Peptide hormone markers in screening for endocrine tumors in multiple endocrine adenomatosis type I

      This paper is only available as a PDF. To read, Please Download here.


      In three families with the multiple endocrine adenomatosis type I (MEA I) trait, 51 members were investigated by measurement of circulating peptide hormones as tumor markers. Twenty-five of 51 members (49 percent) were considered to be affected by MEA I disorders. The incidence rose with age (75 percent in generation II). Both sexes were affected equally. Hyperparathyroidism was present in 20 of 25 affected members (80 percent), and pituitary tumors (prolactinomas) were found in four of 25 (16 percent). Endocrine pancreatic tumors were found in nine of 25 affected members (36 percent), but when “probable” tumors (seven) are included the frequency rises to 72 percent. Hyperparathyroidism was found in all except one member with proved lesions in other organs. Among patients with proved and possible endocrine pancreatic tumors, elevated serum levels of gastrin and pancreatic polypeptide were frequently found, 78 percent and 67 percent, respectively, and we suggest that serum gastrin and pancreatic polypeptide levels are the most useful screening markers at present for pancreatic lesions in MEA I.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to The American Journal of Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Wermer P
        Genetic aspects of adenomatosis of endocrine glands.
        Am J Med. 1954; 16: 363-371
        • Underdahl LO
        • Woolner LB
        • Black BM
        Multiple endocrine adenomatosis. Report of 8 cases in which the parathyroids, pituitary and pancreatic islets were involved.
        J Clin Endocrinol. 1953; 13: 20-47
        • Ballard H
        • Frame B
        • Hartsock RJ
        Familial multiple endocrine adenoma-peptic ulcer complex.
        Medicine (Baltimore). 1964; 43: 481-516
        • Prosser PR
        • Karam JH
        • Townsend J
        • Forsham PH
        Prolactin secreting pituitary adenomas in multiple endocrine adenomatosis type I.
        Ann Intern Med. 1979; 91: 41-44
        • Lamers CBHW
        • Froeling PGAM
        Clinical significance of hyperparathyroidism in familial multiple endocrine adenomatosis type I (MEA I).
        Am J Med. 1979; 66: 422-424
        • Yamaguchi K
        • Kameya T
        • Abe K
        Multiple endocrine neoplasia type I.
        Clin Endocrinol Metab. 1980; 9: 261-284
        • Ingemansson SG
        Pancreatic and intestinal vein catheterization with hormone assay. A localization procedure for gastrointestinal tumours.
        Bulletin No. 13 of the Department of Surgery, University of Lund. 1977; (thesis)
        • Almqvist S
        • Telenius-Berg M
        • Wästhed B
        Serum calcitonin in medullary thyroid carcinoma.
        Acta Med Scand. 1974; 196: 177-180
        • Berg T
        • Nillius SJ
        • Wide L
        Bromocriptin treatment of 42 hyperprolactinaemic women with secondary amenorrhoea.
        Acta Endocrinol (Copenh). 1978; 88: 435-451
        • Lundqvist G
        • Wide L
        Serum gastrin determination with a radioimmunosorbent technique.
        Clin Chim Acta. 1977; 79: 357-362
        • Hällgren R
        • Lundqvist G
        • Chance RE
        Serum levels of human pancreatic polypeptide in renal disease.
        Scand J Gastroenterol. 1977; 12: 923-927
        • Öberg K
        • Wide L
        HCG and HCG subunits as tumour markers in patients with endocrine pancreatic tumours and carcinoids.
        Acta Endocrinol (Copenh). 1981; 98: 256-260
        • Fahrenkrug J
        • Schaffalitzky de Muckadell OB
        Radioimmunoassay of vasoactive intestinal polypeptide (VIP) in plasma.
        J Lab Clin Med. 1977; 89: 1379-1388
        • Öberg K
        • Boström H
        • Fahrenkrug J
        • et al.
        Streptozotocin treatment of a pancreatic tumour producing VIP and gastrin, associated with Verner-Morrison syndrome.
        Acta Med Scand. 1979; 206: 223-227
        • Johnsson GJ
        • Summerskill WHJ
        • Andersson VE
        • Keating FR
        Clinical and genetic investigation of a large kindred with multiple endocrine adenomatosis.
        N Engl J Med. 1967; 277: 1379-1385
        • Snyder N
        • Scurry MT
        • Deiss WP
        Five families with multiple endocrine adenomatosis.
        Ann Intern Med. 1972; 76: 33-58
        • Marx SJ
        • Spiegel AM
        • Brown EM
        • Aurbach GD
        Family studies in patients with primary parathyroid hyperplasia.
        Am J Med. 1977; 62: 698-706
        • Prosser RP
        • Karam JH
        • Towsend JJ
        • Forsham PH
        Prolactinsecreting pituitary adenomas in multiple endocrine adenomatosis type I.
        Ann Intern Med. 1979; 91: 41-44
        • Makhlouf GM
        • Said SI
        • Yau M
        Interplay of vasoactive intestinal polypeptide (VIP) and synthetic VIP fragments with secretin and octapeptide of cholecystokinin (Octa-CCK) on pancreatic and biliary secretion.
        Gastroenterology. 1974; 66: 737
        • Skrabanek P
        • Mc Partlin J
        • Powell D
        Tumour hypercalcemia and “ectopic hyperparathyroidism.”.
        Medicine (Baltimore). 1980; 59: 262-282
        • Franklin RB
        • Tashjian Jr, AH
        Intravenous infusion of prostaglandin E2 raises plasma calcium concentration in the rat.
        Endocrinology. 1975; 97: 240-243
        • Grevsten S
        • Grimelius L
        • Thorén L
        Familial hyperparathyroidism.
        Ups J Med Sci. 1974; 79: 109-115
        • Wilson SD
        Ulcerogenic tumours of the pancreas, the Zollinger-Ellison syndrome.
        in: Carey LC The pancreas. CV Mosby, St Louis1973: 295-318
        • Lamers CBHW
        • Stadil F
        • van Tongeren JH
        Prevalence of endocrine abnormalities in patients with the Zollinger-EIlison syndrome and in their families.
        Am J Med. 1978; 64: 607-612
        • Stefanini P
        • Carboni M
        • Patrassi N
        Surgical treatment and prognosis of insulinoma.
        Clin Gastroenterol. 1974; 3: 697-709
        • Yamaguchi K
        • Abe K
        • Miyakawa S
        • et al.
        Multiple hormone production in endocrine tumors of the pancreas.
        in: Miyoshi A Gut peptides, secretion, function and Clinical aspects. Kodanska, Tokyo1980: 343-350
        • Modlin I
        • Bloom SR
        • Mitchell SJ
        Experimental evidence for vasoactive intestinal peptide as the cause of the watery diarrhea syndrome.
        Gastroenterology. 1978; 75: 1051-1054
        • Kraft AR
        • Tompkins RK
        • Zollinger RM
        Recognition and management of the diarrheal syndrome caused by non-beta islet cell tumors of the pancreas.
        Am J Surg. 1970; 119: 163-170
        • Zollinger RM
        • Ellison EH
        Primary peptic ulceration of the jejunum associated with islet cell tumors of the pancreas.
        Ann Surg. 1955; 142: 709-723
        • Floyd JC
        • Fajans SS
        • Pek S
        • Chance RE
        A newly recognized pancreatic polypeptide, plasma levels in health and disease.
        Recent Prog Horm Res. 1977; 33: 519-570
        • Larsson L-I
        • Schwartz T
        • Lundqvist G
        • et al.
        Occurrence of human pancreatic polypeptide in pancreatic endocrine tumors.
        Am J Pathol. 1976; 85: 675-684
        • Bloom SR
        VIP and watery diarrhoea: IV.
        in: Bloom SR Gut hormones. Churchill Livingstone, Edinburgh1978: 583-588
        • Reuter AM
        • Gaspard KJ
        • Deville J-L
        • Vrindts-Gevaert Y
        • Franchimont P
        Serum concentrations of human chorionic gonadotropin and its alpha and beta subunits during normal singleton and twin pregnancies.
        Clin Endocrinol. 1980; 13: 305-318
        • Kahn CR
        • Rosen SW
        • Weihtraub BD
        • Fajans SS
        • Gorden P
        Ectopic production of chorionic gonadotropin and its subunits by islet cell tumors, a specific marker for malignancy.
        N Engl J Med. 1977; 297: 565-569
        • Öberg K
        • Löōf L
        • Boström H
        • Grimelius L
        • Fahrenkrug J
        • Lundqvist G
        Hypersecretion of calcitonin in patients with the Verner-Morrison syndrome.
        Scand J Gastroenterol. 1981; 16: 135-144
        • Kaplan EL
        • Lee CH
        Recent advances in the diagnosis and treatment of insulinomas.
        Surg Clin North Am. 1979; 59: 119-129