Screening for adrenal medullary disease in patients with medullary thyroid carcinoma

Adrenal medullary disease (AMD) is clinically silent in most patients with medullary thyroid carcinoma (MTC). It was screened yearly by urinary measurements of catecholamines and derivates, and by abdominal ultrasonography (US) in a series of 174 patients with MTC. In cases with suspicion of AMD, abdominal computerized tomography and scintigraphy with meta-iodobenzylguanidine were also performed. AMD was discovered in 10 patients (one adrenal medullary hyperplasia and 9 pheochromocytomas). Three patients were already known to belong to a type II multiple endocrine neoplasia (MEN-2a) family and two had a MEN-2b syndrome. In 5 patients previously considered as having either a sporadic (4 cases) or a familial type of isolated MTC (one case), the occurrence of AMD led to diagnose a MEN-2a syndrome. The diagnostic values of the tests were evaluated by a case-control study. Urinary metanephrine plus normetanephrine (MN+N) had an acceptable sensitivity (0.8) and specificity (0.8). The other urinary tests had a high specificity (range: 0.8 to 1) but a poor sensitivity (range: 0.1 to 0.5). US had a high sensitivity (0.8) and a specificity of one. MN+N and US performed yearly constitute a simple and efficient strategy to screen for AMD in patients with MTC.