Extra-Adrenal Silent Retroperitoneal Paraganglioma: A Rare Case

Extra- adrenal retroperitoneal paragangliomas are extremely rare neuroendocrine neoplasms with an incidence of 2-8 per million. They emanate from embryonic neural crest cells and are composed mainly of chromaffin cells located in the para- aortic sympathetic chain. They synthesize, store and secrete catecholamines due to which they may present with symptoms of hypertension like headache, sweating and palpitation and sometimes they may present with vague symptoms like pain abdomen and lump abdomen. On the other hand, they may remain silent and non- functional. Histopathologically and tumor marker wise, non- functional and the functional tumours are absolutely same. Primary methods of pre-operative diagnosis include imaging techniques which also help in surgical planning and pre-operative preparation of the patient. Non- functional tumours often escape preoperative diagnosis and create intraoperative complications. We present a case of non- functional extra- adrenal retroperitoneal paraganglioma occurring in a 50-year-old male patient presenting with mass per abdomen. On Ultrasonongraphy, suspicion was towards a retroperitoneal mass of probable lymph nodal origin. On CT Scan, A large well defined heterogeneously enhancing mass lesion with solid and necrotic areas and few tiny foci of central calcifications is seen in left side of mesenteric measuring 11.4cm *11.7cm*9.6cm (AP*TD*CC). No bowel invasion is seen. Prominent vessels are seen around the mass. IMP- Mass lesion left side of abdomen likely mesenteric origin ( Malignant). The patient was posted for surgical resection of the mass. Per-operatively, the patient developed hypertensive crisis. Post operative period was uneventful and patient recovered well. This report highlights the importance of pre- operative diagnosis which is vital in the management of extra- adrenal retroperitoneal paraganglioma.