Retroperitoneal Extra-Adrenal Paraganglioma

Abstract

Extra-adrenal paragangliomas (EAP) are rare endocrine tumors arising from embryonic neural crest
cells. Paragangliomas are mainly composed of chromaffin cells located along the paravertebral and
para-aortic axes, extending from the cervical region to the base of the pelvis. Extra-adrenal
paragangliomas (EAP) account for only 10-15% of all paragangliomas of which 85%are located in the
retroperitoneum, commonly arising from the organ of Zuckerkandl.
Functional paragangliomas secrete catecholamines which clinically may present with headache,
sweating, palpitation and symptoms of hypertension. Non-functional may remain silent and present
with vague symptoms like pain in abdomen.
Herein, we report a case of extra-adrenal paraganglioma (EAP) in a 26-year-old male who presented
with headache and abdominal pain. On the basis of clinical suspicion, raised Vanyl Mandelic Acid
(VMA) level and radiological findings diagnosis of extra-adrenal paraganglioma (EAP) was made.
Preoperative blood pressure (BP) was well controlled and operated successfully for exploratory
laparotomy and excision of tumor. Post-operative recovery was un-eventful. Finally histopathological
findings confirmed the clinical and radiological diagnosis of extra-adrenal paraganglioma (EAP).
Herein, we emphasize the importance of an accurate and prompt diagnosis of extra-adrenal
paragangliomas (EAP) in patients to reduce the morbidity and mortality.