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Endocrine-Related Cancer 16 (1) 243 -253     DOI: 10.1677/ERC-08-0059
Copyright © 2009 by the Society for Endocrinology
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Sporadic hypercalcitoninemia: clinical and therapeutic consequences

C Scheuba, K Kaserer1, A Moritz 2, R Drosten 3, H Vierhapper 4, C Bieglmayer 5, O A Haas 2 and B Niederle

Division of General Surgery, Section of Endocrine Surgery, Department of Surgery, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria1 Department of Clinical Pathology, Medical University of Vienna, Wahringer Gürtel 18-20, A-1090 Vienna, Austria2 Children's Cancer Research Institute, Kinderspitalgasse 6, A-1090 Vienna, Austria3 Radiology Department, University of Utah, Salt Lake City, Utah, UT 84132, USA4 Division of Endocrinology and Metabolism, Department of Internal Medicine III, Medical University of Vienna, Wahringer Gürtel 18-20, A-1090 Vienna, Austria5 Clinical Institute for Medical and Chemical Laboratory Diagnostics, Medical University of Vienna, Wahringer Gürtel 18-20, A-1090 Vienna, Austria

(Correspondence should be addressed to C Scheuba; Email: christian.scheuba{at}meduniwien.ac.at; B Niederle; Email: bruno.niederle{at}meduniwien.ac.at)

‘Calcitonin screening’ is not accepted as the standard of care in daily practice. The clinical and surgical consequences of ‘calcitonin screening’ in a series of patients with mildly elevated basal calcitonin and pentagastrin stimulated calcitonin levels are presented. 260 patients with elevated basal (>10 pg/ml) and stimulated calcitonin levels (>100 pg/ml) were enrolled in this prospective study. None of the patients was member of a known medullary thyroid carcinoma family. Thyroidectomy and bilateral central and lateral neck dissections were performed. Testing for the presence of germ-line mutations was performed in all patients. Histological and immunohistochemical findings were compared with basal and stimulated calcitonin levels. All patients were subsequently followed biochemically. C-cell hyperplasia (CCH) was found in 126 (49%) and medullary thyroid cancer was found in 134 (51%) patients. RET proto-oncogen mutations were documented in 22 (8%) patients (medullary thyroid cancer:18, CCH:4). In 56 (46%) of 122 patients, sporadic CCH was classified neoplastic (‘carcinoma in situ’). Of 97 (72%; 10 with hereditary medullary thyroid cancer) had pT1 (International Union against Cancer recommendations 2002) and 33 (25%) had pT2 or pT3 and 4 (3%) pT4 tumors. Of 39 (29.1%) had lymph node metastases. 106 (79.1%; 15 (38.5%) with lymph node metastases) patients were cured. Evaluation of basal and stimulated calcitonin levels enables the prediction of medullary thyroid cancer. All patients with basal calcitonin >64 pg/ml and stimulated calcitonin >560 pg/ml have medullary thyroid cancer. Medullary thyroid cancer was documented in 20% of patients with basal calcitonin >10 pg/ml but <64 pg/ml and stimulated calcitonin >100 pg/ml but <560 pg/ml.




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