Perioperative management of medullary thyroid carcinoma after bilateral adrenal pheochromocytomas resection / 中华普通外科杂志

ABSTRACT

Objective To evaluate perioperative management of MEN 2A patients with medullary thyroid carcinoma after bilateral pheochromocytomas removal.Methods Seven cases of MEN 2A from three families were diagnosed as medullary thyroid carcinoma and adrenal pheochromocytoma by biochemical tests and imaging examinations.All had histories of operations due to medullary thyroid carcinoma and contralateral adrenal pheochromocytoma.Direct sequencing of entire exons was performed in all patients.Firstly they underwent laparoscopic surgery for pheochromocytoma, then after stetoids replacement they underwent total thyroidectomy and bilateral cervical lymph nodes dissection.Results One family had the combined C634Y/D707E mutations of RET mutation.The other two families had C634Y mutation and C634R mutation separately.All patients had histories of adrenal insufficiency because of bilateral laparoscopic adrenal pheochromocytomas removal.They suffered from medullary thyroid carcinoma at the same time and needed to do total thyroidectomy and bilateral cervical lymph node dissection.Under perioperative steroids treatment and closely monitoring, they successfully went through the surgery.No adrenal crisis or related complications happened.Conclusions MEN 2A patients in this study had RET mutations and D707E mutation is a newly discovered mutation.Patients of adrenal insufficiency face the risk of adrenal crisis after a major operation.Correct perioperative monitoring and management can avoid complications.