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Paediatric desmoid tumour of the head and neck: a complete response to chemotherapy and the paradigm shift towards non-surgical management
  1. Filipa Proença1,
  2. Claudia Constantino2,
  3. Maria Manuel Lemos3 and
  4. Alexandra Borges4
  1. 1Department of Neuroradiology, Centro Hospitalar Universitário Lisboa Norte EPE, Lisboa, Portugal
  2. 2Department of Paediatrics, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal
  3. 3Department of Pathology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal
  4. 4Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal
  1. Correspondence to Dr Alexandra Borges; borgalexandra{at}


Desmoid tumours are rare locally aggressive mesenchymal tumours with a high local recurrence rate, particularly in the head and neck locations. We present the case of a 5-year-old child with a locally invasive inoperable desmoid tumour of the masticator space who underwent a low-dose chemotherapy regimen for 17 months, obtaining a long-term complete response. Definitive evidenced-based treatment guidelines are lacking. Therefore, paediatric patients should be managed by specialised multidisciplinary teams to try to achieve the best tumour control while minimising treatment associated morbidity and mortality. Available treatment options include surgery, radiotherapy and different systemic medical therapies. Whereas traditionally, surgery was the mainstay of treatment, more conservative options have increasingly shown adequate tumour control with little associated morbidity. In this decision making it is mandatory to take into account the patient’s age, tumour location and extension, and potential short-term and long-term treatment-related sequelae to minimise functional and cosmetic compromise.

  • Chemotherapy
  • Head and neck cancer
  • Paediatric oncology
  • Paediatrics (drugs and medicines)
  • Radiology

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  • Contributors FP reviewed the subject, collected the images, wrote and reviewed the manuscript. CC was responsible for the clinical management of the patient, reviewed the manuscript. MML collected the pathology slices and reviewed the manuscript. AB conceptualised the manuscript, helped writing, collected the images and reviewed the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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