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Bone sarcomas and cancer predisposition syndromes.

Bull Cancer

January 2025

Department of Paediatric Oncology, Institut d'Haematologie et d'Oncologie Pédiatrique, Centre Léon-Bérard, Lyon, France. Electronic address:

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.

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The management of children with syndromes associated with an increased risk of benign and malignant neoplasms is a complex challenge for healthcare professionals. The 2023 AACR Childhood Cancer Predisposition Workshop provided updated consensus guidelines on cancer surveillance in these syndromes, aiming to improve early detection, intervention, and reduce morbidity associated with such neoplasms. In this paper, we review several of the rare conditions discussed in this workshop.

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Objectives: Microvascular free tissue transfer (MVFTT) for head and neck reconstruction is infrequently performed in pediatric patients. There is a paucity of data on perioperative airway management in pediatric MVFTT, such as the need for tracheostomy, which can pose higher morbidity to young patients due to potential long-term effects on the softer, more pliable laryngotracheal cartilage. Our objective was to report airway outcomes on pediatric patients undergoing MVFTT after segmental mandibulectomy with or without tracheostomy.

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Spinal chondrosarcomas are uncommon malignant bone tumors that are rare in children. The location and imaging features of the lesion help in formulating a differential diagnosis, though in certain instances the lack of typical imaging findings may elude the differential of a chondrosarcoma. There are only a few reported cases in literature and owing to its rarity and lack of specific management protocols in this age group, making treatment decisions can be complex.

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