Prospective endoscopic ultrasonographic evaluation of the frequency of nonfunctioning pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1.
BACKGROUND
The frequency of pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1 (MEN1) remains unknown.


OBJECTIVE
To evaluate prospectively with endoscopic ultrasonography (EUS) the frequency of nonfunctioning (asymptomatic) pancreaticoduodenal tumors.


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 x 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 > or =10 mm and > or = 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.


CONCLUSIONS
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.
Thomas-Marques
Laurence
L
Gastroenterology, Hôpital Robert Debré, CHU de Reims, France.
Murat
Arnaud
A
Delemer
Brigitte
B
Penfornis
Alfred
A
Cardot-Bauters
Catherine
C
Baudin
Eric
E
Niccoli-Sire
Patricia
P
Levoir
Damien
D
Choplin
Hélène du Boullay
Hdu B
Chabre
Olivier
O
Jovenin
Nicolas
N
Cadiot
Guillaume
G
D000293:Adolescent; D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000097:blood; D003937:Diagnosis, Differential; D018450:Disease Progression; D004379:Duodenal Neoplasms / Q000097:blood / Q000000981:diagnostic imaging* / Q000453:epidemiology; D019160:Endosonography; D005260:Female; D005500:Follow-Up Studies; D005755:Gastrins / Q000097:blood; D005934:Glucagon / Q000097:blood; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000097:blood / Q000000981:diagnostic imaging*; D010190:Pancreatic Neoplasms / Q000097:blood / Q000000981:diagnostic imaging* / Q000453:epidemiology; D010191:Pancreatic Polypeptide / Q000097:blood; D010455:Peptides / Q000097:blood; D011446:Prospective Studies; D012720:Severity of Illness Index; D014660:Vasoactive Intestinal Peptide / Q000097:blood
D014408:Biomarkers, Tumor; D005755:Gastrins; D010455:Peptides; C050632:polypeptide C; D014660:Vasoactive Intestinal Peptide; D010191:Pancreatic Polypeptide; D005934:Glucagon