Characteristics of a Family with the MEN-2A Syndrome

Authors

Harm R. Haak
Arie C. Nieuwenhuijzen Kruseman

Abstract

Results of screening and follow-up in a family with the MEN-2A syndrome are described. Fourteen (83%) subjects at risk were affected with medullary thyroid carcinoma (MTC). Asymptomatic pheochromocytoma and parathyroid hyperplasia were found in two instances each. Intrathyroidal MTC without metastases was found in the four youngest subjects and was associated with normal basal but abnormal pentagastrin-stimulated calcitonin (CT) levels. In the ten subjects with abnormal basal CT levels, metastatic spread to at least regional lymph nodes was found. In eight of these ten subjects, total thyroidectomy with excision of affected lymph nodes and additional radioiodine did not return CT levels to normal postoperatively. In the other two subjects, the CT levels returned to normal after thyroidectomy and an ablative dose of radioiodine, and remained normal during follow-up. This study confirms the view that family screening at a young age promotes the detection of MTC at a curable stage. Radioiodine as an adjunct to surgery may be of value in the prevention of tumor recurrence in patients with normal postoperative CT levels but residual thyroid tissue.

Recommended Citation

Haak, Harm R. and Nieuwenhuijzen Kruseman, Arie C. (1987) "Characteristics of a Family with the MEN-2A Syndrome," Henry Ford Hospital Medical Journal : Vol. 35 : No. 2 , 104-106.
Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol35/iss2/6