Summary
A retrospective study of 741 patients with medullary thyroid carcinoma diagnosed between 1967 and 1991 was carried out by members of the German Medullary Thyroid Carcinoma Study Group to evaluate prognostic factors. A total of 559 patients (75%) were considered to have sporadic disease, and 182 (25%) had the familial type. The sex ratio (male to female) was 1:1.4 in sporadic disease patients, and the mean age at diagnosis was 45.9 years (range 5-81 years). For familial disease patients the sex ratio was 1:1.1, and the mean age at diagnosis was 33.4 (range 5–77 years). The follow-up time for 630 patients ranged from 1 month to 20.8 years (mean 13.0 years). The overall adjusted survival rate was 86.7% at 5 years and 64.2% at 10 years. In a univariate analysis the stage of disease at diagnosis, age, sex, and type of disease (sporadic, familial) were relevant prognostic factors, with a better prognosis for young female patients with familial disease and diagnosed at an early stage. In a multivariate proportional hazards analysis, the difference in the survival rate of patients with familial disease versus those with the sporadic form disappeared, while prognostic information provided by age and sex was still significant. The poorer prognosis of patients with sporadic medullary thyroid carcinoma may be related to the patients' older age at detection and more advanced tumor stage at diagnosis. There seems to be no difference in biological behavior between tumors of the sporadic and those of the familial type.
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Abbreviations
- MTC:
-
medullary thyroid carcinoma
- MEN:
-
multiple endocrine neoplasia
- CT:
-
calcitonin
References
Bergholm U, Adami HO, Bergström R, Johannsson H, Lundell G, Telenius-Berg M, Ackerström G (1989) Clinical characteristics in sporadic and familial medulläre thyroid carcinoma: a nationwide study of 249 patients in Sweden from 1959 through 1981. Cancer 63:1196–1204
Bergholm U, Adami HO, Bergström R, Bäckdahl M, Ackerström G (1990) Long-term survival in sporadic and familial medulläre thyroid carcinoma with special reference to clinical characteristic as prognostic factors. Acta Chir Scand 156:37–46
Calmettes C, Chaventre A, Feingold N, Franc B, Guliana JM(1989) Screening for medulläre thyroid cancer in France: a national effort. Henry Ford Hosp Med J 37:120–121
Chong GC, Beahrs OH, Sizemore GW, Woolner LH (1975) Medullary carcinoma of the thyroid gland. Cancer 35:695–704
Farndon JR, Leight GS, Dilley WG, Baylin SB, Smallridge RC, Harrison TS, Wells SA (1986) Familial medulläre thyroid carcinoma without associated endocrinopathies: a distinct clinical entity. Br J Surg 73:278–281
Fletcher JR (1970) Medullary (solid) carcinoma of the thyroid gland. Arch Surg 100:257–262
Frank K, Raue F, Gottswinter J, Heinrich H, Meybier H, Ziegler R (1984) Importance of early diagnosis and follow up in multiple endocrine neoplasia (MEN IIb). Eur J Pediatr 143:112–116
Gordon PR, Huvas AG, Strong EW (1973) Medullary carcinoma of the thyroid gland. Cancer 31:915–924
Grauer A, Raue F, GagelRF (1990) Changing concepts in the management of hereditary and sporadic medulläre thyroid carcinoma. Endocrinol Metab Clin North Am 19:613–635
Hazard JB, Hawk WA, Crile G (1959) Medullary (solid) carcinoma of the thyroid — a clinicopathologic entity. J Clin Endocrinol Metab 19:152–161
Hill CS, Ibanez ML, Samaan NA, Ahearn MJ, Carlk RL (1973) Medullary (solid) carcinoma of the thyroid gland. An analysis of the M.D. Anderson Hospital experience with patients with the tumor, its special feature, and its histogenesis. Medicine 52:141–171
Jackson CE, Talpos GB, Kambouris A, You JB, Tashjian AH, Block MA (1983) The clinical course after definitive operation for medulläre thyroid carcinoma. Surgery 94:995–999
Kakuda K, Carney A, Sizemore GW (1985) Medullary thyroid carcinoma of the thyroid: biological behavior of the sporadic and familial neoplasm. Cancer 55:2818–2821
Normann T, Gautvik KM, Johannessen JV, Brennhovd IO (1976) Medullary carcinoma of the thyroid in Norway. Acta Endocrinol 83:71–85
Ponder BAJ, Finer N, Coffey R, Harmer CL, Maisey M, Ormerod MG, Pembrey ME, Ponder MA, Rossick P, Shalet S (1988) Family screening in medulläre thyroid carcinoma patients presenting without a family history. Oncol J Med 67:299–308
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 HD, Schober O, Schröder S, Seif F, Trede M, Vogt H, Wahl R, Ziegler R (1990) Register für das medulläre Schilddrüsencarcinom in der Bundesrepublik Deutschland. Med Klin 85:113–117
Rougier P, Parmentier C, Laplanche A, Lefevre M, Travagli JP, Caillou B, Schlumberger M, Lacour J, Tubiana M (1983) Medullary thyroid carcinoma: prognostic factors and treatment. Int J Radiat Oncol Biol Phys 9:161–169
Rossi RL, Cady B, Meissner WA, Wool MS, Sedgewick CE, Werber J (1980) Nonfamilial medulläre thyroid carcinoma. Am J Surg 139:554–559
Saad MF, Guido JJ, Samaan NA (1983) Radioactive iodine in the treatment of medulläre carcinoma of the thyroid. J Clin Endocrinol Metab 57:124–128
Saad MF, Ordonez NG, Rashid RK, Guido JJ, Hill CS, Hickey RC, Samaan NA (1984) Medullary carcinoma of the thyroid: a study of the clinical feature and prognostic factors in 161 patients. Medicine 63:319–342
Samaan GA, Schultz PN, Hickey RC (1988) Medullary thyroid carcinoma: Prognosis of familial versus sporadic disease and the role of radiotherapy. J Clin Endocrinol Metab 67:801–805
Schröder S, Böcker W, Baisch H, Bürk CG, Arps H, Meiners I, Kastendieck H, Heitz PU, Klöppel G (1988) Prognostic factors in medulläre thyroid carcinomas: survival in relation to age, sex, stage, history, immunocytochemistry, and DNA content. Cancer 61:806–816
Simpson WJ, Palmer JA, Rosen IB, Mustard RA (1982) Management of medulläre carcinoma of the thyroid. Am J Surg 144:420–422
Sizemore GW (1987) Medullary carcinoma of the thyroid gland. Semin Oncol 14:306–314
Spiessl B, Hermanek P, Scheibe O, Wagner G (1988) UICC: TNM-Atlas, Illustrierter Leitfaden zur TNM/pTNM-Klassifikation maligner Tumoren, 3rd edn. Springer, Berlin Heidelberg New York
Vasen HFA, Nieuwenhuijzen AC, Moers AMJ, Lips CJM, Benkers EKM, Wiersinga WM, Geedink RA (1987) MEN2-A syndrome: the value of screening and central registration; a study of six kindreds in the Netherlands. Henry Ford Hosp Med J 35:101–103
Woolner LB, Beahrs OH, Black BM, McConahey WM, Keating RF (1961) Classification and prognosis of thyroid carcinoma. Am J Surg 102:354–387
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Raue, F., Kotzerke, J., Reinwein, D. et al. Prognostic factors in medullary thyroid carcinoma: evaluation of 741 patients from the German Medullary Thyroid Carcinoma Register. Clin Investig 71, 7–12 (1993). https://doi.org/10.1007/BF00210956
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DOI: https://doi.org/10.1007/BF00210956