Original Contribution
The American Journal of Gastroenterology (2006) 101, 266–273; doi:10.1111/j.1572-0241.2006.00367.x
Prospective Endoscopic Ultrasonographic Evaluation of the Frequency of Nonfunctioning Pancreaticoduodenal Endocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1
Laurence Thomas-Marques1, Arnaud Murat2, Brigitte Delemer3, Alfred Penfornis4, Catherine Cardot-Bauters5, Eric Baudin6, Patricia Niccoli-Sire7, Damien Levoir8, Hélène du Boullay Choplin9, Olivier Chabre10, Nicolas Jovenin11 and Guillaume Cadiot1 Groupe des Tumeurs Endocrines (GTE)
- 1Gastroenterology, Hôpital Robert Debré, CHU de Reims, France
- 2Endocrinology, Hôpital Hôtel Dieu, CHU de Nantes, France
- 3Endocrinology, Hôpital Robert Debré, CHU de Reims, France
- 4Endocrinology, Hôpital Jean Mingoz, CHU de Besançon, France
- 5Endocrinology, Clinique Marc Linquette, CHU de Lille, France
- 6Endocrinology, Institut Gustave Roussy, Villejuif, France
- 7Endocrinology, Hôpital de la Timone, CHU de Marseille, France
- 8Gastroenterology, Hôpital Saint-Joseph, Paris, France
- 9Endocrinology, Hôpital de Chambéry, France
- 10Endocrinology, Hôpital Nord, CHU de Grenoble, France
- 11Statistics, Hôpital Robert Debré, CHU de Reims, France
Correspondence: Pr Guillaume Cadiot, Hépato-Gastroentérologie, Hôpital Robert Debré, 51092 Reims Cedex, France.
Received 8 July 2005; Accepted 4 September 2005.
Abstract
BACKGROUND:
The frequency of pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1 (MEN1) remains unknown.
AIM:
To evaluate prospectively with endoscopic ultrasonography (EUS) the frequency of nonfunctioning (asymptomatic) pancreaticoduodenal tumors.
PATIENTS AND METHODS:
MEN1 patients without functioning pancreatic involvement underwent systematic pancreaticoduodenal EUS in nine GTE (Groupe des Tumeurs Endocrines) centers. Demographic and clinical factors predictive of pancreatic involvement were sought, and standardized biochemical measurements obtained.
RESULTS:
Between November 1997 and July 2004, 51 patients (median age: 39 [range: 16–71] yr) were studied. MEN1 had been diagnosed 3 [0–20] yr earlier, notably by genetic screening for 26 (51%) with asymptomatic disease. Twenty-five patients had minor biochemical anomalies (<2 
normal (N)) and serum somatostatin was 10.8 N in 1; EUS detected pancreatic lesions in 28 patients (54.9%; 95% CI: 41.3–68.7%). A median of three [1–9] tumors with a median diameter of 6 [2–60] mm was found per patient; for 19 (37.3%) patients a tumor measured 
10 mm and 20 mm in 7 (13.7%) patients. Only one duodenal lesion was found and three patients had peripancreatic adenopathies. Pancreatic tumors were not associated with any of the studied parameters, notably age, family history, biochemical anomalies. Sixteen of twenty-six patients underwent EUS monitoring over 50 [12–70] months; six (37.5%) had more and/or larger pancreatic lesions.
CONCLUSION:
The frequency of nonfunctioning pancreatic endocrine tumors is higher (54.9%) than previously thought. The size and number of these tumors can increase over time. Pancreatic EUS should be performed once MEN1 is diagnosed to monitor disease progression.
