Changes in urinary total metanephrine excretion in recurrent and malignant pheochromocytomas and secreting paragangliomas.
We quantified urinary excretion of total metanephrines (metanephrine plus normetanephrine) (UETM) in 261 patients with pheochromocytoma (PH) or secreting paraganglioma, before primary tumor resection. We then determined UETM 2 weeks after primary tumor resection and once per year thereafter. The tumor was considered benign in 242 patients and malignant in 19 patients. Patients with malignant tumors had higher UETM and tumor diameters, lower plasma epinephrine concentrations, and were more likely to have secreting paragangliomas than patients with benign tumors. In the 215 patients with a single-benign primary tumor, preoperative UETM and tumor diameter were significantly correlated. Thirty-six patients subsequently developed recurrences or new tumors, which were malignant in 18 cases. All recurrent or new tumors could be detected before the onset of symptoms, on the basis of an increase in metanephrine concentration. We followed one patient with von Hippel-Lindau disease and a new tumor for 50 months before reoperation and found that UETM and tumor diameter were significantly correlated. In patients with malignant tumors, the logarithm of UETM was significantly correlated with time, suggesting an exponential increase in tumor activity and, presumably, tumor burden. Urinary metanephrine excretion is a marker of tumor activity and a surrogate of tumor burden in patients with pheochromocytoma or secreting paraganglioma. UETM excretion could be used to assess treatment response in malignant cases.
Amar
Laurence
L
Hypertension Unit, Université Paris-Descares, Faculté de Médecine, and Hôpital Européen Georges Pompidou, 20 rue Leblanc, 75908 Paris cedex 15, France.
Peyrard
Séverine
S
Rossignol
Patrick
P
Zinzindohoue
Franck
F
Gimenez-Roqueplo
Anne-Paule
AP
Plouin
Pierre-François
PF
D000310:Adrenal Gland Neoplasms / Q000473:pathology / Q000652:urine*; D000328:Adult; D005260:Female; D006801:Humans; D008297:Male; D008676:Metanephrine / Q000652:urine*; D008875:Middle Aged; D010235:Paraganglioma / Q000473:pathology / Q000652:urine*; D010673:Pheochromocytoma / Q000473:pathology / Q000652:urine*; D012008:Recurrence
D008676:Metanephrine