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Medline ® Abstract for Reference 8

of 'Approach to therapy in multiple endocrine neoplasia type 2'

Long-term clinical and biochemical follow-up in medullary thyroid carcinoma: a single institution's experience over 20 years.
Cupisti K, Wolf A, Raffel A, Schott M, Miersch D, Yang Q, Eisenberger CF, Röher HD, Knoefel WT
Ann Surg. 2007;246(5):815.
OBJECTIVE: Many patients with medullary thyroid carcinomas (MTC) have reoperative surgery in different hospitals, which makes their follow-up difficult. To comprehend these complex courses and to find relevant prognostic factors we report a 20-year single center experience of 289 patients with MTC or precursor C-cell-hyperplasias.
PATIENTS AND METHODS: Between April 1986 and May 2006, 289 consecutive patients with MTC or MEN2 gene carriers were treated at the Department of Surgery at the University Hospital Düsseldorf. Tumor stages were documented according to the classification of the International Union against Cancer 5th edition, 1997 (Schott. Endocr Relat Cancer. 2006;13:779-795). A system to easily comprehend operative procedures is suggested.
RESULTS: There were 159 female and 130 male patients (f/m ratio 1.22). Mean age at time of diagnosis was 32 years (4-77) in the familial cases and 53 years (23-84) years in the sporadic cases. Sixty-six patients (23%) had multifocal disease. Twelve MEN2-patients had only C-cell-hyperplasia (pT0). Tumor stage was pT1 in 86 patients, pT2 in 106 patients, pT3 in 25 patients, pT4 in 52 patients and unclear in 8 patients. In the 289 patients 648 operations were performed. One hundred seventy patients had more than 1 operation (59%). Ninety-nine patients (34%) are calcitonin-negative and 91 patients (31%) live with elevated calcitonin. Median follow-up time of the surviving 211 patients was 8.9 years (range, 0.3-30.7 years). The 5- and 10-year survival of all tumor patients was 86% and 68%, respectively.
CONCLUSION: The chance to achieve biochemical cure in MTC is clearly dependent on the primary tumor size. The chance for long-term biochemical cure in a pT4-tumor is almost nil even after multiple and extended reoperations, whereas a pT1 tumor can be cured in up to 67% of the patients. Long-term survival, however, can be achieved even in pT4 tumor patients in almost 50%.
Department of General, Visceral, and Pediatric Surgery, University Hospital, Heinrich-Heine-University of Düsseldorf, Germany. cupisti@uni-duesseldorf.de