Endocrine manifestations were absent in a patient presenting with pain in the left peri-umbilical region from a paraganglioma of the organs of Zuckerkandl. The authors describe the embryology of paragangliomas and review the published literature concerning the noradrenaline-secreting forms of these tumors. Only 86 references to those of Zuckerkandl, situated on the side of the aorta in relation to the inferior mesenteric artery, were found. Diagnosis of non-secreting forms is confirmed by arteriography which in this present case demonstrated three pedicles arising from the inferior mesenteric, celiac, and one of the lumbar arteries respectively. The only evidence of malignancy is the presence of metastases. Their diagnosis is therefore difficult but essential as one-third of the localisations of malignant pheochromocytomas (10 p. cent of pheochromocytomas) outside of the adrenals affect the organs of Zuckerkandl.
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