Extra- Adrenal Silent Retroperitoneal Paraganglioma: Report of a Rare Case

Abstract

Extra- adrenal retroperitoneal paragangliomas are extremely rare neuroendocrine neoplasms with an incidence of 2-8 per million. They arise 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 because of which they may present with headache, sweating, palpitation and symptoms of hypertension. On the other hand, they may remain silent and non- functional and present with vague symptoms like pain abdomen due to episodic release of catecholamines. Histologically and immunohistochemically, non- functional and the functional tumours are no different from each other. Primary methods of pre-operative diagnosis include imaging techniques which also help in surgical planning and pre-operative preparation. Non- functional tumours of ten escape pre-operative detection and create per- operative complications. We present a case of nonfunctional extra- adrenal retroperitoneal paraganglioma occurring in a 66-year-old female patient presenting with pain and mass per abdomen. On ultrasonongraphy, a retroperitoneal mass of probable mesenchymal origin was suspected. The patient was posted for surgical resection of the mass. Per-operatively, the patient developed hypertensive crisis and massive bleeding. Post-operatively she developed renal failure and succumbed to it after three days. This report highlights the importance of pre- operative diagnosis which is vital in the management of extra- adrenal retroperitoneal paraganglioma.

Keywords

• adrenal pheochromocytoma
• neuroendocrine
• retroperitoneal