Cortical-sparing adrenalectomy is an option for patients with bilateral pheochromocytomas or for those with only one viable adrenal gland to minimize the risk of adrenal insufficiency. Fourteen (93%) of 15 patients undergoing laparotomy for bilateral pheochromocytomas were treated with cortical-sparing adrenalectomy; 13 patients did not require postoperative steroid hormone supplementation, and none experienced acute adrenal insufficiency. Three patients developed recurrent pheochromocytomas at 10 to 27 years after surgery. Similar results were obtained in a series of 26 patients undergoing cortex-sparing surgery for hereditary pheochromocytoma (including MEN 2). This type of surgery may also be accomplished laparoscopically, with intraoperative ultrasound guidance; however, these approaches require long-term follow-up, as recurrence may develop many years after the initial operation.
Level of evidence: 5
Treatment for those with hyperparathyroidism
Most patients with MEN2-related parathyroid disease are either asymptomatic or diagnosed incidentally at the time of thyroidectomy. Typically, the hypercalcemia (when present) is mild, although it may be associated with increased urinary excretion of calcium and nephrolithiasis. As a consequence, the indications for surgical intervention are generally similar to those recommended for patients with sporadic, primary hyperparathyroidism. In general, fewer than four of the parathyroid glands are involved at the time of detected abnormalities in calcium metabolism.
Cure of hyperparathyroidism was achieved surgically in 89% of one large series of patients; however, 22% of resected patients in this study developed postoperative hypoparathyroidism. Five patients (9%) had recurrent hyperparathyroidism. This series employed various surgical techniques, including total parathyroidectomy with autotransplantation to the nondominant forearm, subtotal parathyroidectomy, and resection only of glands that were macroscopically enlarged. Postoperative hypoparathyroidism developed in 4 (36%) of 11 patients, 6 (50%) of 12 patients, and 3 (10%) of 29 patients, respectively. These data indicate that excision of only those parathyroid glands that are enlarged appears to be sufficient in most cases.
Some investigators have suggested using the MEN 2 subtype to decide where to place the parathyroid glands that are identified at the time of thyroid surgery. For patients with MEN 2B in whom the risk of parathyroid disease is quite low, the parathyroid glands may be left in the neck. For patients with MEN 2A and FMTC, it is suggested that the glands be implanted in the nondominant forearm to minimize the need for further surgery on the neck after risk-reducing thyroidectomy and a central lymph node dissection.
All patients who have undergone parathyroid surgery with autotransplantation of parathyroid tissue should be monitored for hypoparathyroidism.[24,151,152]
Medical therapy of hyperparathyroidism has gained popularity with the advent of calcimimetics, agents that sensitize the calcium-sensing receptors on the parathyroid glands to circulating calcium levels and thereby reduce circulating PTH levels. In a randomized, double-blind, placebo-controlled trial, cinacalcet hydrochloride was shown to induce sustained reduction in circulating calcium and PTH levels in patients with primary hyperparathyroidism. In patients who are high-risk surgical candidates, those with recurrent hyperparathyroidism, or those in whom life expectancy is limited, medical therapy may be a viable alternative to a surgical approach.