Retroperitoneal para-aortic paraganglioma

Abstract

Paragangliomas or chemodectomas are neoplasms that arise from neural crest cells and histologically resemble their adrenal counterpart, the pheochromocytoma. Seventy-one percent of the extra-adrenal paragangliomas are located in the superior or inferior para-aortic area. This tumor usually presents as an abdominal mass producing back pain. Tumor localization has improved remarkably through the use of computed tomography and I131MIBG (metaiodobenzylguanidine) scintigraphy, particularly when tumors are hormonally active. Surgical excision remains the mainstay of treatment, although vascularity can at times make excision difficult.