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Case report
Extra-skeletal Ewing’s sarcoma of the frontal sinus: a rare disorder in pediatric age
  1. Isabel Esteves Costa,
  2. Ana Sousa Menezes,
  3. Antonio Fontes Lima and
  4. Berta Rodrigues
  1. Department of Otorhinolaryngology and Head & Neck Surgery, Hospital de Braga, Braga, Portugal
  1. Correspondence to Dr Isabel Esteves Costa; isabelcostaorl{at}


Paediatric sinonasal tumours comprise numerous aetiologies. Ewing’s sarcoma (ES) consists of a malignancy of neuroectodermal origin. This type of sarcoma affects mainly children and adolescents and can assume the skeletal or extra-skeletal form. Primary ES of head and neck is extremely rare, accounting for only 4%–9% of all cases. So far, as much as we know, only a few cases of sinonasal ES have been reported in literature. The authors present a case of a previous healthy 12-year-old girl who presented with a rapidly growing and expansive frontal mass and unilateral nasal obstruction. Immunohistochemical, molecular and cytogenetic analysis of the lesion showed diffuse expression of CD56 and CD99 on tumour cells and a translocation involving chromosome 22q12, confirming ES diagnosis.

  • ear, nose and throat/otolaryngology
  • paediatrics (drugs and medicines)
  • chemotherapy
  • head and neck cancer
  • paediatric oncology

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  • Contributors IEC planned and conceived of the presented idea. IEC, AFL and ASM participated in planning and design of this study. BR encouraged IEC to investigate Ewing’s sarcoma in paediatric age and supervised the findings of this work. Acquisition of data was made by IEC and ASM. The analysis and interpretation of data was responsibility of IEC and AFL. IEC wrote the manuscript with support from AFL, ASM and BR. All authors discussed the results and contributed to the final version of 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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