Abstract
Early identification of pancreaticoduodenal endocrine tumors (PETs) in multiple endocrine neoplasia type 1 (MEN-1) is mandatory, because these tumors represent the most common cause of death within the syndrome. The diagnostic value of imaging procedures has therefore been evaluated in a prospective observational study. Between December 1997 and June 2003 twenty-two MEN-1 patients with genetically confirmed disease were followed for PETs using a standardized screening program with serum hormone measurements, endoscopic ultrasonography (EUS), computed tomography (CT), and somatostatin-receptor scintigraphy (SRS). Results could be validated by surgery and histopathology in 13 patients during 18 operations. In 12 asymptomatic patients with tumors measuring 10 mm or less, who have not yet undergone operation, PETs were detected by EUS in 12/12, by CT in 1/12, and by SRS in 2/11 cases. In 13 patients who have undergone surgical exploration EUS, CT, and SRS were true positive in 12 of 16, 7 of 13, and 12 of 17 cases, respectively, although the number of tumors detected by each imaging procedure alone was lower than the number detected intraoperatively and histopathologically in almost every case. A solitary liver metastasis in one patient and a nonfunctioning PET recurrence in another were identified only by SRS. Endoscopic untrasonography is the most sensitive imaging procedure for the detection of small (≤ 10 mm) PETs in MEN-1, whereas SRS is the procedure of choice for the identification of metastases of MEN-1 PETs—i.e., for staging. Detection of PETs at an early stage by an aggressive screening program using EUS may lead to prompt surgical intervention and improved prognosis of MEN-1 PETs.
Similar content being viewed by others
References
P Wermer (1954) ArticleTitleGenetic aspects of adenomatosis of endocrine glands Am. J. Med. 16 363–371 Occurrence Handle10.1016/0002-9343(54)90353-8 Occurrence Handle1:STN:280:CyuD2MfkvFM%3D Occurrence Handle13138607
SE Carty AK Helm JA Amico et al. (1998) ArticleTitleThe variable penetrance and spectrum of manifestations of multiple endocrine neoplasia type 1 Surgery 124 1106–1114 Occurrence Handle10.1067/msy.1998.93107 Occurrence Handle1:STN:280:DyaK1M%2FntFOmtw%3D%3D Occurrence Handle9854591
D Trump B Farren C Wooding et al. (1996) ArticleTitleClinical studies of multiple endocrine neoplasia type 1 (MEN 1) Q. J. Med. 89 653–659 Occurrence Handle1:STN:280:ByiD2srjsV0%3D
P Langer A Wild C Nies et al. (2001) ArticleTitleVariable expression of multiple endocrine neoplasia type 1—implications for screening strategies Int. J. Surg. Invest. 3 473–481
L Benson S Ljunghall G Akerstrom (1987) ArticleTitleHyperparathyroidism presenting as the first lesion in multiple endocrine neoplasia type 1 Am. J. Med. 82 731–737 Occurrence Handle10.1016/0002-9343(87)90008-8 Occurrence Handle1:STN:280:BiiC1c7nsVE%3D Occurrence Handle2882676
HFA Vasen CB Lamers CJM Lips (1989) ArticleTitleScreening for the multiple endocrine neoplasia syndrome type 1: a study of 11 kindreds in the Netherlands Arch. Intern. Med. 149 2717–2722 Occurrence Handle10.1001/archinte.149.12.2717 Occurrence Handle1:STN:280:By%2BD1cfgsVY%3D Occurrence Handle2574567
GM Doherty JA Olson MM Frisella et al. (1998) ArticleTitleLethality of multiple endocrine neoplasia type I World J. Surg. 22 581–586 Occurrence Handle10.1007/s002689900438 Occurrence Handle1:STN:280:DyaK1c3mtVahsQ%3D%3D Occurrence Handle9597932
HC Weber DJ Venzon JT Lin et al. (1995) ArticleTitleDeterminants of metastatic rate and survival in patients with Zollinger-Ellisonsyndrome: a prospective long-term study Gastroenterology 108 1637–1649
K Oberg B Skogseid (1998) ArticleTitleThe ultimate biochemical diagnosis of endocrine pancreatic tumours in MEN-1 J. Intern. Med. 243 471–476 Occurrence Handle10.1046/j.1365-2796.1998.00280.x Occurrence Handle1:CAS:528:DyaK1cXks1Wktbc%3D Occurrence Handle9681845
B Skogseid K Oberg L Benson et al. (1987) ArticleTitleA standardised meal stimulation test of the endocrine pancreas for early detection of pancreatic endocrine tumours in multiple endocrine neoplasia type 1 syndrome: five years experience J. Clin. Endocrinol. Metab. 64 1233–1240 Occurrence Handle1:STN:280:BiiC1MrislM%3D Occurrence Handle2883195
P Langer A Wild I Celik et al. (2001) ArticleTitleProspective controlled trial of a standardised meal stimulation test in the detection of pancreaticoduodenal endocrine tumours in patients with multiple endocrine neoplasia type 1 Br. J. Surg. 88 1403–1407 Occurrence Handle10.1046/j.0007-1323.2001.01874.x Occurrence Handle1:STN:280:DC%2BD3Mrjt1SmtA%3D%3D Occurrence Handle11578300
ML Brandi RF Gagel A Angeli et al. (2001) ArticleTitleGuidelines for diagnosis and therapy of MEN type 1 and type 2 J. Clin. Endocrinol. Metab. 86 5658–5671
B Skogseid K Oberg G Akerstrom et al. (1998) ArticleTitleLimited tumor involvement found at multiple endocrine neoplasia type i pancreatic exploration: can it be predicted by preoperative tumor localization? World J. Surg. 22 673–677 Occurrence Handle10.1007/s002689900451 Occurrence Handle1:STN:280:DyaK1c3ntV2ksw%3D%3D Occurrence Handle9606280
JH Yim BA Siegel MK DeBenedetti et al. (1998) ArticleTitleProspective study of the utility of somatostatin-receptor scintigraphy in the evaluation of patients with multiple endocrine neoplasia type 1 Surgery 124 1037–1042 Occurrence Handle10.1067/msy.1998.92553 Occurrence Handle1:STN:280:DyaK1M%2FntFOntg%3D%3D Occurrence Handle9854580
MA Anderson S Carpenter NW Thompson et al. (2000) ArticleTitleEndoscopic ultrasound is highly accurate and directs management in patients with neuroendocrine tumors of the pancreas Am. J. Gastroenterol. 95 2271–2277 Occurrence Handle1:STN:280:DC%2BD3cvltFGnsg%3D%3D Occurrence Handle11007228
C Proye P Malvaux F Pattou et al. (1998) ArticleTitleNoninvasive imaging of insulinomas and gastrinomas with endoscopic ultrasonography and somatostatin receptor scintigraphy Surgery 124 1134–1143 Occurrence Handle10.1067/msy.1998.93109 Occurrence Handle1:STN:280:DyaK1M%2FntFOmuw%3D%3D Occurrence Handle9854595
G Cadiot A Vuagnat I Doukhan et al. (1999) ArticleTitlePrognostic factors in patients with Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1 Gastroenterology 116 286–293
G Akerström O Hessman B Skogseid (2002) ArticleTitleTiming and extent of surgery in symptomatic and asymptomatic neuroendocrine tumours of the pancreas in MEN 1 Langenbecks Arch. Surg. 386 558–569 Occurrence Handle10.1007/s00423-001-0274-6 Occurrence Handle11914931
JB Meko GM Doherty BA Siegel (1996) ArticleTitleNorton, J.A.: Evaluation of somatostatin-receptor scintigraphy for detecting neuroendocrine tumours Surgery 120 975–983 Occurrence Handle1:STN:280:ByiC3MnjvFE%3D Occurrence Handle8957483
JB Meko JA Norton (1995) ArticleTitleManagement of patients with Zollinger-Ellison syndrome Annu. Rev. Med. 46 395–411 Occurrence Handle10.1146/annurev.med.46.1.395 Occurrence Handle1:CAS:528:DyaK2MXkvFSksb4%3D Occurrence Handle7598474
T Zimmer H Scherübl S Faiss et al. (2000) ArticleTitleEndoscopic ultrasonography of neuroendocrine tumors Digestion 62 45–50 Occurrence Handle10.1159/000051855 Occurrence Handle10940687
NW Thompson PF Czako LL Fritts et al. (1994) ArticleTitleRole of endoscopic ultrasonography in the localization of insulinomas and gastrinomas Surgery 116 1131–1138 Occurrence Handle1:STN:280:ByqD2snjslc%3D Occurrence Handle7985098
PG Gauger JM Scheiman E-J Wamsteker et al. (2003) ArticleTitleRole of endoscopic ultrasonography in screening and treatment of pancreatic endocrine tumours in asymptomatic patients with multiple endocrine neoplasia type 1 Br. J. Surg. 90 748–754 Occurrence Handle10.1002/bjs.4142 Occurrence Handle1:STN:280:DC%2BD3s3otVCqsw%3D%3D Occurrence Handle12808627
PH Kann B Wirkus A Keth et al. (2003) ArticleTitlePitfalls in endosonographic imaging of suspected insulinomas: pancreatic nodules of unknown dignity Eur. J. Endocrinol. 148 531–534 Occurrence Handle1:CAS:528:DC%2BD3sXktVGjs7c%3D Occurrence Handle12720536
P Kann F Bittinger M Engelbach et al. (2001) ArticleTitleEndosonography of insulin-secreting and clinically non-functioning neuroendocrine tumors of the pancreas: criteria for benignancy and malignancy Eur. J. Med. Res. 28 385–391
P Langer K Cupisti DK Bartsch et al. (2002) ArticleTitleAdrenal involvement in multiple endocrine neoplasia type 1 World J. Surg. 26 891–896 Occurrence Handle10.1007/s00268-002-6492-4 Occurrence Handle12016472
B Skogseid J Rastad A Gobl et al. (1995) ArticleTitleAdrenal lesion in multiple endocrine neoplasia type 1 Surgery 118 1077–1082 Occurrence Handle1:STN:280:BymD1cbgt1E%3D Occurrence Handle7491526
DK Bartsch P Langer A Wild et al. (2000) ArticleTitlePancreticoduodenal endocrine tumors in multiple endocrine neoplasia type 1 – surgery or surveillance? Surgery 128 958–966 Occurrence Handle10.1067/msy.2000.109727 Occurrence Handle1:STN:280:DC%2BD3M%2FnsFentQ%3D%3D Occurrence Handle11114630
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Langer, P., Kann, P., Fendrich, V. et al. Prospective Evaluation of Imaging Procedures for the Detection of Pancreaticoduodenal Endocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1. World J. Surg. 28, 1317–1322 (2004). https://doi.org/10.1007/s00268-004-7642-7
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00268-004-7642-7