profile)

Anna Maria Frezza: (View ORCID Profile)

Jean‐Yves Blay: (View ORCID Profile)

Sylvie Bonvalot: (View ORCID Profile)

Dario Callegaro: (View ORCID Profile)

Abha Gupta: (View ORCID Profile)

Rick L. Haas: (View ORCID Profile)

Bernd Kasper: (View ORCID Profile)

Akira Kawai: (View ORCID Profile)

David G. Kirsch: (View ORCID Profile)

Shreyaskumar R. Patel: (View ORCID Profile)

Damon R. Reed: (View ORCID Profile)

Inga‐Marie Schaefer: (View ORCID Profile)

Andrew J. Wagner: (View ORCID Profile)

Young‐Joo Won: (View ORCID Profile)

Journal

Title: Cancer

Abbreviated Title: Cancer

Publisher: Wiley

ISSN (Print): 0008-543X

ISSN (Electronic): 1097-0142

Publication date (Electronic): April 28 2021

Affiliations

[1 ]Department of Medical Oncology National Cancer Institute of Milan Milan Italy

[2 ]Leon Berard Center Claude Bernard University Lyon 1, UNICANCER Hospital Network Lyon France

[3 ]Department of Radiation Oncology Dana‐Farber/Brigham and Women's Cancer Center Boston Massachusetts

[4 ]Department of Surgical Oncology Curie Institute, University of Paris–Sciences and Letters Paris France

[5 ]Department of Pathology Leiden University Medical Center Leiden Netherlands

[6 ]Department of Surgery National Cancer Institute of Milan Milan Italy

[7 ]Taiwan Cancer Registry Center Taipei Taiwan

[8 ]Institute of Epidemiology and Preventive Medicine College of Public Health National Taiwan University Taipei Taiwan

[9 ]Department of Medical Oncology Ludwig Center at Harvard Medical SchoolDana‐Farber Cancer Institute Boston Massachusetts

[10 ]Department of Pathobiology and Laboratory Medicine University of Toronto/Mount Sinai Hospital Toronto Ontario Canada

[11 ]Department of Medical Oncology Peter MacCallum Cancer Center Melbourne Victoria Australia

[12 ]Department of Oncology Skane University Hospital and Lund University Lund Sweden

[13 ]Department of Medical Oncology Leiden University Medical Center Leiden Netherlands

[14 ]Department of Medicine Memorial Sloan Kettering Cancer Center New York New York

[15 ]Weill Cornell Medical College New York New York

[16 ]Division of Hematology/Oncology The Hospital for Sick Children Toronto Ontario Canada

[17 ]Princess Margaret Cancer Center Toronto Ontario Canada

[18 ]Department of Radiotherapy Netherlands Cancer Institute Amsterdam Netherlands

[19 ]Department of Radiotherapy Leiden University Medical Center Amsterdam Netherlands

[20 ]Department of Surgery the Royal Marsden National Health Service Foundation Trust London United Kingdom

[21 ]Sarcoma Unit, Mannheim University Medical Center University of Heidelberg Mannheim Germany

[22 ]Department of Orthopedics University of Utah Salt Lake City Utah

[23 ]Sarcoma Unit Royal Marsden NHS Foundation Trust/Institute of Cancer Research London UK

[24 ]Department of Musculoskeletal Oncology National Cancer Center Hospital Tokyo Japan

[25 ]Department of Radiation Oncology Duke University Medical Center Durham North Carolina

[26 ]Division of Pediatrics The University of Texas MD Anderson Cancer Center Houston Texas

[27 ]Medical Oncology Gustave Roussy Institute Villejuif, Ile‐de‐France France

[28 ]Division of Cancer Registration and Surveillance National Cancer Center, Ilsandong‐gu Goyang‐si Republic of Korea

[29 ]Department of Epidemiology, Regional Health Council, Biomedical Research Institute of Murcia‐Arrixaca Murcia University Murcia Spain

[30 ]Unit of Oncogenetics and Functional Oncogenomics Aviano IRCCS Oncology Referral Center Aviano Italy

[31 ]Epidemiology Unit and Girona Cancer Registry, Oncology Coordination Plan Department of Health Autonomous Government of Catalonia Catalan Institute of Oncology Girona Spain

[32 ]Medical Oncology Department University Hospital Virgen del Rocio Sevilla Spain

[33 ]National Cancer Registry Section, Center for Cancer Registries, Center for Cancer Control and Information Services, National Cancer Center Tokyo Japan

[34 ]Department of Sarcoma Medical Oncology The University of Texas MD Anderson Cancer Center Houston Texas

[35 ]Department of Surgery Brigham and Women's Hospital Boston Massachusetts

[36 ]Center for Sarcoma and Bone Oncology Dana‐Farber Cancer Institute Harvard Medical School Boston Massachusetts

[37 ]Department of Interdisciplinary Cancer Management Moffitt Cancer Center Tampa Florida

[38 ]Department of Soft Tissue/Bone Sarcoma and Melanoma Maria Sklodowska‐Curie National Research Institute of Oncology Warsaw Poland

[39 ]Department of Pathology University Hospital of Padova Padova Italy

[40 ]Department of Pathology Brigham and Women's HospitalHarvard Medical School Boston Massachusetts

[41 ]Department of Oncology the Norwegian Radium HospitalOslo University Hospital Oslo Norway

[42 ]Garvan Institute of Medical Research Sydney New South Wales Australia

[43 ]Department of Medical Oncology Netherlands Cancer Institute Amsterdam Amsterdam Netherlands

[44 ]Department of Surgical Oncology Netherlands Cancer Institute Amsterdam Netherlands

[45 ]Department of Registration Netherlands Comprehensive Cancer Organization Utrecht Netherlands

[46 ]Department of Hematology and Medical Oncology Fox Chase Cancer Center Philadelphia Pennsylvania

[47 ]Department of Medicine University of Colorado Cancer Center Aurora Colorado

[48 ]Evaluative Epidemiology Unit Department of Research National Cancer Institute of Milan Milan Italy

Article

DOI: 10.1002/cncr.33618

PubMed ID: 33910263

SO-VID: 2cdb7a44-4ba4-457d-9963-918cce630ce0

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Ultra‐rare sarcomas: A consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities

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Most cited references 24

Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology

Bone sarcomas: ESMO–PaedCan–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up†

Pexidartinib versus placebo for advanced tenosynovial giant cell tumour (ENLIVEN): a randomised phase 3 trial

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Cited by 50

Most referenced authors 779