English

Abstract

Catecholamine-producing tumors are rare and may arise in the adrenal medulla (pheochromocytomas) or in extra adrenal chromaffin cells (secreting paragangliomas). Their prevalence is about 0.1% in patients with hypertension. These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis 1 and von Hippel-Lindau disease. The most specific and sensitive diagnostic test for the tumor is the determination of plasma or urinary metanephrine. The tumor can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Treatment requires resection of the tumor, generally by laparoscopic or open surgery. However, resection of these tumors can be life threatening. We present a case of functional extra-adrenal sympathetic paraganglioma excision performed using general anaesthesia with optimal hemodynamic management during the perioperative period. CONCLUSION: Functional Paragangliomas are rare tumors which are often undetected. All hypertensives, especially young should be always screened for this condition. Proper preparation of the case is required and the team should always be on high alert for the various haemodynamic challenges that can come across while managing this case.