Journal of endocrinological investigation · 2025
Role of MEN1 mutations on postoperative outcomes of patients with multiple endocrine neoplasia type 1-related primary hyperparathyroidism: a single center experience.
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Pierotti Laura, Pardi Elena, Sardella Chiara, Valentina Simone Della, Lago Anna Dal, Piaggi Paolo, Materazzi Gabriele, Frustaci Gianluca, Rossi Leonardo, Bogazzi Fausto, Torregrossa Liborio, Michelucci Angela, Caligo Maria Adelaide, Marcocci Claudio, Cetani Filomena
Аннотация
PURPOSE: Subtotal parathyroidectomy (STP) is the preferred surgery for MEN1-related primary hyperparathyroidism (PHPT), balancing recurrence and complication risks. MEN1-negative patients show a milder disease course, supporting the hypothesis that post-operative outcomes may also differ between MEN1-positive and MEN1-negative patients.
METHODS: We conducted a retrospective study of 101 MEN1 patients undergoing parathyroidectomy to compare PHPT outcomes by genetic status and surgical approach. MEN1-positive patients mainly received total parathyroidectomy (TP) or STP, while MEN1-negative patients underwent less than subtotal parathyroidectomy (LSTP).
RESULTS: No significant differences were found between MEN1-positive and MEN1-negative patients in remission (p = 0.423), recurrence (p = 0.509) or persistence (p = 0.814) rates, regardless of surgical approach. Chronic postsurgical hypoparathyroidism occurred only in MEN1-positive patients (12.7%). In a sub-analysis of LSTP cases, MEN1-negative patients showed significantly better outcomes, with higher remission (47.6% vs. 16.6%, p = 0.01), and lower recurrence and persistence. Among MEN1-positive patients, TP or STP led to better outcomes compared to LSTP, with higher remission (60.5% vs. 16.7%, p = 0.00005), and lower recurrence and persistence. Recurrence occurred earlier after LSTP (84.0 vs. 160.0 months, p = 0.032).
CONCLUSIONS: MEN1 genetic status and surgical strategy both shape PHPT outcomes. MEN1-positive patients benefit from more extensive surgery, performed at high-volume centers, whereas MEN1-negative patients have more favorable outcomes with limited resections, though their remission still trails that of sporadic PHPT. These differences underscore the need for individualized surgical planning.
METHODS: We conducted a retrospective study of 101 MEN1 patients undergoing parathyroidectomy to compare PHPT outcomes by genetic status and surgical approach. MEN1-positive patients mainly received total parathyroidectomy (TP) or STP, while MEN1-negative patients underwent less than subtotal parathyroidectomy (LSTP).
RESULTS: No significant differences were found between MEN1-positive and MEN1-negative patients in remission (p = 0.423), recurrence (p = 0.509) or persistence (p = 0.814) rates, regardless of surgical approach. Chronic postsurgical hypoparathyroidism occurred only in MEN1-positive patients (12.7%). In a sub-analysis of LSTP cases, MEN1-negative patients showed significantly better outcomes, with higher remission (47.6% vs. 16.6%, p = 0.01), and lower recurrence and persistence. Among MEN1-positive patients, TP or STP led to better outcomes compared to LSTP, with higher remission (60.5% vs. 16.7%, p = 0.00005), and lower recurrence and persistence. Recurrence occurred earlier after LSTP (84.0 vs. 160.0 months, p = 0.032).
CONCLUSIONS: MEN1 genetic status and surgical strategy both shape PHPT outcomes. MEN1-positive patients benefit from more extensive surgery, performed at high-volume centers, whereas MEN1-negative patients have more favorable outcomes with limited resections, though their remission still trails that of sporadic PHPT. These differences underscore the need for individualized surgical planning.