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Alternative Surgical Strategies and Favorable Outcomes in Patients with Medullary Thyroid Carcinoma in Japan: Experience of a Single Institution

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Abstract

Background

Medullary thyroid carcinoma (MTC) accounts only for 1.4% of all thyroid malignancies in Japan. Since 1996, we have performed hemithyroidectomy, instead of total thyroidectomy, for sporadic nonhereditary MTC when the primary lesion is located in only one lobe. Regarding lymph node dissection, modified radical neck dissection (MND) at least ipsilateral to the tumor has been routinely performed, even if there is no clinically apparent metastasis. We investigated the clinical outcomes of MTC patients in our department.

Methods

A series of 118 patients with MTC who underwent initial surgery between 1975 and 2005 were enrolled in this study. The RET gene mutations were analyzed for all patients and 46 had germline RET gene mutations. Of those 46 patients, 26 were diagnosed as MEN 2A and 2 were diagnosed as MEN 2B. Postoperative follow-up periods averaged 141 months.

Results

Of 115 patients who did not have distant metastasis at surgery and who underwent locally curative surgery, 78 (67.8%) were biochemically cured. All patients without pathological lymph node metastasis were biochemically cured, and 44.8% of patients with node metastasis were also biochemically cured. The 10-year and 20-year disease-free survival rates were 89.0% and 82.5%, respectively. None of the patients who did not show lymph node metastasis and only 2 (2.6%) of 78 patients who were biochemically cured showed clinically apparent carcinoma recurrence. The 10-year and 20-year cause-specific survival rates were 96.6% and 91.7%, respectively. Lymph node metastasis, tumor size >4 cm, extrathyroid and extranodal tumor extensions significantly affected cause-specific survival of patients.

Conclusions

Clinical outcomes of MTC patients in our series were better than those in Western countries, a result that might have resulted in part because of our routine MND regardless of whether clinically apparent node metastasis was detected.

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References

  1. Ezaki H, Ebihara S, Fujimoto Y et al (1992) Analysis of thyroid carcinoma based on material registered in Japan during 1977–1986 with special reference to predominance of papillary type. Cancer 70:808–814

    Article  PubMed  CAS  Google Scholar 

  2. Moley JE, DeBenedetti MK (1999) Patterns of nodal metastases in palpable medullary thyroid carcinoma: recommendations for extent of node dissection. Ann Surg 229:880–887

    Article  PubMed  CAS  Google Scholar 

  3. Kebebew E, Ituarte PH, Siperstein AE et al (2000) Medullary thyroid carcinoma: clinical characteristics, treatment, prognostic factors, and a comparison of staging systems. Cancer 88:1139–1148

    Article  PubMed  CAS  Google Scholar 

  4. Gulben K, Berberoglu U, Boyabatl M (2006) Prognostic factors for sporadic medullary thyroid crcinoma. World J Surg 30:84–90

    Article  PubMed  Google Scholar 

  5. DeLellis RA, Rule AH, Spiler I et al (1978) Calcitonin and carcinoembryonic antigen as tumor markers in medullary thyroid carcinoma. Am J Clin Pathol 70:587–594

    PubMed  CAS  Google Scholar 

  6. Raue R, Kkotzerke J, Reinwein D et al (1993) Prognostic factors in medullary thyroid carcinoma: evaluation of 741 patients from the German medullary thyroid carcinoma register. Clin Invest 71:7–12

    Article  CAS  Google Scholar 

  7. Mulligan LM, Kwok JB, Healey C et al (1993) Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A. Nature 2:851–856

    Google Scholar 

  8. Donis-Keller H, Dou S, Chi D et al (1993) Mutations in the RET proto-oncogene are associated with MEN2A and FMTC. Hum Mol Genet 2:851–856

    Article  PubMed  CAS  Google Scholar 

  9. Carlson KM, Dou S, Chi D et al (1994) Single missense mutation in the tyrosine kinase catalytic domain of the RET protooncogene is associated with multiple endocrine neoplasia type 2B. Proc Natl Acad Sci USA 91:1579–1583

    Article  PubMed  CAS  Google Scholar 

  10. Carlson KM, Dou S, Chi D et al (1994) Single missense mutation in the tyrosine kinase catalytic domain of the RET protooncogene is associated with multiple endocrine neoplasia type 2B. Proc Natl Acad Sci USA 91:1579–1583

    Article  PubMed  CAS  Google Scholar 

  11. Hyer SL, Newbold K, Harmer C (2005) Familial medullary thyroid cancer: clinical aspects and prognosis. Eur J Surg Oncol 31:415–419

    Article  PubMed  CAS  Google Scholar 

  12. Cupisti K, Wolf A, Raffel A et al (2007) Long-term clinical and biochemical follow-up in medullary thyroid carcinoma: a single institution’s experience over 20 years. Ann Surg 246:815–821

    PubMed  Google Scholar 

  13. Lee NC, Norton JA (2000) Multiple endocrine neoplasia type 2B—genetic basis and clinical expression. Surg Oncol 9:111–118

    Article  PubMed  CAS  Google Scholar 

  14. Brauckhoff M, Gimm O, Weiss CL et al (2004) Multiple endocrine neoplasia 2B syndrome due to codon 918 mutation: clinical manifestation and course in early and late onset disease. World J Surg 28:1305–1311

    Article  PubMed  Google Scholar 

  15. Bergholm U, Bergstrom R, Ekbom A (1997) Long term follow-up of patients with medullary carcinoma of the thyroid. Cancer 79:132–138

    Article  PubMed  CAS  Google Scholar 

  16. Kameyama K, Takami H (2004) Medullary thyroid carcinoma: nationwide Japanese survey of 634 cases in 1996 and 271 cases in 2002. Endocrine J 51:453–456

    Article  CAS  Google Scholar 

  17. Saad MF, Ordonez NG, Rashid RK (1984) Medullary carcinoma of the thyroid: a study of the clinical features and prognostic factors in 161 patients. Medicine 63:319–342

    Article  PubMed  CAS  Google Scholar 

  18. Brierley J, Tsang R, Simpson WJ (1996) Medullary thyroid cancer: analysis of survival and prognostic factors and the role of radiation therapy in local control. Thyroid 6:305–310

    PubMed  CAS  Google Scholar 

  19. Dottorini ME, Assi A, Sironi M (1996) Multivariate analysis of patients with medullary carcinoma: prognostic significance and impact on treatment of clinical and pathologic variables. Cancer 77:1556–1565

    Article  PubMed  CAS  Google Scholar 

  20. Clark JR, Friedman TR, Odell MJ (2005) Prognostic variables and calcitonin in medullary thyroid cancer. Laryngoscope 115:1145–1150

    Article  Google Scholar 

  21. Machens A, Hofmann C, Hauptmann S et al (2007) Locoregional recurrence and death from medullary thyroid carcinoma in a contemporaneous series: 5-year results. Eur J Endocrinol 157:85–93

    Article  PubMed  CAS  Google Scholar 

  22. Schroder S, Bocker W, Baisch H et al (1988) Prognostic factors in medullary thyroid carcinomas. Survival in relation to age, sex, stage, histology, immunocytochemistry, and DNA content. Cancer 61:806–816

    Article  PubMed  CAS  Google Scholar 

  23. Gharib H, McConahey WM, Tiegs RD et al (1992) Medullary thyroid carcinoma: clinicopathologic features and long-term follow-up of 65 patients treated during 1946 through 1970. Mayo Clin Proc 67:934–840

    PubMed  CAS  Google Scholar 

  24. Miyauchi A, Matsuzuka F, Hirai K et al (2000) Unilateral surgery supported by germline RET oncogene mutation analysis in patients with sporadic medullary thyroid carcinoma. World J Surg 24:1367–1372

    Article  PubMed  CAS  Google Scholar 

  25. Miyauchi A, Matsuzuka F, Hirai K et al (2002) Prospective trial of unilateral surgery for nonhereditary medullary thyroid carcinoma in patients without germline RET mutations. World J Surg 26:1023–1028

    Article  PubMed  Google Scholar 

  26. Heshmati HM, Gharib H, van Heerden JA et al (1997) Advances and controversies in the diagnosis and management of medullary thyroid carcinoma. Am J Med 103:60

    Article  PubMed  CAS  Google Scholar 

  27. Scopsi I, Sampietro G, Boracchi P et al (1996) Multivariate analysis of prognostic factors in sporadic medullary carcinoma of the thyroid: a retrospective study of 109 consecutive patients. Cancer 78:2173

    Article  PubMed  CAS  Google Scholar 

  28. Gimm O, Ukkat J, Dralle H (1998) Determinative factors of biochemical cure after primary and reoperative surgery for sporadic medullary thyroid carcioma. World J Surg 22:562–568

    Article  PubMed  CAS  Google Scholar 

  29. Beressi N, Campos JM, Beressi JP et al (1998) Sporadic medullary microcarcinoma of the thyroid: a retrospective analysis of eighty cases. Thyroid 8:1039

    Article  PubMed  CAS  Google Scholar 

  30. Ito Y, Miyauchi A. Lateral lymph node dissection guided by preoperative and intraoperative findings in differentiated thyroid carcinoma. World J Surg 32:729–739

  31. Ismailov SI, Pulatova NR (2004) Postoperative calcitonin study in medullary thyroid carcinoma. Endocr Relat Cancer 11:357–363

    Article  PubMed  CAS  Google Scholar 

  32. de Groot J, Plukker J, Wolffenbuttel B et al (2006) Determinants of life expectancy in medullary thyroid cancer: age does not matter. Clin Endocrinol 65:729–736

    Article  Google Scholar 

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Correspondence to Akira Miyauchi.

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Ito, Y., Miyauchi, A., Yabuta, T. et al. Alternative Surgical Strategies and Favorable Outcomes in Patients with Medullary Thyroid Carcinoma in Japan: Experience of a Single Institution. World J Surg 33, 58–66 (2009). https://doi.org/10.1007/s00268-008-9795-2

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