Metachronous multicentric jugulotympanic and retroperitoneal paragangliomas: a case report and review of the literature

Introduction: Paragangliomas(PGLs) are rare neuroendocrine neoplasms originating from cells of the primitive neural crest. Head and neck PGLs represent nearly 20% of all PGLs. Retroperitoneal localisations are rare and represent less than 2% of all PGLs, and are known to be exceptionally nonfunctional. Case report: In this article we report a case of a 46-year-old female who presented, in 2013, a jugulotympanic PGL for which radiotherapy was delivered. In 2016, a second PGL occurred in the retroperitoneum and was completely resected by surgery alone. 6 year follow up showed a stability of the jugulotympanic PGL and no retroperitoneal recurrence. Conclusion: Current knowledge of different management modalities is especially relevant for patients with multicentric PGL. Notably, potential benefits and risks of available treatment options should be taken into consideration for every individual patient in order to provide personalized care. Recently, genetic screening particularly SDH mutations has shown a positive impact in the management of this disease as well as in predicting survival outcomes.