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Medullary Thyroid Carcinoma: The Need for Early Diagnosis and Total Thyroidectomy

Medullary Thyroid Carcinoma: The Need for Early Diagnosis and Total Thyroidectomy Abstract • Forty patients with medullary thyroid carcinoma and 3 patients with C-cell hyperplasia were studied. Seventeen (40%) cases were sporadic and 26 (60%) were hereditary. Eight patients had type Ila multiple endocrine neoplasia, 7 patients had type IIb multiple endocrine neoplasia, and 11 patients had familial nonmultiple endocrine neoplasia medullary thyroid carcinoma. Mean follow-up was 6.3 years, with actuarial survival of 88% and 78% at 5 and 10 years (22 and 13 patients), respectively. Seven patients died 1.5 to 10 years after the initial operation; all had advanced disease at presentation (6 with distant, 1 with lymph node metastasis). No deaths occurred in patients with familial nonmultiple endocrine neoplasia medullary thyroid carcinoma, C-cell hyperplasia, or medullary thyroid carcinoma limited to the thyroid gland. Nineteen (68%) of 28 patients diagnosed without screening had regional or distant metastases, whereas only 6 (40%) of 15 patients diagnosed by screening had metastases. Twenty-six patients treated initially with total thyroidectomy and central neck clearance required an average of one reoperation, whereas those with lesser initial procedures required an average of two reoperations. We concluded that (1) familial nonmultiple endocrine neoplasia medullary thyroid carcinoma, early medullary thyroid carcinoma or C-cell hyperplasia, and asymptomatic patients have a good prognosis; (2) screening for medullary thyroid carcinoma by measuring serum calcitonin levels results in earlier diagnosis; and (3) total thyroidectomy and central neck clearance is the procedure of choice for medullary thyroid carcinoma. (Arch Surg. 1989;124:1206-1210) References 1. Fletcher JR. Medullary (solid) carcinoma of the thyroid gland . Arch Surg . 1970;100:257-262.Crossref 2. Clark OH. Medullary thyroid carcinoma . In: Endocrine Surgery of the Thyroid and Parathyroid Glands . St Louis, Mo: CV Mosby Co; 1985:91-103 3. Brunt LM, Wells SA. Advances in the diagnosis and treatment of medullary thyroid carcinoma . Surg Clin North Am . 1987;67:263-279. 4. Hazard JB, Hawk WA, Crile D. Medullary (solid) carcinoma of the thyroid: a clinicopathologic entity . J Clin Endocrinol Metab . 1959;19:152-161.Crossref 5. Farndon JR, Light GS, Dilley WG, et al. Familial medullary thyroid carcinoma without associated endocrinopathies: a distinct clinical entity . Br J Surg . 1986;73:278-281.Crossref 6. Tashjian AH Jr, Howland BG, Melvin KEW, Hill CS Jr. Immunoassay of human calcitonin: clinical measurement, relation to serum calcium and studies in patients with medullary carcinoma . N Engl J Med . 1970;283:890-895Crossref 7. Becker KL, Silva OL, Snider RH, Moore CF, Geelhoed GW. The surgical implications of hypercalcitoninemia . Surg Gynecol Obstet . 1982;154:897-908 8. Wells SA, Baylin SB, Gann DS, et al. Medullary thyroid carcinoma: relationship of method of diagnosis to pathologic staging . Ann Surg . 1978;188:377-383.Crossref 9. Gagel RF, Tashjian AH Jr, Cummings T, et al. The clinical outcome of prospective screening for multiple endocrine neoplasia type 2a: an 18-year experience . N Engl J Med . 1988;318:478-484.Crossref 10. Vasen HF, Nieuwenhuijzen-Kruseman AC, Berkel H, et al. Multiple endocrine neoplasia syndrome type 2: the value of screening and central registration: a study of 15 kindreds in the Netherlands . Am J Med . 1987;83:847-852.Crossref 11. Saad MF, Ordonez NG, Rashid RK, et al. Medullary carcinoma of the thyroid: a study of the clinical features and prognostic factors in 161 patients . Medicine . 1984;63:319-342.Crossref 12. Russell CF, Van Heerden JA, Sizemore GW, et al. The surgical management of medullary thyroid carcinoma . Ann Surg . 1983;197:42-48. 13. Simpson WJ, Palmer JA, Rosen IB, Mustard RA. Management of medullary carcinoma of the thyroid . Am J Surg . 1982;144:420-422.Crossref 14. Jackson CE, Talpos GB, Kambouris A, Yott JB, Tashjian AH Jr, Block MA. The clinical course after definitive operation for medullary thyroid carcinoma . Surgery . 1983;94:995-1001. 15. Tisell LE, Hansson G, Jansson S, Salander H. Reoperation in the treatment of asymptomatic metastasizing medullary thyroid carcinoma . Surgery . 1986;99:60-66. 16. Saad MF, Guido JJ, Samman NA. Radioactive iodine in the treatment of medullary carcinoma of the thyroid . J Clin Endocinol Metab . 1983;57:124-128.Crossref 17. Riccabona G, Ladurner D, Schmid K. When is medullary thyroid carcinoma'medullary thyroid carcinoma'? World J Surg . 1986;10:745-752.Crossref 18. Samaan NA, Schultz PN, Hickey RC. Medullary thyroid carcinoma: prognosis of familial versus sporadic disease and the role of radiotherapy . J Clin Endocrinol Metab . 1988;67:801-805.Crossref 19. Petursson SR. Metastatic medullary thyroid carcinoma: complete response to combination chemotherapy with dacarbazine and 5-fluorouracil . Cancer . 1988;62:1899-1903.Crossref 20. Ahlman H, Tisell LE. The use of a long-acting somatostatin analogue in the treatment of advanced endocrine malignancies with gastrointestinal symptoms . Scand J Gastroenterol . 1987;22:938-942.Crossref 21. Gorman B, Charboneau JW, James EM, et al. Medullary thyroid carcinoma: role of high-resolution US . Radiology . 1987;162:147-150.Crossref 22. Norton JA, Doppman JL, Brennan MF. Localization and resection ofclinically inapparent medullary carcinoma of the thyroid . Surgery . 1980;87:616-622. 23. Busnardo B, Girelli ME, Simioni N, Nacamulli D, Busetto E. Nonparallel patterns of calcitonin and carcinoembryonic antigen levels in the follow-up of medullary thyroid carcinoma . Cancer . 1984;53:278-285.Crossref 24. Simpson NE, Kidd KK, Goodfellow PJ, et al. Assignment of multipleendocrine neoplasia type 2A to chromosome 10 by linkage . Nature . 1987;328:528-530.Crossref 25. Boorman GA, Hollander CF. Animal model: medullary carcinoma of the thyroid in the rat . Am J Pathol . 1976;83:237-240. 26. Sponenberg DP, McEntee K. Pheochromocytomas and ultimobrachial (C-cell) neoplasm in the bull: evidence of autosomal dominant inheritance in the Guernsey breed . Vet Pathol . 1983;20:396-400.Crossref 27. Bäckdahl M, Tallroth E, Auer G, et al. Prognostic value of nuclear DNA content in medullary thyroid carcinoma . World J Surg . 1985;9:980-987.Crossref 28. Lippman SM, Mendelsohn G, Trump DL, Wells SA Jr, Baylin SB. The prognostic and biological significance of cellular heterogeneity in medullary thyroid carcinoma: a study of calcitonin, L-dopa, dicarboxylase, and histaminase . J Clin Endocrinol Metab . 1982;54:233-240.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

Medullary Thyroid Carcinoma: The Need for Early Diagnosis and Total Thyroidectomy

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References (31)

Publisher
American Medical Association
Copyright
Copyright © 1989 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1989.01410100112019
Publisher site
See Article on Publisher Site

Abstract

Abstract • Forty patients with medullary thyroid carcinoma and 3 patients with C-cell hyperplasia were studied. Seventeen (40%) cases were sporadic and 26 (60%) were hereditary. Eight patients had type Ila multiple endocrine neoplasia, 7 patients had type IIb multiple endocrine neoplasia, and 11 patients had familial nonmultiple endocrine neoplasia medullary thyroid carcinoma. Mean follow-up was 6.3 years, with actuarial survival of 88% and 78% at 5 and 10 years (22 and 13 patients), respectively. Seven patients died 1.5 to 10 years after the initial operation; all had advanced disease at presentation (6 with distant, 1 with lymph node metastasis). No deaths occurred in patients with familial nonmultiple endocrine neoplasia medullary thyroid carcinoma, C-cell hyperplasia, or medullary thyroid carcinoma limited to the thyroid gland. Nineteen (68%) of 28 patients diagnosed without screening had regional or distant metastases, whereas only 6 (40%) of 15 patients diagnosed by screening had metastases. Twenty-six patients treated initially with total thyroidectomy and central neck clearance required an average of one reoperation, whereas those with lesser initial procedures required an average of two reoperations. We concluded that (1) familial nonmultiple endocrine neoplasia medullary thyroid carcinoma, early medullary thyroid carcinoma or C-cell hyperplasia, and asymptomatic patients have a good prognosis; (2) screening for medullary thyroid carcinoma by measuring serum calcitonin levels results in earlier diagnosis; and (3) total thyroidectomy and central neck clearance is the procedure of choice for medullary thyroid carcinoma. (Arch Surg. 1989;124:1206-1210) References 1. Fletcher JR. Medullary (solid) carcinoma of the thyroid gland . Arch Surg . 1970;100:257-262.Crossref 2. Clark OH. Medullary thyroid carcinoma . In: Endocrine Surgery of the Thyroid and Parathyroid Glands . St Louis, Mo: CV Mosby Co; 1985:91-103 3. Brunt LM, Wells SA. Advances in the diagnosis and treatment of medullary thyroid carcinoma . Surg Clin North Am . 1987;67:263-279. 4. Hazard JB, Hawk WA, Crile D. Medullary (solid) carcinoma of the thyroid: a clinicopathologic entity . J Clin Endocrinol Metab . 1959;19:152-161.Crossref 5. Farndon JR, Light GS, Dilley WG, et al. Familial medullary thyroid carcinoma without associated endocrinopathies: a distinct clinical entity . Br J Surg . 1986;73:278-281.Crossref 6. Tashjian AH Jr, Howland BG, Melvin KEW, Hill CS Jr. Immunoassay of human calcitonin: clinical measurement, relation to serum calcium and studies in patients with medullary carcinoma . N Engl J Med . 1970;283:890-895Crossref 7. Becker KL, Silva OL, Snider RH, Moore CF, Geelhoed GW. The surgical implications of hypercalcitoninemia . Surg Gynecol Obstet . 1982;154:897-908 8. Wells SA, Baylin SB, Gann DS, et al. Medullary thyroid carcinoma: relationship of method of diagnosis to pathologic staging . Ann Surg . 1978;188:377-383.Crossref 9. Gagel RF, Tashjian AH Jr, Cummings T, et al. The clinical outcome of prospective screening for multiple endocrine neoplasia type 2a: an 18-year experience . N Engl J Med . 1988;318:478-484.Crossref 10. Vasen HF, Nieuwenhuijzen-Kruseman AC, Berkel H, et al. Multiple endocrine neoplasia syndrome type 2: the value of screening and central registration: a study of 15 kindreds in the Netherlands . Am J Med . 1987;83:847-852.Crossref 11. Saad MF, Ordonez NG, Rashid RK, et al. Medullary carcinoma of the thyroid: a study of the clinical features and prognostic factors in 161 patients . Medicine . 1984;63:319-342.Crossref 12. Russell CF, Van Heerden JA, Sizemore GW, et al. The surgical management of medullary thyroid carcinoma . Ann Surg . 1983;197:42-48. 13. Simpson WJ, Palmer JA, Rosen IB, Mustard RA. Management of medullary carcinoma of the thyroid . Am J Surg . 1982;144:420-422.Crossref 14. Jackson CE, Talpos GB, Kambouris A, Yott JB, Tashjian AH Jr, Block MA. The clinical course after definitive operation for medullary thyroid carcinoma . Surgery . 1983;94:995-1001. 15. Tisell LE, Hansson G, Jansson S, Salander H. Reoperation in the treatment of asymptomatic metastasizing medullary thyroid carcinoma . Surgery . 1986;99:60-66. 16. Saad MF, Guido JJ, Samman NA. Radioactive iodine in the treatment of medullary carcinoma of the thyroid . J Clin Endocinol Metab . 1983;57:124-128.Crossref 17. Riccabona G, Ladurner D, Schmid K. When is medullary thyroid carcinoma'medullary thyroid carcinoma'? World J Surg . 1986;10:745-752.Crossref 18. Samaan NA, Schultz PN, Hickey RC. Medullary thyroid carcinoma: prognosis of familial versus sporadic disease and the role of radiotherapy . J Clin Endocrinol Metab . 1988;67:801-805.Crossref 19. Petursson SR. Metastatic medullary thyroid carcinoma: complete response to combination chemotherapy with dacarbazine and 5-fluorouracil . Cancer . 1988;62:1899-1903.Crossref 20. Ahlman H, Tisell LE. The use of a long-acting somatostatin analogue in the treatment of advanced endocrine malignancies with gastrointestinal symptoms . Scand J Gastroenterol . 1987;22:938-942.Crossref 21. Gorman B, Charboneau JW, James EM, et al. Medullary thyroid carcinoma: role of high-resolution US . Radiology . 1987;162:147-150.Crossref 22. Norton JA, Doppman JL, Brennan MF. Localization and resection ofclinically inapparent medullary carcinoma of the thyroid . Surgery . 1980;87:616-622. 23. Busnardo B, Girelli ME, Simioni N, Nacamulli D, Busetto E. Nonparallel patterns of calcitonin and carcinoembryonic antigen levels in the follow-up of medullary thyroid carcinoma . Cancer . 1984;53:278-285.Crossref 24. Simpson NE, Kidd KK, Goodfellow PJ, et al. Assignment of multipleendocrine neoplasia type 2A to chromosome 10 by linkage . Nature . 1987;328:528-530.Crossref 25. Boorman GA, Hollander CF. Animal model: medullary carcinoma of the thyroid in the rat . Am J Pathol . 1976;83:237-240. 26. Sponenberg DP, McEntee K. Pheochromocytomas and ultimobrachial (C-cell) neoplasm in the bull: evidence of autosomal dominant inheritance in the Guernsey breed . Vet Pathol . 1983;20:396-400.Crossref 27. Bäckdahl M, Tallroth E, Auer G, et al. Prognostic value of nuclear DNA content in medullary thyroid carcinoma . World J Surg . 1985;9:980-987.Crossref 28. Lippman SM, Mendelsohn G, Trump DL, Wells SA Jr, Baylin SB. The prognostic and biological significance of cellular heterogeneity in medullary thyroid carcinoma: a study of calcitonin, L-dopa, dicarboxylase, and histaminase . J Clin Endocrinol Metab . 1982;54:233-240.Crossref

Journal

Archives of SurgeryAmerican Medical Association

Published: Oct 1, 1989

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