A Case Report of Paraganglioma: A Diagnosis Not to Be Missed

The autonomic nervous system, a derivative of neural crest cells, may undergo the neoplastic transformation and give rise to paragangliomas, which can arise either at intra-adrenal or extra-adrenal sites, commonly referred to as pheochromocytomas and paragangliomas, respectively. Although indistinguishable at the cellular level and usually identical in clinical manifestation, these two tumors differ in means of malignancy risk, genetic testing, and the probability of detecting an associated neoplasm, highlighting the importance of making a correct diagnosis to prevent additional complications.

We report a 17-year-old female patient presented with sudden blurred vision and palpitation lasting for 1 week. The physical examination revealed tachycardia, hypertension, and a mildly tender mass on the left side of the umbilicus. A spiral abdominal and pelvic computed tomography (CT) scan was performed, which depicted a hypervascular retroperitoneal mass in the left infrarenal region. Mildly-elevated urine metanephrine and normetanephrine levels were suggestive of a paraganglioma (PGL). Iodine-131 metaiodobenzylguanidine (MIBG) scan was also in favor of PGL of the organ of Zuckerkandl. The mass was completely resected and the immunohistochemistry (IHC) study of the specimen confirmed the diagnosis.

The diversity of manifestations and rarity of PGL can delay the diagnosis, which may lead to potential complications. This makes it compelling to include PGLs in differential diagnoses in a clinical setting compatible with catecholamine hypersecretion.