Diagnosis and treatment of multiple endocrine neoplasia type 2A / 中华全科医师杂志

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.