Modern Surgical Management of Familial and Sporadic Parathyroid and Adrenal Disorders

Abstract

Primary hyperparathyroidism (pHPT) is a common disease of the parathyroid glands. Multiple endocrine neoplasia (MEN) 1-related pHPT is most often caused by multiglandular disease and can best be treated with subtotal parathyroidectomy (3-3½ glands) and bilateral transcervical thymectomy to lower the risk of recurrent and persistent disease. Also, the risk of permanent hypoparathyroidism (hypocalcaemia) is significantly lower than after total parathyroidectomy with autotransplantation.MEN2A-related and sporadic pHPT are most often caused by solitary parathyroid disease and can be treated effectively (low recurrence rate) and safely (low risk of hypoparathyroidism and nerve damage) with minimally invasive parathyroidectomy. Laparoscopic adrenalectomy is the procedure of choice for most adrenal tumors. Tumor size is the most significant predictor of short-term outcome after laparoscopic adrenalectomy. Patients with tumors larger than 3 cm have a longer operation time, a higher conversion rate, more estimated blood loss, more intraoperative and postoperative complications, and a longer postoperative hospital stay compared with patients with tumors smaller than 3 cm. In addition, the clinicopathological diagnosis has an effect on surgical outcome, however to a lesser degree than tumor size. Patients with adrenal metastases, hypercortisolism, and pheochromocytoma had more blood loss than patients with aldosteronoma, while operation time for patients with pheochromocytoma was longer. Identification and control of the adrenal vein are critical steps in laparoscopic adrenalectomy. Variants of adrenal venous anatomy, in the number of veins draining the adrenal gland or in the location of the adrenal vein in relation to the hepatic vein or inferior phrenic vein, occur in a significant percentage of patients, particularly in patients with pheochromocytomas and large tumors. The presence of variant anatomy doesn’t, however, lead to more perioperative complications of laparoscopic adrenalectomy. A pheochromocytoma is a rare (nor)epinephrine-secreting, neuro-endocrine tumor of the adrenal gland. It can cause severe hypertension, headache, and palpitations, but also a crisis, which can lead to shock with multi-organ failure and possibly death. Elective (laparoscopic) adrenalectomy is the treatment of choice. Preoperative treatment with alpha- (and beta-) blockers is necessary to lower the risk of intraoperative hemodynamic instability, multi-organ failure and severe hypotension after tumor resection. Both patients presenting in crisis as well as patients with MEN2 and small tumors, who often present with less symptoms and less severe hypertension, should not be excluded from this (pre)treatment regime. Pheochromocytomas in MEN2 occur in half of the patients, and are frequently bilateral. Laparoscopic unilateral subtotal adrenalectomy is a feasible surgical strategy for pheochromocytoma in MEN2 patients. It is an acceptable method, because malignant pheochromocytoma rarely occur in MEN2 patients and because of the generally slow progression of the disease in MEN2, yearly screening and timely surgery can minimize the risk of a hypertensive crisis from recurrent pheochromocytoma. Subtotal adrenalectomy has low recurrence rates and the great advantage of preserving adrenocortical function, thereby preventing the need for and complications of chronic steroid replacement.