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Year of Diagnosis, Features at Presentation, and Risk of Recurrence in Patients with Pheochromocytoma or Secreting Paraganglioma

Hypertension Unit (L.A., A.S., P.-F.P.) and Departments of Genetics (A.-P.G.-R.), Visceral Surgery (F.Z.), and Clinical Epidemiology (G.C.), Hôpital Européen Georges Pompidou, 75908 Paris, France

Address all correspondence and requests for reprints to: Dr. Pierre-François Plouin, Hypertension Unit, Hôpital Européen Georges Pompidou, 20 rue Leblanc, 75908 Paris Cedex 15, France. E-mail: pierre-francois.plouin{at}egp.ap-hop-paris.fr.

Pheochromocytoma or secreting paraganglioma may recur after initial surgery. Diagnostic methods have improved in recent decades. We determined whether features at presentation have changed over time and are associated with long-term outcome. In 192 patients with pheochromocytoma/paraganglioma seen between 1975 and 2003, we compared time from onset of hypertension to first operation, total metanephrine excretion, tumor size and site, the proportion of cases presenting as incidentalomas, and the probability of recurrence according to date of operation (divided into quartiles). The duration of hypertension and tumor size at first operation decreased significantly over time. Right-sided adrenal tumors were more frequent and larger, excreted greater amounts of metanephrines, and presented more frequently as incidentalomas than left-sided tumors. Age, familial disease, and tumor site and size were independent predictors of recurrence. The risk of recurrence was 3.4-fold higher in patients with familial disease than in those with sporadic tumors; it was, respectively, 3.1- and 11.2-fold higher in patients with right adrenal and extraadrenal tumors than in patients with left adrenal tumors. In conclusion, pheochromocytomas/paragangliomas can now be diagnosed earlier, with smaller tumors, and more frequently as incidentalomas. Familial, right adrenal, and extraadrenal tumors recur more frequently than left adrenal tumors.

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