Bone sarcomas: ESMO–EURACAN–GENTURIS–ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up

Strauss, Sandra J.; Frezza, Anna Maria; Abecassis, N.; Bajpai, Jyoti; Bauer, Sebastian; Biagini, R.; Bielack, Stefan; Blay, Jean‐Yves; Bolle, Stéphanie; Bonvalot, Sylvie; Boukovinas, I.; Bovée, Judith V.M.G.; Boye, Kjetil; Brennan, Bernadette; Brodowicz, Thomas; Buonadonna, Angela; Álava, Enrique de; Tos, Angelo Paolo Dei; Muro, Xavier García del; Dufresne, Armelle; Eriksson, Mikael; Fagioli, Franca; Феденко, А. А.; Ferraresi, Virginia; Ferrari, Andrea; Gaspar, Nathalie; Gasperoni, Silvia; Gelderblom, Hans; Gouin, F.; Grignani, Giovanni; Gronchi, Alessandro; Haas, Rick L.; Hassan, A B; Hecker‐Nolting, Stefanie; Hindi, Nadia; Hohenberger, Peter; Joensuu, Heikki; Jones, Robin L.; Jungels, Christiane; Jutte, Paul C.; Kager, Leo; Kasper, Bernd; Kawai, Akira; Kopečková, Kateřina; Krákorová, Dagmar Adámková; Cesne, Axel Le; Grange, Franel le; Legius, Eric; Leithner, Andreas; Pousa, Antonio López; Martin‐Broto, Javier; Merimsky, Ofer; Messiou, Christina; Miah, Aisha; Mir, Olivier; Montemurro, Michael; Morland, Bruce; Morosi, Carlo; Palmerini, Emanuela; Pantaleo, Maria Abbondanza; Piana, Raimondo; Piperno‐Neumann, Sophie; Reichardt, Peter; Rutkowski, Piotr; Safwat, Akmal; Sangalli, Claudia; Sbaraglia, Marta; Scheipl, Susanne; Schöffski, Patrick; Sleijfer, Stefan; Strauß, D.; Hall, Kirsten Sundby; Trama, Annalisa; Unk, Mojca; Sande, Michiel A.J. van de; Graaf, Winette T.A. van der; Houdt, Winan J. van; Frébourg, Thierry; Ladenstein, Ruth; Casali, Paolo G.; Stacchiotti, Silvia

doi:10.1016/j.annonc.2021.08.1995

Cited by 168 publications

(109 citation statements)

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“…Primary malignant bone tumors (PMBT) are extremely rare neoplasms across all ages with an overall estimated incidence between 0.8–0.9 cases per 100,000 per year [ 113 ]. While osteosarcoma (OS) and Ewing sarcoma (ES) have a relatively high incidence in young patients (mostly around the second decade of life), chondrosarcoma (CS) will often arise in older ages [ 113 ].…”

Section: Primary Malignant Bone Tumors and Bone Metastasesmentioning

confidence: 99%

“…However, in comparison with OS, ES will present a higher incidence in the pelvis, ribs and vertebrae [ 115 ]. CS is the most common malignant bone tumor in adults and arises most frequently in the pelvis and distal and proximal femur, as de novo or as result of the differentiation from pre-existing lesions, such as osteochondroma or enchondroma [ 113 , 116 ].…”

Section: Primary Malignant Bone Tumors and Bone Metastasesmentioning

confidence: 99%

“…The clinical presentation for PMBT is characterized by bone pain, predominantly at night, swelling and functional impairment, however, these clinical features can be overlapped by any BM or even other diseases, such as osteomyelitis [ 113 ]. Nonetheless, BM or bone lesions arising in a multiple myeloma setting are much more common.…”

Section: Primary Malignant Bone Tumors and Bone Metastasesmentioning

confidence: 99%

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A Tailored Approach for Appendicular Impending and Pathologic Fractures in Solid Cancer Metastases

Brito

Lopes-Brás

Abrunhosa-Branquinho

et al. 2022

Cancers

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Advances in medical and surgical treatment have played a major role in increasing the survival rates of cancer patients with metastatic bone disease. The clinical course of patients with bone metastases is often impaired by bone complications, such as bone fractures, which have a substantial negative impact on clinical outcomes. To optimize clinical results and prevent a detrimental impact on patients’ health, a tailored approach should be defined for any given patient. The optimal management of impending or pathologic fractures is unknown and relies on a multidisciplinary approach to tailor clinical decisions to each individual patient. The ability to control systemic disease, the extent, location and nature of bone metastases, and the biology of the underlying tumor, are the main factors that will define the strategy to follow. The present review covers the most recent data regarding impending and pathologic fractures in patients with bone metastases, and discusses the medical and surgical management of patients presenting with metastatic bone disease in different clinical settings.

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Section: Primary Malignant Bone Tumors and Bone Metastasesmentioning

confidence: 99%

Section: Primary Malignant Bone Tumors and Bone Metastasesmentioning

confidence: 99%

Section: Primary Malignant Bone Tumors and Bone Metastasesmentioning

confidence: 99%

See 1 more Smart Citation

A Tailored Approach for Appendicular Impending and Pathologic Fractures in Solid Cancer Metastases

Brito

Lopes-Brás

Abrunhosa-Branquinho

et al. 2022

Cancers

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“…Ewing sarcoma (ES) is a rare, highly aggressive disease, the second most frequent bone tumor in children and adolescents, and third in the overall population [ 1 ]. Disseminated disease is detected in about 20–30% of patients at the time of initial diagnosis.…”

Section: Introductionmentioning

confidence: 99%

“…The chemotherapy of second and subsequent lines is based on the standards of the treatment of localized disease. In addition, the combination of alkylates with topoisomerase inhibitors, or irinotecan with temozolomide, or gemcitabine with docetaxel, or high-dose ifosfamide/carboplatin with etoposide are other therapeutic options; however, the duration of response is short, and the outcomes are disappointing [ 1 , 9 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

Mutational landscape of primary and recurrent Ewing sarcoma

Jagodzińska-Mucha¹,

Sobczuk²,

Mikula³

et al. 2021

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Introduction: Ewing sarcoma (ES) is a highly aggressive malignancy of bone and soft tissues characterized by the presence of a genetic fusion involving the EWSR1 gene. More than one-third of patients develop distant metastases, which are associated with unfavorable prognosis. Knowledge about the disease's genetic landscape may help foster progress in using targeted therapies in the treatment of ES. Aim of the study: The objective is to assess the mutational landscape of ES in pretreatment samples, tumor samples after neoadjuvant chemotherapy, and in metastatic/recurrent tumors in children and adults Material and methods: DNA from 39 for malin-fixed paraffin-embedded tumor samples of 22 patients (17 adults, 5 children) were analyzed by targeted next generation sequencing (NGS) using the Oncomine Comprehensive Assay v3gene panel. Additional functional analyses were performed between patient subgroups. Results: All samples were characterized by low tumor mutation burden (< 10 mut/Mb). The most commonly mutated genes were PIK3R1 (59%) and POLE (50%). The most widely detected variants in biopsy samples were PIK3R1 T369I (50%), FGFR1 E159K, and TP53 at codon 72 (both in 27.3%). Additionally, the ATR, BRCA1, RAD50, ATM, CHEK1, and NBN genes showed a significantly higher number of mutations in ES. Mutations in PIK3R1 were significantly more frequent in adults, while mutations in the pathways responsible for cell cycle control, DNA repair, and transcriptional regulation were more frequent in children. Conclusions: Besides EWSR1 fusion, ES is characterized by numerous point mutations that are potential targets for precision medicine. There is high genomic heterogeneity that may explain differences in outcomes between patient subgroups.

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Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group

et al. 2022

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Bone sarcomas: ESMO–EURACAN–GENTURIS–ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up

Cited by 168 publications

References 100 publications

A Tailored Approach for Appendicular Impending and Pathologic Fractures in Solid Cancer Metastases

A Tailored Approach for Appendicular Impending and Pathologic Fractures in Solid Cancer Metastases

Mutational landscape of primary and recurrent Ewing sarcoma

Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group

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