Bulletin Du Cancer最新文献

{"title":"Recommandations pour la prise en charge des ostéosarcomes de la mandibule de l’enfant, l’adolescent et l’adulte","authors":"Morbize Julieron , Anne Gomez-Mascard , Cécile Vérité , Maud Toulmonde , Cyril Lervat , Michèle Kind , Juliette Thariat","doi":"10.1016/j.bulcan.2024.03.009","DOIUrl":"10.1016/j.bulcan.2024.03.009","url":null,"abstract":"<div><div>Les ostéosarcomes de la mandibule représentent 3 à 8 % des ostéosarcomes. La rareté de cette localisation et la spécificité de sa prise en charge expliquent que seules des études rétrospectives et quelques études prospectives sont disponibles dans la littérature. Il existe pourtant des données concordantes sur l’histoire naturelle et le traitement de ces tumeurs qui les différencient clairement des ostéosarcomes des os longs. Le but de ce travail a été d’élaborer des recommandations à partir de ces données et d’une étude rétrospective du Groupe sarcome français (GSF-GETO). Les ostéosarcomes de la mandibule doivent faire l’objet d’un prélèvement frais pour congélation et d’une relecture centralisée, par un pathologiste expert, avec recherche des statuts de <em>MDM2</em>, <em>GNAS</em> et <em>RASAL1</em>. L’exérèse chirurgicale avec de larges marges, guidées par l’imagerie par résonance magnétique, est la pierre angulaire du traitement. Les techniques de reconstruction mandibulaire permettent d’en diminuer les séquelles. Contrairement au traitement validé des ostéosarcomes des membres, la place de la chimiothérapie dans la prévention des métastases et/ou des récidives locales doit encore être précisée pour les ostéosarcomes mandibulaires. La question de la place de la radiothérapie postopératoire chez l’adulte doit être soulevée pour ces tumeurs dont la résection large, au niveau des tissus mous, peut être difficile à affirmer. Chez l’enfant, dans ces cas, on discutera plutôt la chimiothérapie adjuvante. Les rechutes des ostéosarcomes mandibulaires sont principalement locales. Les métastases pulmonaires sont retardées et moins fréquentes que pour les ostéosarcomes des os longs. La survie globale à cinq ans est d’environ 70 %.</div></div><div><div>Osteosarcomas of the mandible represent 3–8% of osteosarcomas. The rarity of this anatomic site and its specific treatment explain that only retrospective and a few prospective studies are available in literature. However, there is a consistent evidence on the natural history and treatment of these tumors, which clearly differentiates them from osteosarcomas of the long bones. The aim of this study was to draw up recommendations based on these data and on a retrospective study by the French Sarcoma Group (GSF-GETO). Osteosarcomas of the mandible should be centrally reviewed by an expert pathologist. <em>MDM2</em>, <em>GNAS</em> and <em>RASAL1</em> status should be checked, and a fragment should be frozen. Complete surgical resection with wide margins is the cornerstone of treatment. Mandibular reconstruction techniques can reduce the sequelae. Contrary to the validated treatment for osteosarcomas of limbs, the role of chemotherapy to prevent metastasis or local recurrence has yet to be clarified for mandibular osteosarcomas. The role of postoperative radiotherapy, in adults, should be discussed for these tumors, whose wide soft-tissue resection may be difficult to confirm. In children, adjuvant c","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 574-591"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143607587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Toripalimab en association avec cisplatine et gemcitabine pour le traitement des carcinomes indifférenciés du nasopharynx récidivants ou métastatique (UCNT RM) en première ligne","authors":"Thomas Cochin , Esma Saâda-Bouzid","doi":"10.1016/j.bulcan.2025.02.006","DOIUrl":"10.1016/j.bulcan.2025.02.006","url":null,"abstract":"","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 557-558"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143722996","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bone sarcomas and cancer predisposition syndromes","authors":"Camille Tlemsani , Gaëlle Bougeard , Marion Gauthier-Villars , Philippe Denizeau , Sarah Winter , Caroline Michot , Geneviève Baujat , Brigitte Bressac , Tiphaine Adam de Beaumais , Aymeric Rouchaud , Fadila Mihoubi-Bouvier , Franck Bourdeaut , Laurence Brugières , Thierry Leblanc , Edwige Kasper , Nadège Corradini , GroupOs SFCE oncogenetic's groups","doi":"10.1016/j.bulcan.2024.10.014","DOIUrl":"10.1016/j.bulcan.2024.10.014","url":null,"abstract":"<div><div>Bone sarcomas, constituting less than 1% of malignant neoplasms across all age groups, are rare tumours possibly associated with genetic susceptibility syndromes. This review aims to provide recommendations for the detection of cancer predisposition syndromes associated with bone sarcomas and managing affected patients. Recommendations were formulated by a multidisciplinary working and reviewing group from GroupOs and SFCE oncogenetic's group, including geneticists, oncologists, and radiologists. For various bone sarcomas including osteosarcomas, chondrosarcomas and Ewing sarcomas, we delineate tumour presentation, management strategies, and follow-up within the context of cancer predisposition syndromes. The inherited predisposition syndrome, associated with germline <em>TP53</em> variants, known as the Li-Fraumeni syndrome, is the most frequent implicated in osteosarcoma cases. Other cancer predisposition syndromes, such as <em>RB1</em>, <em>RECQ</em> or <em>CDKN2A</em> disorders in osteosarcomas and Ollier and Maffucci diseases in chondrosarcomas, are also recognized. Additionally, we discuss rarer cancer predisposition syndromes associated with bone sarcomas and suggest tailored treatment approaches in some cancer predisposition syndromes to mitigate severe toxicities or secondary oncological events. Furthermore, we emphasize the role of identification somatic molecular variations in identifying constitutional germline variants and describe national and international screening programs, reference networks and molecular tumour boards available for collegial and collaborative management discussion. This comprehensive review provides insights into the intricate interplay between genetic predisposition, tumour biology, and therapeutic interventions in bone sarcoma patients with cancer predisposition syndrome.</div></div><div><div>Les sarcomes osseux, représentant moins d’un pour cent des tumeurs malignes toutes catégories d’âge confondues, sont rares et possiblement associés à des syndromes de prédisposition génétique. Cette revue vise à formuler des recommandations pour la détection des syndromes de prédisposition au cancer liés aux sarcomes osseux et à la gestion des patients affectés. Élaborées par un groupe de travail multidisciplinaire du GroupOs et du groupe oncogénétique de la SFCE, comprenant des généticiens, des oncologues et des radiologues, des recommandations de diagnostic, de prise en charge et de suivi pour divers sarcomes osseux, tels que l’ostéosarcome, le chondrosarcome et le sarcome d’Ewing, dans le contexte d’un syndrome de prédisposition au cancer, ont été établies. Le syndrome de Li-Fraumeni, syndromes de prédisposition génétique le plus souvent identifié dans les ostéosarcomes, est associé aux mutations constitutionnelles du gène <em>TP53</em>. D’autres syndromes de prédisposition au cancer sont également reconnus, comme les altérations génétiques des gènes <em>RB1</em>, <em>RECQ</em> ou <em>CDKN2A</em> pour les os","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 664-680"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143030438","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erdafitinib en monothérapie dans le carcinome urothélial avancé avec altération FGFR3 après au moins une ligne de traitement avec un anti-PD-1/PD-L1","authors":"Clément Grosnon, Mathieu Jamelot","doi":"10.1016/j.bulcan.2024.12.020","DOIUrl":"10.1016/j.bulcan.2024.12.020","url":null,"abstract":"","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 566-568"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143796291","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Radiothérapie des sarcomes d’Ewing","authors":"Line Claude ,&nbsp;Sylvie Helfre ,&nbsp;Nadège Corradini ,&nbsp;Nathalie Gaspar ,&nbsp;Pablo Berlanga ,&nbsp;Laure Saumet ,&nbsp;Perrine Marec-Bérard ,&nbsp;Cyril Lervat ,&nbsp;Mickael Ropars ,&nbsp;Sophie Piperno-Neumann ,&nbsp;Pascaline Boudou-Roquette ,&nbsp;Gabriel Revon-Riviere ,&nbsp;Anne Ducassou ,&nbsp;Valentine Martin ,&nbsp;pour le GroupOs français","doi":"10.1016/j.bulcan.2024.08.019","DOIUrl":"10.1016/j.bulcan.2024.08.019","url":null,"abstract":"<div><div>Les sarcomes d’Ewing sont la deuxième cause de tumeurs osseuses malignes chez l’enfant ou l’adulte jeune. Après chimiothérapie d’induction, le traitement local est essentiel et comprend le plus souvent chirurgie de la tumeur primitive. La radiothérapie peut être nécessaire en geste local exclusif quand la chirurgie n’est pas possible, ou en situation adjuvante quand le geste chirurgical doit être complété (résection incomplète ou mauvaise réponse histologique notamment). La radiothérapie peut aussi concerner les sites métastatiques, notamment en cas de métastases pulmonaires et/ou en situation oligo-métastatique. Cet article propose une revue des indications actuelles de radiothérapie, des modalités techniques d’irradiation (délinéation des volumes, doses recommandées), et les résultats des études récentes.</div></div><div><div>Ewing sarcomas are the 2nd cause of malignant bone tumors in children and young adults. After induction chemotherapy, local treatment is essential and most often includes surgery of the primary tumor. Radiotherapy may be necessary as an exclusive local procedure when surgery is not possible, or in an adjuvant situation when the surgical procedure must be completed (incomplete resection or poor histological response in particular). Radiotherapy can also concern metastatic sites, particularly in cases of pulmonary metastases and/or in an oligo-metastatic situation. This article provides a review of current indications for radiotherapy, technical modalities of irradiation (delineation of volumes, recommended doses), and the results of recent studies.</div></div>","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 656-663"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143660099","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nivolumab et ipilimumab dans les cancers colorectaux avancés avec instabilité des microsatellites","authors":"Valentine Gangneux ,&nbsp;Stanislas Quesada","doi":"10.1016/j.bulcan.2025.02.017","DOIUrl":"10.1016/j.bulcan.2025.02.017","url":null,"abstract":"","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 562-564"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144038337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Actualisation des recommandations pour la prise en charge et le traitement des tumeurs osseuses à cellules géantes de l’adulte – Place du dénosumab","authors":"Pascaline Boudou-Rouquette ,&nbsp;Frédérique Larousserie ,&nbsp;Valérie Dumaine ,&nbsp;Anne Gomez-Mascard ,&nbsp;Valérie Bousson ,&nbsp;Axel Le Cesne ,&nbsp;Gualter Vaz ,&nbsp;Gonzague de Pinieux ,&nbsp;Gilles Missenard ,&nbsp;Christine Chevreau ,&nbsp;François Gouin","doi":"10.1016/j.bulcan.2024.01.013","DOIUrl":"10.1016/j.bulcan.2024.01.013","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Introduction&lt;/h3&gt;&lt;div&gt;Les tumeurs osseuses à cellules géantes sont des tumeurs osseuses primitives bénignes, qui présentent fréquemment une récidive locale et occasionnellement une transformation maligne en sarcome de haut grade. La chirurgie est le pilier du traitement et elle consiste généralement en un curetage intralésionnel. Le dénosumab a été approuvé par l’Agence européenne des médicaments (EMA) en 2014 pour le traitement des adultes et adolescents ayant atteint la maturité squelettique et souffrant d’une tumeur osseuse à cellules géantes non résécable ou lorsque la résection est susceptible d’entraîner une morbidité sévère.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Méthodes&lt;/h3&gt;&lt;div&gt;Sur la base de recommandations nationales établies en 2016 et à partir d’une synthèse de l’état actuel des connaissances, un groupe de travail du GroupOS s’est créé pour actualiser et élaborer des recommandations sur la prise en charge et le traitement des tumeurs osseuses à cellules géantes de l’adulte, ainsi que sur la place du dénosumab.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Résultats&lt;/h3&gt;&lt;div&gt;Nous proposons ici sept recommandations : (1) démarche diagnostique et bilan d’imagerie ; (2) principes du traitement des tumeurs osseuses à cellules géantes des membres ; (3) indications du dénosumab dans les tumeurs osseuses à cellules géantes des membres ; (4) spécificités des tumeurs osseuses à cellules géantes de localisation axiale et des tumeurs osseuses à cellules géantes multifocales ; (5) stratégie thérapeutique après reprise évolutive après traitement par dénosumab ; (6) progression sous traitement par dénosumab et suspicion de forme maligne de tumeurs osseuses à cellules géantes sous dénosumab ; (7) modalités de la surveillance.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Conclusion&lt;/h3&gt;&lt;div&gt;Il reste des questions non résolues, comme la durée optimale du traitement ou l’intervalle précis entre les doses d’entretien du dénosumab. Rarement, les tumeurs osseuses à cellules géantes subissent une transformation maligne. Aucun critère clinique, histologique ou radiographique ne permet de prédire avec précision un comportement agressif ultérieur.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Introduction&lt;/h3&gt;&lt;div&gt;Giant cell tumours (GCTs) are benign primary bone tumours that frequently present with local recurrence and occasionally malignant transformation to high-grade sarcoma. Surgery is the mainstay of treatment and generally consists of intralesional curettage. Denosumab was approved by the European Medicines Agency (EMA) in 2014 for the treatment of skeletally mature adults and adolescents with unresectable GCTs or where resection is likely to result in severe morbidity.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;On the basis of national recommendations established in 2016 and a summary of the current state of knowledge, a GroupOS working group was set up to update and develop recommendations on the management and treatment of GCTs in adults, as well as on the place of denosumab.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;Seven recommendations are p","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 626-637"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144044696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Des recommandations issues du GroupOs bienvenues","authors":"Daniel Orbach ,&nbsp;Virginie Gandemer ,&nbsp;Isabelle Pellier","doi":"10.1016/j.bulcan.2025.02.003","DOIUrl":"10.1016/j.bulcan.2025.02.003","url":null,"abstract":"","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 569-570"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143588830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary cutaneous/subcutaneous Ewings sarcoma","authors":"Thibaud Valentin ,&nbsp;Sarah Winter ,&nbsp;Valentine Martin ,&nbsp;Marie Csanyi ,&nbsp;Perrine Marec Berard ,&nbsp;Valérie Laurence ,&nbsp;Nathalie Gaspar","doi":"10.1016/j.bulcan.2023.10.011","DOIUrl":"10.1016/j.bulcan.2023.10.011","url":null,"abstract":"&lt;div&gt;&lt;div&gt;Amongst Ewing sarcoma family of tumours, (EFST), cutaneous/subcutaneous Ewing sarcoma are defined as tumours arising from cutaneous or subcutaneous tissue, not invading the underlying aponeurosis. They are rare tumours, with less than 200 patients published. They are typically small tumours (less than 5&lt;!--&gt; &lt;!--&gt;cm), and can arise at any anatomical location, with a particular tropism for distal, truncal and head/neck locations, compared to classical Ewing sarcoma. Like other conjunctive tumours, they have to be treated in specialized centers, with a diagnostic procedure following ESMO guidelines about soft-tissue tumours, favoring a core needle biopsy in most cases. They share classical pathological and molecular features of EFST (including EWSR1 rearrangement). Metastatic presentation is rare (less than 5% at diagnosis), but must be carefully searched using appropriated imaging considered the bad prognosis of these patients. Treatment strategy relies on neoadjuvant and adjuvant chemotherapy, surrounding the local treatment. Patients with localized disease have good prognosis and have to be treated with the dual objective of curability, and of minimizing acute and late toxicity. That is why in case of small tumours (&lt;200&lt;!--&gt; &lt;!--&gt;mL), patients can be treated with less intensive protocols, as Saint Jude's (low-dose semi-continuous cyclophosphamide/doxorubicin regimen as induction chemotherapy and vincristine/actinomycin courses as maintenance therapy), setting aside the option of classical VDC/IE protocol for larger tumors. Local treatment must rely on carcinologic surgery, with the aim to avoid radiotherapy when possible. Patients with metastatic disease have bad prognosis resemble classical Ewing sarcoma, and have to be treated accordingly.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;div&gt;Les tumeurs d’Ewing cutanés/sous-cutanés sont des tumeurs rares (moins de 200 patients publiés) appartiennent aux tumeurs de la famille Ewing (TE), avec lesquelles elles partagent leurs caractéristiques anatomopathologiques et moléculaires (réarrangement du gène &lt;em&gt;EWSR1&lt;/em&gt;). Elles sont définies par leur développement dans les tissus cutanés/sous-cutanés, sans invasion de l’aponévrose sous-jacente. Ce sont typiquement des tumeurs de petite taille, de localisations ubiquitaires, avec un tropisme particulier pour les sites distaux des membres, tronculaires et ORL. Leur prise en charge, comme celles des tumeurs conjonctives malignes, doit se faire en centre spécialisé. Les métastases sont rares au diagnostic (&lt;5 %), mais doivent être recherchées par un bilan d’extension adapté (TEP TDM/TDM thoracique). Leur traitement repose sur un traitement local (basé sur une chirurgie carcinologique), encadrée par une chimiothérapie néoadjuvante et adjuvante. Les patients porteurs d’une maladie localisée ont un excellent pronostic, (en comparaison aux tumeurs d’Ewing classiques) et doivent être traités avec le double objectif de curabilité, et de réduction des toxicités post-thérapeu","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 611-618"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143617233","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Chondrosarcomas: Multidisciplinary review and practical recommendations, on behalf of GroupOs","authors":"Pascaline Boudou-Rouquette ,&nbsp;Nadège Corradini ,&nbsp;Marie-Pierre Sunyach ,&nbsp;Frédérique Larousserie ,&nbsp;Camille Cordero ,&nbsp;Aude Marie Cardine ,&nbsp;Sophie Piperno-Neumann ,&nbsp;Emmanuelle Bompas ,&nbsp;Gonzague de Pinieux ,&nbsp;François Gouin ,&nbsp;Nayla Nicolas ,&nbsp;Amandine Crombé ,&nbsp;Sylvie Helfre ,&nbsp;Antoine Feydy ,&nbsp;Marie Faruch ,&nbsp;David Biau ,&nbsp;on behalf of GroupOs","doi":"10.1016/j.bulcan.2024.07.013","DOIUrl":"10.1016/j.bulcan.2024.07.013","url":null,"abstract":"<div><div>Chondrosarcomas are rare tumors occurring in middle age and older adults, defined by malignant cartilaginous matrix-producing neoplasms. Chondrosarcomas represent a heterogeneous group of tumors with diverse characteristics, management strategies and prognosis. The aim is to establish recommendations to support optimal practice for the diagnosis and treatment of chondrosarcomas within the framework of an expert group at the request of <strong>GroupOs</strong> and the French Sarcoma Group. The recommendations were developed by a multidisciplinary working and underwent thorough proofreading. The level of evidence in scientific literature and the grading of recommendations by the French Haute Autorité de santé (HAS) were taken into account. Key recommendations cover: (i) diagnosis, management and follow-up enchondromatosis; (ii) initial assessment, diagnosis and staging of cartilaginous tumor; (iii) management of low-grade, clear cell and high-grade, localized resectable chondrosarcomas; (iv) indications for radiotherapy in chondrosarcomas; (v) management of locally advanced and metastatic disease; (vi) management of mesenchymal chondrosarcomas. Surgical resection at a specialized center remains the mainstay for localized chondrosarcomas management, tailored to grade and anatomical location. Limited evidence supports the use of neoadjuvant or adjuvant treatment in chondrosarcomas, except in mesenchymal chondrosarcomas. For patients with dedifferentiated chondrosarcomas, neo or adjuvant treatment with osteosarcoma-like regimens may be proposed. In cases of recurrence or metastasis, local treatment should be prioritized or participation in clinical trials including new targeted or immune therapies should be considered. This article presents consensus recommandations from adult and pediatric sarcoma experts of various disciplines on the practical management of chondrosarcomas patients.</div></div>","PeriodicalId":9365,"journal":{"name":"Bulletin Du Cancer","volume":"112 6","pages":"Pages 638-655"},"PeriodicalIF":1.1,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143722539","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}