Soft tissue sarcomas and malignant nerve sheath tumours in the maxillofacial region are rare, represent a varied group of conditions (and causes), and affect a diverse group of people. Accordingly, the possible and most suitable treatment modalities vary widely too. For example, considerations about the individually best possible treatment approach clearly will be different for a child with rhabdomyosarcoma from those relevant for an elderly person diagnosed with advanced-stage liposarcoma.

Despite this heterogeneity in patients and tumours, there is one common aspect: radical surgical resection, where possible and usually with the need for reconstruction to repair the resulting defect, is the first-line intervention with a view to curative treatment. Wide excision and clear margins are necessary for disease-free survival. In some instances, sufficiently radical surgery may not be a viable option and chemo- and/or radiotherapy interventions are necessary. The efficacy of chemo- and radiotherapy in the treatment of sarcomas in adults is limited, whereas there is a role for chemo/radiotherapy in the treatment of children. Limitations for radical surgery typically arise from the location and size & shape of a tumour when nearby vital structures prevent ‘radical enough’ excision and/or access. Immunotherapy for the time being does not play a role in the treatment of these soft-tissue malignancies.

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