Abstract
The multiple endocrine neoplasia (MEN) syndromes are genetically transmitted endocrinopathies. In both types of MEN syndrome, usually one organ predominantly is affected; however, the individual manifestation of the disease with regard to time and extent of organ involvement varies considerably. Unlike in MEN 1, in MEN 2 surgical treatment represents the therapy of choice for each part of the disease.
We are grateful to Mrs. G. Martin for typing the manuscript and drawing the graphs.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsPreview
Unable to display preview. Download preview PDF.
References
Bigner SH, Mendelsohn G, Wells SA, Cox EB, Baylin SB, Eggleston JC (1981) Medullary carcinoma of the thyroid in multiple endocrine neoplasia IIA syndrome. Am J Pathol 5: 459–472
Block MA (1990) Surgical treatment of medullary carcinoma of the thyroid. Otolaryngol Clin North Am 23: 453–473
Block MA, Jackson CE, Tashjian AH (1975) Management of parathyroid glands in surgery for medullary thyroid carcinoma. Arch Surg 110: 617–624
Block MA, Jackson CE, Greenawald KA, Yott JB, Tashjian AH (1980) Clinical characteristics distinguishing hereditary from sporadic medullary thyroid carcinoma. Arch Surg 115: 142–148
Brunt LM, Wells SA (1987) Advances in the diagnosis and treatment of medullary thyroid carcinoma. Surg Clin North Am 67: 263–279
Carney JA, Sizemore GW, Sheps SG (1976) Adrenal medullary disease in multiple endocrine neoplasia, type 2. Am J Clin Pathol 66: 279–290
Carney JA, Sizemore GW, Lovestedt SA (1976) Musosal ganglioneuromatosis, medullary thyroid carcinoma, and pheochromocytoma: multiple endocrine neoplasia, type 2b. Oral Surg 41: 739–752
Carney JA, Sizemore GW, Hayles AB (1976) Alimentary-tract ganglioneuromatosis. A major component of the syndrome of multiple endocrine neoplasia, type 2. N Engl J Med 295: 1287–1291
Carney JA, Roth SI, Heath H III, Sizemore W, Hayles AB (1980) The parathyroid glands in multiple endocrine neoplasia type 2b. Am J Pathol 99: 387–398
Cho KJ, Freier DT, McCormick TL, Nishiyama RH, Forrest ME, Kaufman A, Borlaza GS (1980) Adrenal medullary disease in multiple endocrine neoplasia type II. AJR 134: 23–29
Chong GC, Beahrs OH, Sizemore GW, Woolner LH (1975) Medullary carcinoma of the thyroid gland. Cancer 35: 695–704
Decker RA, Wells SA (1989) Multiple endocrine neoplasia. Jpn J Surg 19: 645–657
Dralle H, Schürmeyer T, Kotzerke J, Kemnitz J, Grosse H, von zur Mühlen A (1989) Surgical aspects of familial pheochromocytoma. Horm Metab Res 21: 34–38
Dralle H, Schröder S, Gratz KF, Grote R, Padberg B, Hesch RD (1990) Sporadic unilateral adrenomedullary hyperplasia with hypertension cured by adrenalectomy. World J Surg 14: 308–316
Dralle H, Damm I, Scheumann GFW, Kotzerke J, Kupsch E (1992) Compartment-oriented microdissection of regional lymph nodes in medullary thyroid carcinoma. World J Surg
Dralle H, Damm I, Scheumann GFW, Kotzerke J, Kupsch E (1992) Frequency and significance of mediastinal lymph node metastases in medullary thyroid carcinoma — results of a compartment-oriented microdissection method. Henry Ford Hosp Med J
Dralle H, Scheumann GFW, Hundeshagen H, Massmann J, Pidelmayr R (1992) Die transsternale zervikomediastinale Primârtumorresektion and Lymphadenektomie beim Schilddrüsenkarzinom. Langenbecks Arch Chir 377: 34–44
Duh QY, Sancho JJ, Greenspan FS, Hunt TK, Galante M, de Lorimier AA, Conte FA, Clark OH (1989) Medullary thyroid carcinoma. The need for early diagnosis and total thyroidectomy. Arch Surg 124: 1206–1210
Farndon JR, Leight GS, Dilley WG, Baylin SB, Smallridge RC, Harrison TS, Wells SA (1986) Familial medullary thyroid carcinoma without associated endocrinopathies: a distinct clinical entity. Br J Surg 73: 278–281
Gagel RF, Tashjian AH, Cummings T, Pathanasopoulos N, Kaplan MM, DeLellis RA, Wolfe HJ, Reichlin S (1988) The clinical outcome of prospective screening for multiple endocrine neoplasia type 2a. N Engl J Med 318: 478–484
Gagel RF, Jackson CE, Ponder BAJ, Raue F, Simpson NE, Ziegler R (1989) Multiple endocrine neoplasia type 2 syndromes: nomenclature recommendation from the Workshop Organizing Committee. Henry Ford Hosp Med J 37: 99
Grosse H, Schröder D, Schober O, Hansen B, Dralle H (1990) Die Bedeutung einer hochdosierten alpha-Rezeptorenblockade für Blutvolumen und Hämodynamik beim Phaeochromocytom. Anasthesist 39: 313–318
Hamberger B, Telenius-Berg M, Cedermark B, Grondai S, Hansson BG, Werner S (1987) Subtotal adrenalectomy in multiple endocrine neoplasia type 2. Henry Ford Hosp Med J 35: 127–128
Heath H III, Sizemore GW, Carney JA (1976) Preoperative diagnosis of occult parathyroid hyperplasia by calcitonin infusion in patients with multiple endocrine neoplasia, type 2a. J Clin Endocrinol Metab 43: 428–435
Hermanek P, Sobin LH (eds) (1987) TNM classification of malignant tumors 4th edn. Springer, Berlin Heidelberg New York
Jackson CE, Talpos GB, Kambouris A, Yott JB, Tashjian AH, Block MA (1983) The clinical course after definitive operation for medullary thyroid carcinoma. Surgery 94: 995–1001
Jackson CE, Talpos GB, Block MA, Norum RA, Lloyd RV, Tashjian AH (1984) Clinical value of tumor doubling estimations in multiple endocrine neoplasia type II. Surgery 96: 981–987
Jansson S, Hansson G, Salander H, Stenström G, Tisell LE (1984) Prevalence of C-Cell hyperplasia and medullary thyroid carcinoma in a consecutive series of pheochromocytoma patients. World J Surg 8: 493–500
Kakudo K, Carney JA, Sizemore GW (1985) Medullary carcinoma of thyroid. Biologic behavior of the sporadic and familial neoplasm. Cancer 55: 2818–2821
Khairi MR, Dexter RN, Burzynski NT, Johnston CC (1975) Mucosal neuroma, pheochromocytoma and medullary thyroid carcinoma: multiple endocrine neoplasia type 3. Medicine (Baltimore) 54: 89–112
Knudson AG (1971) Mutation and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci USA 68: 820–823
Lips KJM, van der Sluys Veer J, Struyvenberg A, Alleman M, Leo JR, Wittebol P, Minder WH, Kooiker CJ, Geerding RA, van Waes PFGM, Hackeng WHL (1981) Bilateral occurrence of pheochromocytoma on patients with the multiple endocrine neoplasia syndrome type 2A ( Sipple’s syndrome ). Am J Med 70: 1051–1060
Marks AD, Channick BJ (1974) Extraadrenal pheochromocytoma and medullary thyroid carcinoma with pheochromocytoma. Arch Intern Med 134: 1106–1109
Melicow MM (1977) One hundred cases of pheochromocytoma (107 tumors) at the Columbia-Presbyterian Medical Center, 1926–1976. A clinicopathological analysis. Cancer 40: 1987–2004
Norton JA, Froome LC, Farrell RE, Wells SA (1979) Multiple endocrine neoplasia type IIb. The most aggressive form of medullary thyroid carcinoma. Surg Clin North Am 59: 109–118
Raue F, Späth-Röger M, Winter J, Benker G, Buhr P, Dorn R, Dralle H, Frilling A, Herrmann J, Hörnig I, Meybier H, Klempa I, Kotzerke J, Pfannenstiel P, Reinwein D, Ritter M, Röher H-D, Schober O, Schröder S, Seif F, Trede M, Vogt H, Wahl R, Ziegler R (1990) Register für das medulläre Schilddrüsenkarzinom in der Bundesrepublik Deutschland. Med Klin 3: 113–116
Russell CF, van Heerden JA, Sizemore W, Edis Ai, Taylor WF, ReMine WH, Carney JA (1983) The surgical management of medullary thyroid carcinoma. Ann Surg 197: 42–48
Russell WO, Ibanez ML, Clark RL, White EC (1963) Thyroid carcinoma. Cancer 16: 1425–1460
Saad MF, Ordonez NG, Rashid RK, Giudo JJ, Hill CST, Hickey RC, Samaan NA (1984) Medullary carcinoma of the thyroid. A study of the clinical features and prognostic factors in 161 patients. Medicine (Baltimore) 63: 319–342
Samaan NA, Yang KPP, Schultz P, Hickey RC (1989) Diagnosis, management, and pathogenetic studies in medullary thyroid carcinoma syndrome. Henry Ford Hosp Med J 37: 132–137
Schröder S, Böcker W, Baisch H, Bürk CG, Arps H, Meiners I, Kastendieck H, Heitz PU, Klöppel G (1988) Prognostic factors in medullary thyroid carcinomas. Survival in relation to age, sex, stage, histology, immunocytochemistry, and DNA content. Cancer 61: 806–816
Sobol H, Narod SA, Schuffenecker I, Amos C, Ezekowitz RAB, Lenoir GM (1989) Hereditary medullary thyroid carcinoma: genetic analysis of three related syndromes. Henry Ford Hosp Med J 37: 109–111
Steiner AL, Goodman AD, Powers SR (1968) Study of a kindred with pheochromocytoma, medullary thyroid carcinoma, hyperparathyroidism and cushing’s disease: multiple endocrine neoplasia, type 2. Medicine (Baltimore) 47: 371–409
Telander RL, Zimmermann D, Sizemore GW (1989) Medullary carcinoma in children. Results of early detection and surgery. Arch Surg 124: 841–843
Telenius-Berg M, Ponder MA, Berg B, Ponder BAJ, Werner S (1989) Quality of life after bilateral adrenalectomy in MEN 2. Henry Ford Hosp Med J 37: 160–163
Thompson NW (1984) Commentary to Jackson CE, Talpos GB, Block MA, Norum RA, Lloyd RV, Tashjian AH: Clinical value of tumor doubling estimations in multiple endocrine neoplasia type II. Surgery 96: 981–987
Tibblin S (1984) Commentary to van Heerden JA, Sizemore GW, Carney JA, Grant CS, ReMine WH, Sheps SG: Surgical management of the adrenal glands in the multiple endocrine neoplasia type II syndrome. World J Surg 8: 612–621
Tibblin S, Dymling JF, Ingemansson S, Telenius-Berg M (1983) Unilateral versus bilateral adrenalectomy in multiple endocrine neoplasia IIA. World J Surg 7: 201–208
Tisell LE, Hansson G, Jansson S, Salander H (1986) Reoperation in the treatment of asymptomatic metastasizing medullary thyroid carcinoma. Surgery 99: 60–66
Van Heerden JA, Sheps SG, Hamberger B, Sheedy PF II, Poston JG, ReMine WH (1982) Pheochromocytoma: current status and changing trends. Surgery 91: 367–373
Van Heerden JA, Sizemore GW, Carney JA, Grant CS, ReMine WH, Sheps SG (1984) Surgical management of the adrenal glands in the multiple endocrine neoplasia type II syndrome. World J Sur 8: 612–621
Van Heerden JA, Sizemore GW, Carney JA, Brennan MD, Sheps SG (1985) Bilateral subtotal adrenal resection for bilateral pheochromocytomas in multiple endocrine neoplasia, type 2a: a case report. Surgery 98: 363–366
Van Heerden JA, Grant CS, Gharib H, Hay ID, Ilstrup DM (1990) Long-term course of patients with persistent hypercalcitonemia after apparent curative primary surgery for medullary thyroid carcinoma. Ann Surg 212: 395–401
Wells SA, Dilley WG, Farndon JA, Leight GS, Baylin SB (1985) Early diagnosis and treatment of medullary thyroid carcinoma. Arch Intern Med 145: 1248–1252
Westfried M, Mandel D, Alderete MN, Groopman J, Minkowitz ST (1978) Sipple’s syndrome with a malignant pheochromocytoma presenting as a pericardial effusion. Cardiology 63: 305–311
Woolner LB, Beahrs OH, Black BM, McConahey WM, Keating FR (1968) Thyroid carcinoma: general considerations and follow-up data on 1181 cases. In: Young S, Inman DR (eds) Thyroid neoplasia. Academic, New York, pp 51–79
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1992 Springer-Verlag Berlin · Heidelberg
About this chapter
Cite this chapter
Dralle, H., Scheumann, G.F.W., Kotzerke, J., Brabant, E.G. (1992). Surgical Management of MEN 2. In: Raue, F. (eds) Medullary Thyroid Carcinoma. Recent Results in Cancer Research, vol 125. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-84749-3_9
Download citation
DOI: https://doi.org/10.1007/978-3-642-84749-3_9
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-84751-6
Online ISBN: 978-3-642-84749-3
eBook Packages: Springer Book Archive