Sinonasal Sarcomas Management: An International Consensus Statement

Alessandro Vinciguerra¹, Francesca Caspani², Marco Valentini¹, Anna Maria Camarda^{3

4}, Salvatore Provenzano⁵, Eric W Wang⁶, Cem Meço^{7

8

9}, Marco Ferrari^{10

11}, Pavol Surda¹², Semi Harrabi¹³, Fernando Augusto Batista Campos¹⁴, Javier Martin Broto^{15

16}, B Ashleigh Guadagnolo¹⁷, Pierina Navarria¹⁸, Ali Hosni¹⁹, Nadia Hindi^{15

16}, Antoine Italiano²⁰, Daniel M Trifiletti²¹, Bernd Kasper²², Benjamin Verillaud²³, Alberto Schreiber²⁴, Paolo Castelnuovo¹, Piero Nicolai^{10

11}, Iacopo Dallan²⁵, Matias Chacon²⁶, Shirley Y Su²⁷, Juliette Thariat^{28

29}, Claudia Valverde³⁰, Matt Lechner³¹, Jean Yves Blay³², Maria Rosaria Fiore^{3

4}, Jean Anderson Eloy³³, Mehdi Brahmi³², James N Palmer³⁴, Victor Castro Oliden³⁵, Daniela Greto³⁶, Iwona Lugowska³⁷, Angelo Paolo Dei Tos³⁸, Thibaut van Zele³⁹, Alkis J Psaltis⁴⁰, Paolo Giovanni Casali⁵, Armelle Dufresne³², Claudia Sangalli⁴¹, Christos Georgalas⁴², Ester Orlandi^{3

4}, Peter-John Wormald⁴⁰, Philippe Herman²³, Ricardo Carrau⁴³, Ehab Y Hanna²⁷, Lisa Licitra⁵, Mario Turri-Zanoni¹, Paolo Battaglia¹

Affiliations

¹ Division of Otorhinolaryngology, Department of Biotechnology and Life Sciences, University of Insubria, ASST Lariana, Como, Italy.
² Head and Neck Medical Oncology Department, ASST Lariana, Como, Italy.
³ Radiation Oncology Clinical Department, National Center for Oncological Hadrontherapy (CNAO), Pavia, Italy.
⁴ Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences, University of Pavia, Pavia, Italy.
⁵ Department of Oncology and Hematology, Fondazione IRCCS Istituto Nazionale Dei Tumori, Milan, Italy.
⁶ Department of Otolaryngology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
⁷ Department of Otorhinolaryngology - Head and Neck Surgery, Ankara University Medical School, Ankara, Turkiye.
⁸ Department of Otorhinolaryngology - Head and Neck Surgery, Salzburg Paracelsus Medical University, Salzburg, Austria.
⁹ Department of Otolaryngology - Head and Neck Surgery, Cornell University, Weill Cornell Medical College, New York, New York, USA.
¹⁰ Section of Otorhinolaryngology - Head and Neck Surgery, Department of Neuroscience (DNS), University of Padova, Padova, Italy.
¹¹ Unit of Otorhinolaryngology - Head and Neck Surgery, "Azienda Ospedale-Università Padova", Padova, Italy.
¹² Otolaryngology, Guy's and St Thomas' Hospitals NHS Trust, London, UK.
¹³ Heidelberg Ion-Beam Therapy Center (HIT), Department of Radiation Oncology, Heidelberg University Hospital, Heidelberg, Germany.
¹⁴ Department of Medical Oncology, A.C.Camargo Cancer Center, São Paulo, Brazil.
¹⁵ Medical Oncology Department, Fundacion Jimenez Diaz University Hospital, University Hospital General De Villalba, Madrid, Spain.
¹⁶ Instituto De Investigacion Sanitaria Fundacion Jimenez Diaz (IIS/FJD; UAM), Madrid, Spain.
¹⁷ Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
¹⁸ Department of Radiotherapy and Radiosurgery, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy.
¹⁹ Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada.
²⁰ Faculty of Medicine, University of Bordeaux, Bordeaux, France.
²¹ Department of Radiation Oncology, Mayo Clinic, Jacksonville, Florida, USA.
²² Sarcoma Unit, University of Heidelberg, Mannheim Cancer Center (MCC), Mannheim University Medical Center, Mannheim, Germany.
²³ Otorhinolaryngology and Skull Base Center, AP-HP, Hôpital Lariboisière, Université De Paris, Paris, France.
²⁴ Unit of Otorhinolaryngology, Manerbio and Desenzano Hospitals, ASST Del Garda, Brescia, Italy.
²⁵ Skull Base and Rhino-orbital Surgery Unit, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.
²⁶ Department of Medical Oncology, Alexander Fleming Cancer Institute, Buenos Aires, Argentina.
²⁷ Department of Head and Neck Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
²⁸ Department of Radiation Oncology, Centre François-Baclesse, Caen, France.
²⁹ Université De Normandie, Caen, France.
³⁰ Department of Medical Oncology, Vall D'hebron University Hospital, Barcelona, Spain.
³¹ Division of Surgery and Interventional Science and UCL Cancer Institute, London, UK.
³² Medical Oncology Department, Centre Leon Berard, Lyon, France.
³³ Department of Otolaryngology, Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA.
³⁴ Department of Otorhinolaryngology, Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
³⁵ Department of Oncology, Instituto Nacional de Enfermedades Neoplásicas, Lima, Peru.
³⁶ Azienda Ospedaliero-Universitaria Careggi, Radiation Oncology Unit, Florence, Italy.
³⁷ Maria Sklodowska-Curie Institute of Oncology Warsaw, Warszawa, Poland.
³⁸ Surgical Pathology and Cytopathology Unit, Department of Medicine- DIMED, University of Padua School of Medicine, Padua, Italy.
³⁹ Department of Otorhinolaryngology, Ghent University Hospital, Ghent, Belgium.
⁴⁰ Department of Otolaryngology, Head and Neck Surgery, Queen Elizabeth Hospital, Adelaide, South Australia, Australia.
⁴¹ Department of Radiation Therapy, Fondazione IRCCS Istituto Nazionale Dei Tumori, Milan, Italy.
⁴² Department of Otorhinolaryngology, Head and Neck Surgery, University of Nicosia Medical School, Nicosia, Cyprus.
⁴³ Department of Otolaryngology, Head and Neck Surgery, The Ohio State University, Columbus, Ohio, USA.

PMID: 41025537
DOI: 10.1002/alr.70038

Free article

Sinonasal Sarcomas Management: An International Consensus Statement

Alessandro Vinciguerra et al. Int Forum Allergy Rhinol. 2025.

Free article

. 2025 Sep 30.

doi: 10.1002/alr.70038. Online ahead of print.

Authors

Affiliations

¹ Division of Otorhinolaryngology, Department of Biotechnology and Life Sciences, University of Insubria, ASST Lariana, Como, Italy.
² Head and Neck Medical Oncology Department, ASST Lariana, Como, Italy.
³ Radiation Oncology Clinical Department, National Center for Oncological Hadrontherapy (CNAO), Pavia, Italy.
⁴ Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences, University of Pavia, Pavia, Italy.
⁵ Department of Oncology and Hematology, Fondazione IRCCS Istituto Nazionale Dei Tumori, Milan, Italy.
⁶ Department of Otolaryngology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
⁷ Department of Otorhinolaryngology - Head and Neck Surgery, Ankara University Medical School, Ankara, Turkiye.
⁸ Department of Otorhinolaryngology - Head and Neck Surgery, Salzburg Paracelsus Medical University, Salzburg, Austria.
⁹ Department of Otolaryngology - Head and Neck Surgery, Cornell University, Weill Cornell Medical College, New York, New York, USA.
¹⁰ Section of Otorhinolaryngology - Head and Neck Surgery, Department of Neuroscience (DNS), University of Padova, Padova, Italy.
¹¹ Unit of Otorhinolaryngology - Head and Neck Surgery, "Azienda Ospedale-Università Padova", Padova, Italy.
¹² Otolaryngology, Guy's and St Thomas' Hospitals NHS Trust, London, UK.
¹³ Heidelberg Ion-Beam Therapy Center (HIT), Department of Radiation Oncology, Heidelberg University Hospital, Heidelberg, Germany.
¹⁴ Department of Medical Oncology, A.C.Camargo Cancer Center, São Paulo, Brazil.
¹⁵ Medical Oncology Department, Fundacion Jimenez Diaz University Hospital, University Hospital General De Villalba, Madrid, Spain.
¹⁶ Instituto De Investigacion Sanitaria Fundacion Jimenez Diaz (IIS/FJD; UAM), Madrid, Spain.
¹⁷ Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
¹⁸ Department of Radiotherapy and Radiosurgery, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy.
¹⁹ Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada.
²⁰ Faculty of Medicine, University of Bordeaux, Bordeaux, France.
²¹ Department of Radiation Oncology, Mayo Clinic, Jacksonville, Florida, USA.
²² Sarcoma Unit, University of Heidelberg, Mannheim Cancer Center (MCC), Mannheim University Medical Center, Mannheim, Germany.
²³ Otorhinolaryngology and Skull Base Center, AP-HP, Hôpital Lariboisière, Université De Paris, Paris, France.
²⁴ Unit of Otorhinolaryngology, Manerbio and Desenzano Hospitals, ASST Del Garda, Brescia, Italy.
²⁵ Skull Base and Rhino-orbital Surgery Unit, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.
²⁶ Department of Medical Oncology, Alexander Fleming Cancer Institute, Buenos Aires, Argentina.
²⁷ Department of Head and Neck Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
²⁸ Department of Radiation Oncology, Centre François-Baclesse, Caen, France.
²⁹ Université De Normandie, Caen, France.
³⁰ Department of Medical Oncology, Vall D'hebron University Hospital, Barcelona, Spain.
³¹ Division of Surgery and Interventional Science and UCL Cancer Institute, London, UK.
³² Medical Oncology Department, Centre Leon Berard, Lyon, France.
³³ Department of Otolaryngology, Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA.
³⁴ Department of Otorhinolaryngology, Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
³⁵ Department of Oncology, Instituto Nacional de Enfermedades Neoplásicas, Lima, Peru.
³⁶ Azienda Ospedaliero-Universitaria Careggi, Radiation Oncology Unit, Florence, Italy.
³⁷ Maria Sklodowska-Curie Institute of Oncology Warsaw, Warszawa, Poland.
³⁸ Surgical Pathology and Cytopathology Unit, Department of Medicine- DIMED, University of Padua School of Medicine, Padua, Italy.
³⁹ Department of Otorhinolaryngology, Ghent University Hospital, Ghent, Belgium.
⁴⁰ Department of Otolaryngology, Head and Neck Surgery, Queen Elizabeth Hospital, Adelaide, South Australia, Australia.
⁴¹ Department of Radiation Therapy, Fondazione IRCCS Istituto Nazionale Dei Tumori, Milan, Italy.
⁴² Department of Otorhinolaryngology, Head and Neck Surgery, University of Nicosia Medical School, Nicosia, Cyprus.
⁴³ Department of Otolaryngology, Head and Neck Surgery, The Ohio State University, Columbus, Ohio, USA.

PMID: 41025537
DOI: 10.1002/alr.70038

Abstract

Introduction: Sinonasal sarcomas are exceedingly rare entities, constituting less than 7% of head and neck sarcomas. Their complex histology needs specialized treatment, which is often based on multimodal approaches including surgery, radiation therapy, and/or chemotherapy. This manuscript aims to gather expert opinions to establish common management principles for sinonasal sarcomas.

Methods: This international consensus followed a modified Delphi method in seven steps, including statements definition by the core group, expert panel recruitment, and a two-round survey. Sixty-two statements on sinonasal sarcoma management were developed. Experts from multiple continents participated, and results were anonymized and analyzed between March and May 2025.

Results: A total of 44 invited experts were recruited, 43.2% otorhinolaryngologists/head and neck surgeons, 31.8% medical oncologists, and 25% radiation oncologists. Participants varied in age and experience, representing Europe (70.5%), North America (18.2%), South America (6.8%), and Asia (4.5%). Among all histologies, biphenotypic sarcoma, chondrosarcoma, leiomyosarcoma, and myofibrosarcoma are principally treated with an upfront surgical management, differently from Ewing sarcoma and rhabdomyosarcoma in which chemotherapy, eventually associated with radiotherapy, is often chosen. In the remaining histologies (angiosarcoma, liposarcoma, malignant peripheral nerve sheath tumor [MPNST], osteosarcoma, and synovial sarcoma), a precise multimodal treatment is less standardized and needs to be discussed on a case-by-case basis.

Conclusion: Sinonasal sarcomas require a histology-driven approach to determine upfront treatment, whether surgical, medical, or multimodal. Despite this structured strategy, prognosis remains highly variable across subtypes. Multidisciplinary evaluation and individualized management in referral centers are crucial to address the biological diversity and anatomical complexity of these rare malignancies.

Keywords: Ewing sarcoma; angiosarcoma; biphenotypic sinonasal sarcoma; chondrosarcoma; leiomyosarcoma; malignant peripheral nerve sheath tumor; osteosarcoma; rhabdomyosarcoma; sinonasal sarcomas; synovial sarcoma.

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References

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Sinonasal Sarcomas Management: An International Consensus Statement

Affiliations

Sinonasal Sarcomas Management: An International Consensus Statement

Authors

Affiliations

Abstract

References

LinkOut - more resources

Full Text Sources