Diagnosis and therapy of sporadic and familial medullary thyroid carcinoma

Medullary thyroid carcinoma (MTC) is a rare thyroid malignancy. About 75% a re sporadic (sMTC) while the remaining 25% are hereditary (hMTC). The treat ment of choice for both sMTC and hMTC is surgery. An adequate initial opera tion provides the best chance of cure. Hence, the diagnosis of MTC should b e made preoperatively. In sMTC, ultrasound, ultrasound-guided fine-needle a spiration cytology and measurement of calcitonin levels (basal and after in jection of calcitonin-stimulating reagents, e.g., pentagastrin) are sensiti ve diagnostic tools. In hMTC, identification of a germline mutation in the proto-oncogene RET is sufficient for making the diagnosis. Total thyroidect omy is recommended in all patients, sporadic and hereditary. In addition, l ymphadenectomy of the cervicocentral and both cervicolateral compartments s hould be performed. The only indication to perform a less extensive operati on may be given in young patients with hMTC. Sufficient treatment of MTC be yond local disease is still nonexistent. Future research should concentrate on this issue.