Purpose <p>Subtotal parathyroidectomy (STP) is the preferred surgery for MEN1-related primary hyperparathyroidism (PHPT), balancing recurrence and complication risks. <i>MEN1</i>-negative patients show a milder disease course, supporting the hypothesis that post-operative outcomes may also differ between <i>MEN1</i>-positive and <i>MEN1</i>-negative patients.</p> Methods <p>We conducted a retrospective study of 101 MEN1 patients undergoing parathyroidectomy to compare PHPT outcomes by genetic status and surgical approach. <i>MEN1</i>-positive patients mainly received total parathyroidectomy (TP) or STP, while <i>MEN1</i>-negative patients underwent less than subtotal parathyroidectomy (LSTP).</p> Results <p>No significant differences were found between <i>MEN1</i>-positive and <i>MEN1</i>-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 <i>MEN1</i>-positive patients (12.7%). In a sub-analysis of LSTP cases, <i>MEN1</i>-negative patients showed significantly better outcomes, with higher remission (47.6% vs. 16.6%, p = 0.01), and lower recurrence and persistence. Among <i>MEN1</i>-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&#xa0;months, p = 0.032).</p> Conclusions <p><i>MEN1</i> genetic status and surgical strategy both shape PHPT outcomes. <i>MEN1</i>-positive patients benefit from more extensive surgery, performed at high-volume centers, whereas <i>MEN1</i>-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.</p>

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Role of MEN1 mutations on postoperative outcomes of patients with multiple endocrine neoplasia type 1-related primary hyperparathyroidism: a single center experience

  • Laura Pierotti,
  • Elena Pardi,
  • Chiara Sardella,
  • Simone Della Valentina,
  • Anna Dal Lago,
  • Paolo Piaggi,
  • Gabriele Materazzi,
  • Gianluca Frustaci,
  • Leonardo Rossi,
  • Fausto Bogazzi,
  • Liborio Torregrossa,
  • Angela Michelucci,
  • Maria Adelaide Caligo,
  • Claudio Marcocci,
  • Filomena Cetani

摘要

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.