Prospective Study of Surgery for Primary Hyperparathyroidism (HPT) in Multiple Endocrine Neoplasia-Type 1 and Zollinger-Ellison Syndrome

Abstract

Background-HPT in MEN1 patients with ZES is caused by parathyroid hyperplasia. Surgery for parathyroid hyperplasia is tricky and difficult. Long-term outcome in ZES/MEN1 /HPT is not well known.

Early-onset, progressive, frequent, extensive, and severe bone mineral and renal complications in multiple endocrine neoplasia type 1–associated primary hyperparathyroidism

“…The mild biochemical disease contrasted with the early-onset, progressive, frequent, extensive, and severe bone demineralization in our HPT/MEN1 subset. The relative excess of symptomatic HPT patients in our sample (81%) also has been reported in other HPT/MEN1 cohorts, (13,15,16,(26)(27)(28) contrasting with the modern SPHPT series (20%). (21)(22)(23)(24)(25) In addition, our data suggest that the asymptomatic stage of MEN1-associated HPT seems to be relatively short because up to 60% of patients younger than 30 years of age were already symptomatic (urolithiasis).…”

“…The high prevalence of urolithiasis-related renal comorbidities in this study confirmed previous data, (27) and the high frequency of urolithiasis found in our gastrinoma/HPT patients is in agreement with others. (13) The progressive impairment of renal function that we observed may be related to the early onset of renal calculi, long-standing uncontrolled HPT, and increasing PTH values with aging. The mechanisms leading to the higher susceptibility to urolithiasis in HPT/MEN1 than in SPHPT remain unclear.…”

“…Also, high frequencies of urolithiasis (>30% to 75%) have been reported systematically in HPT/MEN1, unlike what has been observed in modern SPHPT series (<20%). (1)(2)(3)13,15,(25)(26)(27)(28) Moreover, a progressive decrease in renal function has been reported in patients with SPHPT, although data on the early and late outcomes of urolithiasis-related renal complications in the context of the HPT/MEN1 subset are limited. (1)(2)(3)9,25,27) In the present investigation, we studied 36 patients with uncontrolled primary HPT from 8 unrelated MEN1 families, all of them harboring MEN1 germ-line mutations.…”

Neuroendocrine tumors of the small bowels are on the rise: Early aspects and management

“…Therefore, the ZES in MEN1 has to be treated long-term and effectively with a proton pump inhibitor [64] . Furthermore, the presence of hyperparathyroidism in these patients can make medical control of the gastric hypersecretion more difficult with more frequent and higher PPI doses needed [64,65] . It is therefore recommended that the hyperparathyroidism be treated appropriately (surgical resection of at least 3.5 glands) and the patient followed carefully because it can relapse [64,65] .…”

“…Furthermore, the presence of hyperparathyroidism in these patients can make medical control of the gastric hypersecretion more difficult with more frequent and higher PPI doses needed [64,65] . It is therefore recommended that the hyperparathyroidism be treated appropriately (surgical resection of at least 3.5 glands) and the patient followed carefully because it can relapse [64,65] . With this in mind, MEN1/ ZES patients should be treated in specialized clinics; the surgical option should be considered only in specialized centers and only after an interdisciplinary discussion of the individual patient.…”