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Postoperative hypoparathyroidism from inadequate parathyroid hormone is of concern after multigland resections in multiple endocrine neoplasia type 1-related primary hyperparathyroidism. We evaluated risk factors, long-term outcomes, and roles of autotransplantation and cryopreservation in postoperative hypoparathyroidism in multiple endocrine neoplasia type 1.Retrospective cohort study of patients with multiple endocrine neoplasia type 1 and parathyroidectomy who were evaluated at MD Anderson from 1990 to 2020.We included 206 patients. Median follow-up after the last operation (index 65%, reoperation 35%) was 8 years. Index parathyroidectomy was subtotal in 47%, less than subtotal in 42%, and total in 12%; hypoparathyroidism was more frequent after total parathyroidectomy. Forty-seven patients (23%) had hypoparathyroidism ≥6 months; odds were significantly higher when cumulative ≥4 glands were resected (odds ratio 6 [2.96-12.24]) or when immediate postoperative parathyroid hormone was15 pg/mL (odds ratio 13.10 [3.61-47.47]). After median 26 months postoperatively, 30% recovered parathyroid function spontaneously; this was less likely when ≥4 glands were resected (odds ratio 0.19 [0.05-0.72]). None of the 4 patients who were aparathyroid (parathyroid hormone undetectable or ≤3 pg/mL) at 6 months postoperatively recovered parathyroid function. Immediate autotransplantation success rate was 72%. Cryopreservation was performed in 96 operations with delayed autotransplantation in 10 patients (10% utilization), of whom 5 recovered parathyroid function (time to recovery 12-93 months).Odds of prolonged hypoparathyroidism are higher when cumulative ≥4 glands are resected or postoperative parathyroid hormone is15 pg/mL. Spontaneous recovery occurred but was less likely when ≥4 glands were resected or patients were aparathyroid at 6 months postoperatively. Cryopreservation should be sparingly used, but there is value in select high-risk patients such as reoperative parathyroidectomy/cervical surgery.

Surgical intervention remains the mainstay of treatment of hyperparathyroidism and provides the highest chance at cure. After the disease is confirmed by biochemical testing, surgeons must use a combination of patient clinical history and radiographic imaging to determine the most appropriate surgical strategy. Through either minimally invasive parathyroidectomy or bilateral cervical exploration, surgeons provide high rates of cure for hyperparathyroidism with low rates of persistence or recurrence.

Surgical intervention remains the mainstay of treatment of hyperparathyroidism and provides the highest chance at cure. After the disease is confirmed by biochemical testing, surgeons must use a combination of patient clinical history and radiographic imaging to determine the most appropriate surgical strategy. Through either minimally invasive parathyroidectomy or bilateral cervical exploration, surgeons provide high rates of cure for hyperparathyroidism with low rates of persistence or recurrence.

Neuroendocrine tumors can cause ectopic Cushing syndrome, and most patients have metastatic disease at diagnosis. We identified risk factors for outcome, evaluated ectopic Cushing syndrome management, and explored the role of bilateral adrenalectomy in this population.This was a retrospective study including patients with diagnosis of ectopic Cushing Syndrome secondary to neuroendocrine tumors with adrenocorticotropic hormone secretion treated at our quaternary referral center over a 40-year period (1980-2020).Seventy-six patients were included. Mean age at diagnosis was 46.3 ± 15.8 years. Most patients (N = 61, 80%) had metastases at ectopic Cushing syndrome diagnosis. Average follow-up was 2.9 ± 3.7 years (range, 4 months-17.2 years). Patients with neuroendocrine tumors before ectopic Cushing syndrome had more frequent metastatic disease and resistant ectopic Cushing syndrome. Patients with de novo hyperglycemia, poor neuroendocrine tumor differentiation, and metastatic disease had worse survival. Of those with nonmetastatic disease, 8 (53%) had ectopic Cushing syndrome resolution after neuroendocrine tumor resection, 3 (20%) were medically controlled, and 4 (27%) underwent bilateral adrenalectomy. In patients with metastatic neuroendocrine tumors, hypercortisolism was initially medically managed in 92%, 3% underwent immediate bilateral adrenalectomy, 2% had control after primary neuroendocrine tumor debulking, and 2% were lost to follow-up. Medical treatment resulted in hormonal control in 7 (13%) patients. Of the 49 patients with metastatic disease and medically resistant ectopic Cushing syndrome, 23 ultimately had bilateral adrenalectomy with ectopic Cushing syndrome cure in all.Patients with neuroendocrine tumors before ectopic Cushing syndrome development were more likely metastatic and had worse survival. De novo hyperglycemia and poor neuroendocrine tumor differentiation were predictive of worse prognosis. Medical control of hypercortisolism is difficult to achieve in patients with neuroendocrine tumors-ectopic Cushing syndrome. Well-selected patients may benefit from bilateral adrenalectomy early in the treatment algorithm, and multidisciplinary management is essential in this complex disease.