Clinical Use of Cinacalcet in MEN1 Hyperparathyroidism

Abstract

Background. Management of multiple-endocrine neoplasia type 1- (MEN1-) associated hyperparathyroidism is associated with high recurrence rates and high surgical morbidity due to multiple neck explorations. Cinacalcet, a calcimimetic agent licensed for the treatment of secondary hyperparathyroidism and parathyroid carcinoma, may provide a medical alternative for the management of these complex patients. Methods. A prospective audit was performed of eight patients; three males and five females, aged 20–38 at diagnosis. Two patients commenced cinacalcet as primary treatment and six had previous surgery. Six patients had complications of hyperparathyroidism: renal calculi, renal dysfunction, and reduced bone mineral density. All were commenced on cinacalcet 30 mg bd for MEN1 associated hyperparathyroidism; doses were subsequently reduced to 30 mg od in four patients. Results. Significant reductions were observed in serum calcium and PTH measurements. Serum calcium reduced by a median of 0.35 mmol/L (P = .012 Wilcoxon Signed Rank). Serum PTH levels decreased by a median of 5.05 pmol/L (P = .012). There was no change in urine calcium. Duration ranged from 10–35 months with maintenance of control. Cinacalcet was well tolerated by six patients; one experienced nausea and one experienced diarrhoea. Conclusion. Cinacalcet is an effective and well-tolerated medical treatment for the management of complex primary hyperparathyroidism.

MEN1-related hyperparathyroidism: response to cinacalcet and its relationship with the calcium-sensing receptor gene variant Arg990Gly

“…Although MEN1-related PHPT patients are likely the best candidates for a medical treatment, the effectiveness of cinacalcet in MEN1 has been poorly investigated, probably due to low frequency of the disorder that is estimated in two to ten cases per 100 000 in the general population. Indeed, three different studies described the efficacy of cinacalcet in a total of 11 patients (10,11,12).…”

“…According to the European Medicines Agency (EMA) prescription labeling, cinacalcet is indicated for the treatment of patients with secondary HPT due to chronic kidney disease, patients with PHPT due to parathyroid carcinoma, and patients with PHPT meeting the criteria for parathyroidectomy who have contraindications to surgery or persistent PHPT after surgery. Although MEN1 patients are good candidates for a medical treatment, the effectiveness of cinacalcet has been so far reported in 11 patients described in three different studies (10,11,12).…”

Current evidence for recommendation of surgery, medical treatment and vitamin D repletion in mild primary hyperparathyroidism

“…Cinacalcet has been effectively used to lower serum calcium levels in patients with MEN1-associated PHPT, a condition in which the rate of a successful surgery is lower than in the sporadic forms of PHPT (79,80).…”

Cystic parathyroid glands in MEN1: A rare entity?

“…Cinacalcet hydrochloride has been approved for the treatment of hypercalcemia in patients with parathyroid carcinoma and in primary HPT patients unable to undergo surgery [54]. Three separate studies have reported its effectiveness in 11 MEN1 patients [54][55][56].…”

“…Three separate studies have reported its effectiveness in 11 MEN1 patients [54][55][56]. Cinacalcet is also the only available potent calcimimetic for the management of secondary HPT [57].…”