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Unusual presentation of a rare catecholamine-secreting sinonasal paraganglioma
  1. Jian Li Tan1,2,
  2. Pei Yinn Toh1 and
  3. Shane Ling1
  1. 1Otolaryngology, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia
  2. 2Otorhinolaryngology, Tan Tock Seng Hospital, Singapore
  1. Correspondence to Dr Jian Li Tan; Jian_Li_TAN{at}ttsh.com.sg

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.

  • Ear, nose and throat/otolaryngology
  • Adrenal disorders
  • Otolaryngology / ENT
  • Neurosurgery

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Footnotes

  • Contributors JLT: Literature search, coordination of author efforts, manuscript writing, proof-reading and submission. PYT: Manuscript writing, revision and proof-reading. SL: Manuscript writing, revision and proof-reading.

  • 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.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

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