Polymyalgia rheumatica and vagal paraganglioma

Publication date: Available online 12 April 2017
Source:European Annals of Otorhinolaryngology, Head and Neck Diseases
Author(s): V. L'Huillier, O. Mauvais, S. Valmary-Degano, L. Tavernier
IntroductionVagal 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 reportA 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.ConclusionParaganglioma 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.



http://ift.tt/2p9xyAk