Sinonasal Paraganglioma With Long-Delayed Recurrence and Metastases: Genetic and Imaging Findings

J Clin Endocrinol Metab. 2013 Nov;98(11):4262-6. doi: 10.1210/jc.2013-2320. Epub 2013 Sep 12.

Abstract

Context: Sinonasal paragangliomas have rarely been reported in the literature. They are often aggressive.

Patient: We report an original case of sinonasal paragangliomas with a tumor recurrence diagnosed 13 years after resection of the primary tumor. Somatostatin receptor scintigraphy and [18F]-fluorodeoxyglucose positron emission tomography/computed tomography were the most sensitive functional imaging techniques, and they ruled out distant metastases. Interestingly, [18F]-fluorodihydroxyphenylalanine positron emission tomography/computed tomography was negative, a feature that may be considered a sign of functional dedifferentiation. Screening for germline mutations of the SDHB, SDHC, SDHD, SDHAF2, VHL, MAX, and TMEM127 was negative.

Conclusion: The diagnosis of malignancy remains challenging at initial diagnosis, and patients should be followed during their entire lifetime.

Publication types

  • Case Reports

MeSH terms

  • Endoscopy
  • Humans
  • Male
  • Middle Aged
  • Multimodal Imaging*
  • Neoplasm Recurrence, Local / genetics
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / surgery
  • Nose Neoplasms / genetics*
  • Nose Neoplasms / pathology*
  • Nose Neoplasms / surgery
  • Paraganglioma / genetics*
  • Paraganglioma / secondary*
  • Paraganglioma / surgery
  • Paranasal Sinuses
  • Positron-Emission Tomography
  • Tomography, X-Ray Computed