Article Text

Download PDFPDF
Adult nasal chondromesenchymal hamartoma: a rare and benign tumour with aggressive malignant transformation
  1. Rachael Collins1,2,
  2. George Lafford3,
  3. Sheneen Meghji2 and
  4. Stuart Burrows2
  1. 1Norwich Medical School, University of East Anglia Norwich Medical School, Norwich, UK
  2. 2ENT/Otolaryngology, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK
  3. 3Plastic Surgery, Norfolk and Norwich University Hospital NHS Trust, Norwich, UK
  1. Correspondence to Dr Rachael Collins; rachael.collins{at}uea.ac.uk

Abstract

Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign tumour of the nasal cavity predominantly described in infants. We report a case involving a 48-year-old woman who had been diagnosed with NCMH a year earlier and now re-presented with a short history of progressive nasal blockage, recurrent epistaxis and orbital apex syndrome. Histopathology was suggestive of malignant transformation into sinonasal sarcoma. However, following multidisciplinary team (MDT) discussions, including second and third opinions from external departments, the histological diagnosis was revised to ‘NCMH with bizarre stromal cells’. Despite this, the lesion demonstrated malignant features of rapid, invasive growth and was treated with palliative radiotherapy. The patient later developed radiological evidence of lung and liver metastases with subsequent pulmonary emboli. Shortly after this, she passed away. This case is unique in its diagnostic challenge, with ambiguous histopathological findings, and highlights the importance of an MDT approach when managing complex sinonasal tumours.

  • ear
  • nose and throat/otolaryngology
  • head and neck cancer
  • head and neck surgery
  • radiotherapy
  • palliative care

Statistics from Altmetric.com

Footnotes

  • Contributors RC and GL are co-first authors. SB and SM edited the manuscript and supervised.

  • 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.

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

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.