Pheochromocytoma is a tumor of adrenal medulla. Pheochromocytoma arising outside the adrenal medulla is commonly called paraganglioma. These tumours arise from paraganglia of the autonomic nervous system, most commonly occurring in head and neck region, and much less frequently in the retroperitoneum. But when they occur below the diaphragm in the organ of zuckerkandl or retroperotoneum, they are also called as extra adrenal pheochromocytoma. Composite pheochromocytoma/paraganglioma is a tumor composed of pheochromocytoma/ paraganglioma along with another tumor of neural crest origin composed of either a ganglio-neuroma/ ganglio- neuroblastoma /neuroblastoma or schwannoma. They are mostly located in the adrenal gland, while extra adrenal composite pheochromocytoma is extremely rare. Only 4 cases in the retroperitoneum have been described in the literature. Composite tumors of the adrenal medulla are rare tumors accounting for less than 3% of all sympatho-adrenal tumors. We report a rare case of retroperitoneal extra adrenal composite pheochromocytoma in a 19 year old female with a history of 3 months amenorrhoea.
Extra-adrenal pheochromocytoma, Composite paraganglioma, Retroperitoneum, IHC