CURRENT DIAGNOSIS AND MANAGEMENT OF MEDULLARY-THYROID CARCINOMA

Background: Medullary thyroid carcinoma (MTC) originates in the thyroi d C cells, accounting for 5% to 10% of all thyroid malignancies. Appro ximately 75% of cases are sporadic. Significant advances have been mad e in the molecular biology of MTC, but some aspects of diagnosis and m anagement still remain controversial. Design: We reviewed relevant art icles published in major English-language medical journals. We used th e MEDLINE database, selected bibliographies, and articles available in our personal files. Results: Mutations of the RET proto-oncogene have been identified in the germline DNA of patients with familial MTC syn dromes. Genetic testing can identify patients affected by multiple end ocrine neoplasia types IIA and IIB and familial MTC, allowing early di agnosis and possible cure. Surgical treatment is total thyroidectomy. Plasma calcitonin measurements are excellent markers for postoperative follow-up, adjunctive therapy includes radiotherapy and chemotherapy. The overall prognosis is worse than papillary thyroid carcinoma. Conc lusions: Recent advances in genetic testing allow early diagnosis and treatment of familial MTC syndromes. Despite some advances in treatmen t, optimal management remains controversial.