Abstract
The multiple endocrine neoplasia (MEN) syndromes are a family of genetic conditions characterized by a predisposition to the development of neoplasms in multiple endocrine glands. The pathologic change in affected glands is characteristically multicentric and may be expressed as hyperplasia, adenoma, or carcinoma. The MEN syndromes are inherited in an autosomal dominant manner and are classified according to the pattern of involvement (Table 1). MEN type I is characterized by parathyroid hyperplasia, pancreatic islet cell neoplasms, and adenomas of the anterior pituitary gland. Less commonly, patients with MEN-I can have adrenocortical or thyroid adenomas, lipomas, and carcinoid tumors. MEN-IIa is characterized by medullary thyroid carcinoma (MTC), adrenal pheochromocytoma, and parathyroid hyperplasia. MEN-IIb consists of medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas, and a characteristic marfanoid habitus. MEN-IIb does not involve the parathyroid glands. Familial medullary thyroid carcinoma (FMTC) consists of medullary thyroid carcinoma alone without other abnormalities.
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References
Erdheim J. Zur normalen und pathologischen Histologie der Glandula thyreoidea, parathyreoidea, und hypophesis. Beitr Pathol Anat Allg Pathol 33: 158, 1903.
Cushing H, Davidoff SM. The Pathological Findings in Four Autopsied Cases of Acromegaly with a Discussion of their Significance. Monograph 22. New York. The Rockefeller Institute for Medical Research, 1927.
Wermer P. Endocrine adenomatosis and peptic ulcer in a large kindred: Inherited multiple tumors and mosaic pleiotropism in man. Am J Med 35: 205, 1963.
Zollinger RM, Ellison EH. Primary peptic ulceration of the jejunum associated with islet cell tumors of the pancreas. Ann Surg 142: 709, 1955.
McGuigan JE. Gastric mucosal intracellular localization of gastrin by immunofluorescence. Gastroenterology 55: 315, 1968.
Larsson C, Skogseid B, Oberg K, Nakamura V, Nordenskjold M. Multiple endocrine neoplasia type 1 gene maps to chromosome 11 and is lost in insulinoma. Nature 332: 85, 1988.
Arnold A. Molecular mechanisms of parathyroid neoplasia. Endocrinol Metab Clin North Am 23: 1, 1994.
Melmed S. Pituitary neoplasia. Endocrinol Metab Clin North Am 23: 1, 1994.
Larsson C, Friedman E. Localization and identification of the multiple endocrine neoplasia type 1 disease gene. Endocrinol Metab Clin North Am 23: 1, 1994.
Lyons J, Landis CA, Harsh G, et al. Two G-protein oncogenes in human endocrine tumors. Science. 249: 655–659, 1990.
Landis CA, Masters SB, Spada A, Pace AM, Bourne HR, Vallar L. GTPase inhibiting mutations activate the alpha chain of Gs and stimulate adenyl cyclase in human pituitary tumors. Nature 340: 692–696, 1989.
Chandrasekharappa SC, Guru SC, Manickam P, et al. Positional cloning of the gene for Multiple Endocrine Neoplasia-type 1. Science 276: 404–407, 1997.
Mallette LE. Management of hyperparathyroidism in the multiple endocrine neoplasia syndromes and other familial endocrinopathies. Endocrinol Metab Clin North Am 23: 1, 1994.
Prinz RA, Bamvros OI, Sellu D, Lynn JA. Subtotal parathyroidectomy for primary chief cell hyperplasia in the multiple endocrine neoplasia type I syndrome. Ann Surg 193: 26, 1981.
van Heerden JA, Kent RB, Sizemore GW, Grant CS, ReMine WM. Primary hyperparathyroidism in patients with multiple endocrine neopalsia syndromes: Surgical experience. Arch Surg 118: 533, 1983.
Wells SA, Jr., Farndon JR, Dale JK, Leight GE, Dilley WG. Long term evaluation of patients with primary parathyroid hyperplasia managed by total parathyroidectomy and heterotopic autotransplantation. Ann Surg 192: 451, 1980.
Hazard JB, Hawk WA, Crile G, Jr. Medullary (solid) carcinoma of the thyroid: A clinico-pathologic entity. J Clin endocrinol Metab 19: 152, 1959.
Sipple JH. The association of pheochromocytoma with carcinoma of the thyroid gland. Am J Med 31: 163, 1961.
Williams ED. A review of 17 cases of carcinoma of the thyroid and pheochromocytoma. J Clin Pathol 18: 288, 1965.
Williams ED. Histogenesis of medullary carcinoma of the thyroid. J Clin Pathol 19: 114, 1966.
Tashjihan AH, Jr., Howland BG, Melvin KEW, Hill CS, Jr. Immunoassay of human calcito-nin: Clinical measurement, relation to serum calcium, and studies in patients with medullary carcinoma. N Engl J Med 283: 890, 1970.
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 47: 371, 1968.
Williams ED, Pollack DJ. Multiple mucosal neuromata with endocrine tumours: A syndrome allied to von Recklinghausen’s disease. J Pathol Bacteriol 91: 71, 1966.
Chong GC, Beahrs OH, Sizemore GW, Woolner LH. Medullary carcinoma of the thyroid gland. Cancer 35: 695, 1975.
Lairmore TC, Dou S, Howe JR, et al. A 1.5 megabase yeast artificial chromosome cintig from human chromosome 10q11.2 connecting three genetic loci (RET, D10S94, and D10S102) closely linked to the MEN-2A locus. Proc Natl Acad Sci USA 90: 492–496, 1993.
Nagao M, Ishizaka Y, Nakagawara A, et al. Expression of RET proto-oncogene in human neuroblastomas. Jpn J Cancer Res 81: 309–312, 1990.
Santoro M, Rosati R, Grieco M, Berlingieri MT, D’Amato GL, de Franciscis V, Fusco A. The RET proto-oncogene is consistently expressed in human pheochromocytomas and medullary thyroid carcinomas. Oncogene 5: 1595–1598, 1990.
Pachnis V, Mankoo B, Costantini F. Expression of the c-ret proto-oncogene during mouse embryogenesis. Development 119: 1005–1017, 1993.
Santoro M, Carlomagno F, Romano A, et al. Activation of RET as a dominant transforming gene by germline mutations of MEN 2A and MEN 2B. Science 267: 381–383, 1995.
Romeo G, Ranchetto P, Luo V, et al. Point mutations affecting the tyrosine kinase domain of the RET proto-oncogene in Hirschprung’s disease. Nature 367: 377–378, 1994.
Edery P, Lyonnet S, Mulligan LM, et al. Mutations of the RET proto-oncogene in Hirschprung’s disease. Nature 367: 378–380, 1994.
vanHeyningen V. One gene: Four syndromes. Nature 367: 319–320, 1994.
Wells SA, Jr., Baylin SB, Linehan WM, Farrell RE, Cox EB, Cooper CW. Provocative agents and the diagnosis of medullary carcinoma of the thyroid gland. Ann Surg 188: 139, 1978.
Wells SA, Jr., Baylin SB, Gann DS, Farrell RE, Dilley WG, Preissig SH, Linehan WM, Cooper CW. Medullary thyroid carcinoma: Relationship of method of diagnosis to pathologic staging. Ann Surg 188: 377, 1978.
Tisell LE, Hansson G, Jansson S, Salander H. Reoperation in the treatment of asymptomatic metastasizing medullary thyroid carcinoma. Surgery 99: 60–66, 1986.
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Miller, J.A., Norton, J.A. (1997). Multiple endocrine neoplasia. In: Pollock, R.E. (eds) Surgical Oncology. Cancer Treatment and Research, vol 90. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-6165-1_11
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DOI: https://doi.org/10.1007/978-1-4615-6165-1_11
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