Caveat for Vascular Surgeons: Lesson Learned from Acute Onset of a Rare Aortic Paraganglioma in a Young Boy.

ABSTRACT

BACKGROUND: Paragangliomas (PGs) are rare neuroendocrine tumors arising from the extra-adrenal autonomic paraganglia that are tiny organs formed by bundles of neuroendocrine cells derived from the embryonic neural crest and capable of catecholamines secretion. Diagnosis and treatment of aortic PGs could be a challenging issue when they present as an emergency setup (sudden abdominal pain and radiological images resembling a vascular emergency). CASE REPORT We present a rare case of a 16-year-old man with a symptomatic and bleeding left para-aortic mass, treated in emergency with embolization, before a staged videolaparoscopic resection. Histology of the mass showed the presence of a large aortic PG. CONCLUSIONS: In case of active bleeding, in emergency, vascular consultants are always involved. Sometimes, circumstances are very atypical; therefore, it is essential to keep in mind rare pathologies. In such settings, multidisciplinary approach is primary to obtain a prompt diagnosis and appropriate treatment.