Dökmetaş, Hatice Sebila Cumhuriyet University - Faculty of Medicine - Department of Endocrinology and Metabolism, Turkey , Kılıçlı, Fatih Cumhuriyet University - School of Medicine - Department of Endocrinology and Metabolism, Turkey , Özben, Volkan Istanbul University - Cerrahpaşa School of Medicine - Department of General Surgery, Turkey , Tapan, Ümit Steward Carney Hospital - Department of Internal Medicine, USA

New advances in the diagnosis, medical management and follow-up of medullary thyroid cancer

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Medullary thyroid cancer (MTC), which behaves more aggressively than the other well-differentiated thyroid cancers of follicular cell origin, accounts for 10% of all thyroid cancer-related deaths. Most MTCs are sporadic however approximately 25% is hereditary as part of the multiple endocrine neoplasia syndrome type 2 (MEN 2a and 2b). Both sporadic and inherited forms of MTC have a propensity for lymphatic and distant metastasis. The primary treatment of MTC is total thyroidectomy and removal of all neoplastic tissues present in the neck. After surgical treatment, measurements of serum calcitonin and carcinoembryonic antigen are of paramount importance in the postoperative follow-up since these markers reflect the presence of persistent or recurrent disease. Systemic chemotherapy with dacarbazine, 5-fluorouracil and doxorubicin, either alone or in combination, has shown very limited efficacy, producing only partial responses in the range of 10-20% and of short duration. Although current treatment options for patients with metastatic and refractory MTC are limited, recent advances in molecular oncology have fostered the invention of novel small molecules which target specific pathways which are thought to be essential in the MTC carcinogenesis. Several kinase inhibitors are currently under evaluation and preliminary results are promising.

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Medullary thyroid cancer , thyrosine kinase inhibitors

Cumhuriyet Medical Journal

515