Damle Rajshri P*, Suryawanshi Kishor H*, Patil Tushar B** ,Dravid N. V*, Newadkar D.V*,
*Department of Pathology, **Department of Surgery, A.C.P.M. Medical College, Dhule.
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.
Key words: Catecholamines, Extra-adrenal paragangliomas, Vanyl Mandelic Acid (VMA).