Anesthetic Management of Multiple Endocrine Neoplasia Type 2A (MEN2A) Syndrome With Contralateral Recurrent Pheochromocytoma: A Case Report
Source: PubMed Central Open Access, NCBI / U.S. National Library of Medicine
This is a classic and complete representation of the multiple endocrine neoplasia type 2A (MEN2A) syndrome, involving primary hyperparathyroidism, medullary thyroid carcinoma, and pheochromocytomas. This case is particularly noteworthy for the recurrent, contralateral presentation of the pheochromocytoma, which emerged years after the initial surgical management. Recurrent and contralateral pheochromocytomas present unique anesthetic, surgical, and postoperative problems due to excess catecholamines and severe, potentially life-threatening hemodynamic instability in the perioperative phase. We report an unusual case of a 29-year-old female with MEN2A who had a right adrenalectomy, subsequently total thyroidectomy and parathyroidectomy, and was under follow-up with an endocrinologist. During follow-up, she was found to have very high plasma normetanephrine levels (>760 pg/mL), and radiological imaging revealed an avid adrenal nodule in the left adrenal gland, which was consistent with the diagnosis of recurrent contralateral pheochromocytoma. She was managed preoperatively on doxazosin and metoprolol. During the anesthetic evaluation, she had orthostatic hypotension, but no other symptoms related to pheochromocytomas. Elective open left adrenalectomy was carried out under general anesthesia, aiming to blunt stress responses. Hemodynamics were monitored invasively and managed with vasodilator and vasopressor infusions. After the removal of the adrenal tumor, the noradrenaline i
Abstract
This is a classic and complete representation of the multiple endocrine neoplasia type 2A (MEN2A) syndrome, involving primary hyperparathyroidism, medullary thyroid carcinoma, and pheochromocytomas. This case is particularly noteworthy for the recurrent, contralateral presentation of the pheochromocytoma, which emerged years after the initial surgical management. Recurrent and contralateral pheochromocytomas present unique anesthetic, surgical, and postoperative problems due to excess catecholamines and severe, potentially life-threatening hemodynamic instability in the perioperative phase. We report an unusual case of a 29-year-old female with MEN2A who had a right adrenalectomy, subsequently total thyroidectomy and parathyroidectomy, and was under follow-up with an endocrinologist. During follow-up, she was found to have very high plasma normetanephrine levels (>760 pg/mL), and radiological imaging revealed an avid adrenal nodule in the left adrenal gland, which was consistent with the diagnosis of recurrent contralateral pheochromocytoma. She was managed preoperatively on doxazosin and metoprolol. During the anesthetic evaluation, she had orthostatic hypotension, but no other symptoms related to pheochromocytomas. Elective open left adrenalectomy was carried out under general anesthesia, aiming to blunt stress responses. Hemodynamics were monitored invasively and managed with vasodilator and vasopressor infusions. After the removal of the adrenal tumor, the noradrenaline infusion was stopped as she maintained her stable hemodynamic parameters. She had an uneventful recovery and an orderly discharge from the post-anesthesia recovery unit. This case highlights the importance of meticulous preoperative optimization, vigilant intraoperative monitoring, and readiness with vasoactive agents in managing pheochromocytomas in patients with MEN2A. A multidisciplinary approach is essential to achieve a safe anesthetic course and a favorable outcome in such high-risk scenarios.