The surgical management of medullary thyroid carcinoma.

Medullary carcinoma of the thyroid may occur in three patient groups: multiple endocrine neoplasia, type 2b (MEN2b), MEN2a, and sporadic. The prognosis is best in MEN2a and worst in MEN2b. Multicentric disease occurs in approximately 90% of patients in the MEN groups and in 20% of the patients in the sporadic group. The minimal surgical procedure advocated is total thyroidectomy with dissection of the central compartment nodes. When neck dissection is performed, there appears to be no advantage in resecting the internal jugular vein or the sternomastoid muscle. Primary relatives of all patients with medullary carcinoma should be screened by measurement of plasma immunoreactive calcitonin to identify C-cell disease in a generally unsuspecting group/reservoir and because it results in earlier diagnosis, which leads to a less extensive surgical procedure and a higher percentage of patients with a disease-free state.