Hyperparathyroidism in multiple endocrine neoplasia type I: surgical trends and results of a 256-patient series from Groupe D'etude des Néoplasies Endocriniennes Multiples Study Group

Abstract

The French and Belgian GENEM study group's multiple endocrine neoplasia type I (MEN-I) database was used to evaluate trends in clinical presentation, surgical treatment of primary hyperparathyroidism (pHPT) (n = 245), and prognostic factors for hypercalcemia correction among 256 MEN-I cases. The patients were retrieved through the GENEM network from various Belgian and French institutions with the help of genetics laboratories. Among the 245 pHPT patients (96%), 42% were men. The mean age at the time of diagnosis was 39.5 +/- 13.3 years. Trends were studied for three periods: before 1986, from 1986 to 1990, and thereafter. After 1990 MEN-I patients were more often diagnosed with isolated pHPT (8%, 11%, 28%, for the three periods, respectively; p = 0.002); it was seen more often in screened patients (31%, 28%, 53%; p = 0.001), more often among those in already known MEN-I families (64%, 45%, 72%; p = 0.005), and among those with lower preoperative calcemia (2.93, 2.87, 2.79 mmol/L; p = 0.001). The age at pHPT diagnosis remained constant throughout the study. The percentage of cervical explorations dropped during the entire study (87%, 87%, 53%; p < 0.0001). After 1985 the percentage of subtotal parathyroidectomies increased (25%, 59%, 51%; p = 0.0004). Pathology disclosed more hyperplasias (59%, 85%, 74%; p = 0.008). Postoperative hypercalcemia decreased (47%, 15%, 19%; p < 0.0001); and postoperative hypocalcemia increased nonsignificantly (5%, 15%, 15%; p = 0.1). Subtotal parathyroidectomy [odds ratio (OR) 13], no MEN-I family background (OR 3), and the most recent study period (> 1985) (OR 3) were significant predictive factors of hypercalcemia correction according to the multivariate analysis. This is the first multicentric study on the management of MEN-I-related pHPT. Immediate postoperative hHPT cure increased, but only 80% of the operated patients were cured after 1990. Fifteen percent were hypocalcemic. Because MEN-I-related hHPT cure remains difficult to achieve, we advocate that subtotal parathyroidectomies be performed in specialized centers.