German medullary thyroid carcinoma/multiple endocrine neoplasia registry. German MTC/MEN Study Group. Medullary Thyroid Carcinoma/Multiple Endocrine Neoplasia Type 2.
BACKGROUND
A national registry for medullary thyroid carcinoma (MTC) and multiple endocrine neoplasia type 2 (MEN2) was set up to evaluate epidemiological, clinical and prognostic factors of the sporadic and hereditary forms of MTC.


METHODS
Coded data from 1217 patients with MTC from 57 participating centers in Germany were registered and statistically analyzed. The series included 515 (42%) males (mean age 43.1+/-16.1 years) and 702 (58%) females (mean age 44.4+/-17.6 years), with a mean follow-up of 5.2 years; 865 (71%) exhibited the sporadic form and 352 (29%) the familial form (244 MEN2a, 32 MEN2b, 76 FMTC). The mean age at diagnosis was 49 years for sporadic and 30 years for the familial form.


RESULTS
Of the patients, 17% presented at stage I, according to the UICC, and 30%, 45%, and 8% presented at stages II, III, and IV, respectively. There were 12% of patients who died of the disease. The overall adjusted survival rate was 87% at 5 years, and 76% at 10 years. In a univariate analysis, the stage of disease at diagnosis, age, gender, and form were relevant prognostic factors. In a multivariate proportional-hazards analysis, the difference between patients with sporadic and familial disease disappeared.


CONCLUSIONS
As the tumor stage at presentation is the major prognostic factor, early diagnosis and surgical intervention before cervical lymph node metastases appear is necessary to improve survival especially in sporadic cases.
Raue
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Endokrinologische Gemeinschaftspraxis, Heidelberg, Germany.
D000328:Adult; D017677:Age Distribution; D000368:Aged; D000704:Analysis of Variance; D018276:Carcinoma, Medullary / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D016016:Proportional Hazards Models; D012042:Registries; D012307:Risk Factors; D017678:Sex Distribution; D018709:Statistics, Nonparametric; D015996:Survival Rate; D013964:Thyroid Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery