Organs of Zuckerkandl: their surgical significance and a review of a century of literature

BackgroundOrgans of Zuckerkandl (O of Z) harbor the potential for deadly paragangliomas. Paragangliomas are one of the surgical causes of hypertension. Major treatises of medicine offer very little information on this topic.MethodsPubMed Medline and Google searches were performed to obtain reported cases of paragangliomas of the O of Z. A total of 135 cases of paragangliomas of the O of Z were found. Each case was reviewed and charted. Charts then were analyzed.ResultsSeventy-four percent of patients with this neoplasm have hypertension. Eighty percent of patients undergo an operation with more than 30% having an incorrect preoperative diagnosis. These tumors have a 43% mortality when presenting acutely.ConclusionsIndividuals aged ≤50 years with hypertension should be considered for screening for pheochromocytoma/paraganglioma with uring and/or serum catecholamines. Twenty-four–hour collections for urinary metanephrines and vanillylmandelic acid are diagnostic of functional tumors. A high degree of suspicion should follow with radiologic and chemical diagnostic studies. Triple-phase helical computed tomographic scans, metaiodobenzyl-guanidine scans, and magnetic resonance imaging with gadolinium-diethylenetriaminepentaacetic acid infusion are the standard for radiologic diagnosis. Treatment is always surgical excision. Phase II trials are in order for neoadjuvant and adjuvant treatment options.