Polymyalgia rheumatica and vagal paraganglioma

Eur Ann Otorhinolaryngol Head Neck Dis. 2017 Dec;134(6):427-430. doi: 10.1016/j.anorl.2017.03.005. Epub 2017 Apr 12.

Abstract

Introduction: Vagal paraganglioma are rare tumors that are mostly asymptomatic. We report a case of vagal paraganglioma associated with paraneoplastic polymyalgia rheumatica and review the literature on benign paragangliomas of the head and neck associated with paraneoplastic syndrome.

Case report: A 53-year-old man presented with atypical polymyalgia rheumatica. MRI revealed a tumor that was then surgically excised. Histological examination confirmed the diagnosis of benign vagal paraganglioma. Rapid, complete and permanent resolution of all rheumatological symptoms were observed postoperatively, confirming the diagnosis of paraneoplastic polymyalgia rheumatica.

Conclusion: Paraganglioma of the neck associated with paraneoplastic syndrome remains exceptional. A predisposing gene mutation must be systematically investigated. Long-term surveillance must be ensured due to the risk of local recurrence, second tumors or metastasis.

Keywords: Paraganglioma; Paraneoplastic syndrome; Polymyalgia rheumatica; Vagus nerve lesion.

Publication types

  • Case Reports

MeSH terms

  • Cranial Nerve Neoplasms / complications
  • Cranial Nerve Neoplasms / diagnosis*
  • Cranial Nerve Neoplasms / surgery*
  • Diagnosis, Differential
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Paraganglioma, Extra-Adrenal / complications
  • Paraganglioma, Extra-Adrenal / diagnosis*
  • Paraganglioma, Extra-Adrenal / surgery*
  • Polymyalgia Rheumatica / complications*
  • Treatment Outcome
  • Vagus Nerve Diseases / complications
  • Vagus Nerve Diseases / diagnosis*
  • Vagus Nerve Diseases / surgery*