Diagnosis and treatment of multiple endocrine neoplasia type 2A

Abstract

Objective To review clinical characteristics and treatment of multiple endocrine neoplasia type 2A (MEN-2A). Methods The clinical data of 13 patients with MEN-2A admitted to our hospital between 1988 and 2011 were retrospectively reviewed. All 13 cases were diagnosed as pheochromocytoma with medullary thyroid carcinoma, presenting no hyperparathyroidism, including 8 cases who had medullary thyroid carcinoma before pheochromocytoma and 5 cases who had medullary thyroid carcinoma and pheochromocytoma simultaneously. All 13 cases underwent resection for pheochromocytoma; 9 cases had bilateral adrenal resection, including 4 cases undergoing laparoscopic resection for pheochromocytoma. Thyroidectomy with bilateral dissection of regional lymph nodes was performed in 10 patients, and nodule enucleation was performed in 3 remaining patients. Results Adrenal pathology reported pheochromocytoma in all cases, including 3 malignant cases. Thyroid pathology reported medullary thyroid carcinoma in all cases. All 13 patients were followed-up visit, 10 cases survived and 3 died from distant metastasis of medullary thyroid carcinoma. Conclusions MEN-2A is a rare disease. Surgery is the only treatment for this disease; when patients have both pheochromocytoma and medullary thyroid carcinoma, to first remove the pheochromocytoma is preferable. Key words: Multiple endocrine neoplasia type 2a; Diagnosis; Treatment