Unusual presentation of a rare catecholamine-secreting sinonasal paraganglioma

BMJ Case Rep. 2023 May 2;16(5):e254848. doi: 10.1136/bcr-2023-254848.

Abstract

Our patient is a man in his mid-20s with an atypical presentation of a catecholamine-secreting sinonasal paraganglioma. He was referred to our tertiary otolaryngology unit for persistent right infraorbital numbness. A nasoendoscopic examination demonstrated a smooth mass arising from the posterior aspect of the right middle meatus. There was also right infraorbital paraesthesia. Imaging revealed a lesion in the right pterygopalatine fossa. Blood investigations revealed significantly elevated serum normetanephrine levels. The lesion was demonstrated to be octreotide-avid with no other lesions detected. The presumptive diagnosis of a catecholamine-secreting paraganglioma was made, and an endoscopic resection of the tumour was performed. Histopathology of the tumour demonstrated a 'zellballen' growth pattern consistent with a paraganglioma. Catecholamine-secreting sinonasal paragangliomas are exceedingly rare with multifaceted challenges. More studies are required to improve our knowledge of this condition.

Keywords: Adrenal disorders; Ear, nose and throat/otolaryngology; Neurosurgery; Otolaryngology / ENT.

Publication types

  • Case Reports

MeSH terms

  • Catecholamines*
  • Humans
  • Male
  • Normetanephrine
  • Paraganglioma* / diagnostic imaging
  • Paraganglioma* / surgery
  • Tomography, X-Ray Computed

Substances

  • Catecholamines
  • Normetanephrine