Abstract
MTC should be screened in all children over 4y-old born from affected cases, since MTC may present as an inherited tumor. In our experience, a combined test injecting pentagastrin (Pg) 0.5 UI/Kg and calcium (Ca++) 2mg/Kg, with serum calcitonin (CT) measurements (0.2.5 and 10 min), has been proved to be a reliable screening test for occult MTC in children. In this study 12 prepubertal and pubertal individuals (M:4, F:8) were tested, they belonged to two sibs-hips with an affected pattern. Five of these cases showed CT hyperresponsiveness (>350 pg/ml) during Pg+Ca++ tests, all were females. At least two abnormal tests were detected in each case in order to establish the biochemical diagnosis of MTC and/or āCā cell hyperplasia, that preceeds the familial form of MTC. Three of the affected cases had no palpable thyroid nodules whereas it was palpable in two. Total thyroidectomy associated with preventive lymphnode resection was performed in all affected cases. Pathologycal findings confirmed the presence of MTC in all. After surgery, basal CT levels dropped to normal values and the exagerated response to Pg+Ca++ disappeared in the cases without palpable nodules. In those cases with palpable thyroid nodules, basal CT remained high despite a significant reduction after surgery, augmented CT responsiveness was also present in these latter cases. It is concluded that 1) In prepubertal and pubertal cases early detection of MTC usually leads to definitive cure after surgery. 2) cases with palpable thyroid nodules(late detection)due to MTC frequently do not achieve cure, as revealed by persistently high serum levels of the tumor marker CT.
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Abelin, N., Ezabella, M., Dahia, P. et al. 22 EARLY DETECTION OF MEDULLARY THYROID CARCINOMA (MTC) AND PREVENTIVE TOTAL THYROIDECTOMYI IN PREPUBERTAL AND PUBERTAL CASES. Pediatr Res 36, 681 (1994). https://doi.org/10.1203/00006450-199411000-00080
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DOI: https://doi.org/10.1203/00006450-199411000-00080