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July 28, 1989, Vol 262, No. 4 >
ARTICLE | July 28, 1989

Multiple Endocrine Neoplasia Type II With Zollinger-Ellison Syndrome Caused by a Solitary Pancreatic Gastrinoma FREE

Paul N. Maton, MD; Jeffrey A. Norton, MD; Lynnette K. Nieman, MD; John L. Doppman, MD; Robert T. Jensen, MD
[+] Author Affiliations

Reprint requests to National Institutes of Health, Bldg 10, Room 9C-103, Bethesda, MD 20892 (Dr Maton).


JAMA. 1989;262(4):535-537. doi:10.1001/jama.1989.03430040107035
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References

THERE are three familial forms of multiple endocrine neoplasia (MEN), all of which are inherited as autosomal dominant traits. Multiple endocrine neoplasia type I is characterized by hyperparathyroidism, pituitary tumors, pancreatic islet cell tumors, and other less common manifestations.1,2 Multiple endocrine neoplasia type II is characterized by medullary carcinoma of the thyroid, pheochromocytoma, and hyperparathyroidism.3,4 Patients with MEN type III, otherwise named "MEN type lib," are similar to patients with MEN type II but also have mucosal neuromas and marfanoid habitus.4,5 These three conditions are inherited separately and are distinct entities. Thus, mucosal neuromas are restricted to MEN type IIb, pheochromocytoma and medullary thyroid carcinoma occur only in MEN type II, and pituitary tumors and islet cell tumors occur only in MEN type I. However, because increasing numbers of patients with MEN syndromes are now being followed up, together with the increasing longevity of these patients, features

REFERENCES

1 +
Ballard HS, Frame B, Hartstock RJ.  Familial multiple endocrine adenoma-peptic ulcer complex . Medicine. 1964;;43:481-516.
2 +
Eberle F, Grun R.  Multiple endocrine neoplasia type 1 (MEN-1) . Ergeb Inn Med Kinderheilkd. 1981;;46:1-148.
3 +
Steiner AL, Goodman AD, Powers SR.  Study of a kindred with pheochromocytoma, medullary thyroid carcinoma, hyperparathyroidism and Cushing's disease: multiple endocrine neoplasia type 2 . Medicine. 1968;;47:371-406.
4 +
Grun R, Eberle F.  Multiple endocrine neoplasia type II (MEN II) . Ergeb Inn Med Kinderheilkd. 1981;;46:150-201.
5 +
Khairi MRA, Dexter RN, Burzynski NJ, Johnston CC.  Mucosal neuroma, pheochromocytoma, and medullary thyroid carcinoma: multiple endocrine neoplasia type III . Medicine. 1975;;54:89-112.
6 +
Zollinger RM, Ellison ED, O'Dorisio TM, Sparks J.  Thirty years experience with gastrinoma . World J Surg. 1984;;8:427-435.
7 +
Hamilton B, Landsberg L, Levine L.  Measurement of urinary epinephrine in screening for pheochromocytoma in multiple endocrine neoplasia type II . Am J Med. 1978;;65:1027-1032.
8 +
Cameron D, Spiro HM, Landsberg L.  Zollinger-Ellison syndrome with multiple endocrine adenomatosis type-II . N Engl J Med. 1976;;299:152-153.
9 +
Jensen RT, Gardner JD, Raufman J-P, Pandol SJ, Doppman JL, Collen MJ.  Zollinger-Ellison syndrome: current concepts and management . Ann Intern Med. 1983;;98:59-75.
10 +
Goldstein DS, Feuerstein G, Izzo JL Jr, Kopin IJ, Keiser HR.  Validity and reliability of liquid chromatography with electrochemical detection for measuring plasma levels of norepinephrine and epinephrine in man . Life Sci. 1981;;28:467-475.
11 +
Bravo EL, Tarazi RC, Gifford RW, Stewart BH.  Circulating and urinary catecholamines in pheochromocytoma: diagnostic and pathophysiologic implications . N Engl J Med. 1979;;301:682-686.
12 +
Bravo EL, Tavazi RC, Fonad FM, Vidt DG, Gifford RW.  Clonidine-suppression test: a useful aid in the diagnosis of pheochromocytoma . N Engl J Med. 1981;;305:623-626.
13 +
Maton PN, Gardner JD, Jensen RT.  The incidence and etiology of Cushing's syndrome in patients with Zollinger-Ellison syndrome . N Engl J Med. 1986;;315:1-5.
14 +
Nieman LK, Chrousos GP, Oldfield EH, Avgerinos PC, Cutler GB Jr, Loriaux DL.  The ovine corticotropin releasing factor stimulation test and the dexamethasone suppression test in the differential diagnosis of Cushing's syndrome . Ann Intern Med. 1986;;105:862-867.
15 +
Oldfield EH, Chrousos GP, Schulte HM, et al.  Preoperative lateralization of ACTH-secreting pituitary microadenomas by bilateral and simultaneous inferior petrosal and venous sinus sampling . NEngl J Med. 1985;;312:100-103.
16 +
Azzopardi JG, Williams ED.  Pathology of nonendocrine tumors associated with Cushing's syndrome . Cancer. 1968;;22:274-286.
17 +
Liddle GW, Nicholson WE, Island DP, Orth DN, Abe K, Lowder SC.  Clinical and laboratory studies of ectopic humoral syndromes . Recent Prog Horm Res. 1969;;25:283-314.
18 +
Melvin KEW, Tashjian AH Jr, Cassidy CE, Givens JR.  Cushing's syndrome caused by ACTH-and calcitonin-secreting medullary carcinoma of the thyroid . Metabolism. 1970;;19:831-838.
19 +
Keusch G, Binswanger U, Dambacher MA, et al.  Ectopic ACTH syndrome and medullary thyroid carcinoma . Acta Endocrinol. 1977;;86:306-316.
20 +
Rosenberg EM, Hahn TJ, Orth DN, Deftos LJ, Tanaka K.  ACTH-secreting medullary carcinoma of the thyroid presenting as severe idiopathic osteoporosis and senile purpura: report of a case and review of the literature . J Clin Endocrinol Metab. 1978;;47:255-262.
21 +
Jolivet J, Beauregard H, Somma M, et al.  ACTH-secreting medullary carcinoma of the thyroid: monitoring of clinical course with calcitonin and cortisol assays and immunohistochemical studies . Cancer. 1980;;46:2667-2670.
22 +
Slaff JI, Howard JM, Maton PN, et al.  Prospective assessment of gastrin tests in 81 consecutive patients with Zollinger-Ellison syndrome (ZES) . Gastroenterology. 1986;;90:1637. Abstract.
23 +
Hayes JR, Ardill J, Kennedy TL, Shanks RG, Buchanan KD.  Stimulation of gastrin release by catecholamines . Lancet. 1972;;1:819-821.
24 +
Sizemore GW, Go VLW, Kaplan EL, Sanzenbacher LJ, Holtermuller KH, Arnaud CD.  Relations of calcitonin and gastrin in the Zollinger-Ellison syndrome . NEngl J Med. 1973;;288:641-644.
25 +
FahrenkrugJ, Hornum I, Rehfeld JF.  Effect of calcitonin on serum gastrin concentration and component pattern in man . J Clin Endocrinol Metab. 1975;;41:149-152.
26 +
O'Dorisio TM, Falko JM, Cataland S, et al.  Gastrin responses to a test meal in patients with familial medullary thyroid carcinoma . J Clin Endocrinol Metab. 1982;;54:798-802.
27 +
Norton JA, Doppman JL, Collen MJ, et al.  Prospective study of gastrinoma localization and resection in patients with Zollinger-Ellison syndrome . Ann Surg. 1986;;204:468-479.
28 +
Jensen RT, Doppman JL, Gardner JD.  Gastrinoma . In: Go VLW, Gardner JD, Brooks PP, Lebenthal E, DiMagno EP, Scheele GA, eds. The Exocrine Pancreas: Biology, Pathobiology and Diseases . New York, NY: Raven Press; 1986;:727-744.
29 +
Nathan DM, Daniels GH, Ridgeway EC.  Gastrinoma and pheochromocytoma: is there a mixed multiple endocrine adenoma syndrome? Acta Endocrinol . 1980;;93:91-93.

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1 +
Ballard HS, Frame B, Hartstock RJ.  Familial multiple endocrine adenoma-peptic ulcer complex . Medicine. 1964;;43:481-516.
2 +
Eberle F, Grun R.  Multiple endocrine neoplasia type 1 (MEN-1) . Ergeb Inn Med Kinderheilkd. 1981;;46:1-148.
3 +
Steiner AL, Goodman AD, Powers SR.  Study of a kindred with pheochromocytoma, medullary thyroid carcinoma, hyperparathyroidism and Cushing's disease: multiple endocrine neoplasia type 2 . Medicine. 1968;;47:371-406.
4 +
Grun R, Eberle F.  Multiple endocrine neoplasia type II (MEN II) . Ergeb Inn Med Kinderheilkd. 1981;;46:150-201.
5 +
Khairi MRA, Dexter RN, Burzynski NJ, Johnston CC.  Mucosal neuroma, pheochromocytoma, and medullary thyroid carcinoma: multiple endocrine neoplasia type III . Medicine. 1975;;54:89-112.
6 +
Zollinger RM, Ellison ED, O'Dorisio TM, Sparks J.  Thirty years experience with gastrinoma . World J Surg. 1984;;8:427-435.
7 +
Hamilton B, Landsberg L, Levine L.  Measurement of urinary epinephrine in screening for pheochromocytoma in multiple endocrine neoplasia type II . Am J Med. 1978;;65:1027-1032.
8 +
Cameron D, Spiro HM, Landsberg L.  Zollinger-Ellison syndrome with multiple endocrine adenomatosis type-II . N Engl J Med. 1976;;299:152-153.
9 +
Jensen RT, Gardner JD, Raufman J-P, Pandol SJ, Doppman JL, Collen MJ.  Zollinger-Ellison syndrome: current concepts and management . Ann Intern Med. 1983;;98:59-75.
10 +
Goldstein DS, Feuerstein G, Izzo JL Jr, Kopin IJ, Keiser HR.  Validity and reliability of liquid chromatography with electrochemical detection for measuring plasma levels of norepinephrine and epinephrine in man . Life Sci. 1981;;28:467-475.
11 +
Bravo EL, Tarazi RC, Gifford RW, Stewart BH.  Circulating and urinary catecholamines in pheochromocytoma: diagnostic and pathophysiologic implications . N Engl J Med. 1979;;301:682-686.
12 +
Bravo EL, Tavazi RC, Fonad FM, Vidt DG, Gifford RW.  Clonidine-suppression test: a useful aid in the diagnosis of pheochromocytoma . N Engl J Med. 1981;;305:623-626.
13 +
Maton PN, Gardner JD, Jensen RT.  The incidence and etiology of Cushing's syndrome in patients with Zollinger-Ellison syndrome . N Engl J Med. 1986;;315:1-5.
14 +
Nieman LK, Chrousos GP, Oldfield EH, Avgerinos PC, Cutler GB Jr, Loriaux DL.  The ovine corticotropin releasing factor stimulation test and the dexamethasone suppression test in the differential diagnosis of Cushing's syndrome . Ann Intern Med. 1986;;105:862-867.
15 +
Oldfield EH, Chrousos GP, Schulte HM, et al.  Preoperative lateralization of ACTH-secreting pituitary microadenomas by bilateral and simultaneous inferior petrosal and venous sinus sampling . NEngl J Med. 1985;;312:100-103.
16 +
Azzopardi JG, Williams ED.  Pathology of nonendocrine tumors associated with Cushing's syndrome . Cancer. 1968;;22:274-286.
17 +
Liddle GW, Nicholson WE, Island DP, Orth DN, Abe K, Lowder SC.  Clinical and laboratory studies of ectopic humoral syndromes . Recent Prog Horm Res. 1969;;25:283-314.
18 +
Melvin KEW, Tashjian AH Jr, Cassidy CE, Givens JR.  Cushing's syndrome caused by ACTH-and calcitonin-secreting medullary carcinoma of the thyroid . Metabolism. 1970;;19:831-838.
19 +
Keusch G, Binswanger U, Dambacher MA, et al.  Ectopic ACTH syndrome and medullary thyroid carcinoma . Acta Endocrinol. 1977;;86:306-316.
20 +
Rosenberg EM, Hahn TJ, Orth DN, Deftos LJ, Tanaka K.  ACTH-secreting medullary carcinoma of the thyroid presenting as severe idiopathic osteoporosis and senile purpura: report of a case and review of the literature . J Clin Endocrinol Metab. 1978;;47:255-262.
21 +
Jolivet J, Beauregard H, Somma M, et al.  ACTH-secreting medullary carcinoma of the thyroid: monitoring of clinical course with calcitonin and cortisol assays and immunohistochemical studies . Cancer. 1980;;46:2667-2670.
22 +
Slaff JI, Howard JM, Maton PN, et al.  Prospective assessment of gastrin tests in 81 consecutive patients with Zollinger-Ellison syndrome (ZES) . Gastroenterology. 1986;;90:1637. Abstract.
23 +
Hayes JR, Ardill J, Kennedy TL, Shanks RG, Buchanan KD.  Stimulation of gastrin release by catecholamines . Lancet. 1972;;1:819-821.
24 +
Sizemore GW, Go VLW, Kaplan EL, Sanzenbacher LJ, Holtermuller KH, Arnaud CD.  Relations of calcitonin and gastrin in the Zollinger-Ellison syndrome . NEngl J Med. 1973;;288:641-644.
25 +
FahrenkrugJ, Hornum I, Rehfeld JF.  Effect of calcitonin on serum gastrin concentration and component pattern in man . J Clin Endocrinol Metab. 1975;;41:149-152.
26 +
O'Dorisio TM, Falko JM, Cataland S, et al.  Gastrin responses to a test meal in patients with familial medullary thyroid carcinoma . J Clin Endocrinol Metab. 1982;;54:798-802.
27 +
Norton JA, Doppman JL, Collen MJ, et al.  Prospective study of gastrinoma localization and resection in patients with Zollinger-Ellison syndrome . Ann Surg. 1986;;204:468-479.
28 +
Jensen RT, Doppman JL, Gardner JD.  Gastrinoma . In: Go VLW, Gardner JD, Brooks PP, Lebenthal E, DiMagno EP, Scheele GA, eds. The Exocrine Pancreas: Biology, Pathobiology and Diseases . New York, NY: Raven Press; 1986;:727-744.
29 +
Nathan DM, Daniels GH, Ridgeway EC.  Gastrinoma and pheochromocytoma: is there a mixed multiple endocrine adenoma syndrome? Acta Endocrinol . 1980;;93:91-93.

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