Multiple endokrine Neoplasie Typ 1

Langer, Peter; Wild, Anja; Schilling, Tobias; Nies, C.; Rothmund, M.; Bartsch, Detlef K.

doi:10.1007/s00104-004-0838-4

Cited by 17 publications

(7 citation statements)

References 29 publications

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“…We investigated if different genotypes could help to predict surgical outcomes. Previous studies did not find genotype–phenotype correlation in relation to clinical presentation or severity (8, 24, 25), but one study including 73 patients with MEN1 and surgically treated PHPT found a significantly lower risk of persistence and recurrence after <SPTX, but not after SPTX/TPTX, in patients with nonsense or frameshift mutations in exons 2, 9, and 10 when compared with patients with other mutations (10). Another study found that mutations in exon 3 were associated with worse recurrence rates; however, all patients with mutations in exon 3 had been initially operated with <SPTX (11).…”

Section: Discussionmentioning

confidence: 92%

MEN1-associated primary hyperparathyroidism in the Spanish Registry: clinical characterictics and surgical outcomes

Lamas

Navarro

Casteràs

et al. 2019

Endocrine Connections

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Primary hyperparathyroidism is the most frequent manifestation of multiple endocrine neoplasia type 1 (MEN1) syndrome. Bone and renal complications are common. Surgery is the treatment of choice, but the best timing for surgery is controversial and predictors of persistence and recurrence are not well known. Our study describes the clinical characteristics and the surgical outcomes, after surgery and in the long term, of the patients with MEN1 and primary hyperparathyroidism included in the Spanish Registry of Multiple Endocrine Neoplasia, Pheochromocytomas and Paragangliomas (REGMEN). Eighty-nine patients (49 men and 40 women, 34.2 ± 13 years old) were included. Sixty-four out of the 89 underwent surgery: a total parathyroidectomy was done in 13 patients, a subtotal parathyroidectomy in 34 and a less than subtotal parathyroidectomy in 15. Remission rates were higher after a total or a subtotal parathyroidectomy than after a less than subtotal (3/4 and 20/22 vs 7/12, P < 0.05), without significant differences in permanent hypoparathyroidism (1/5, 9/23 and 0/11, N.S.). After a median follow-up of 111 months, 20 of the 41 operated patients with long-term follow-up had persistent or recurrent hyperparathyroidism. We did not find differences in disease-free survival rates between different techniques, patients with or without permanent hypoparathyroidism and patients with different mutated exons, but a second surgery was more frequent after a less than subtotal parathyroidectomy.

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Section: Discussionmentioning

confidence: 92%

MEN1-associated primary hyperparathyroidism in the Spanish Registry: clinical characterictics and surgical outcomes

Lamas

Navarro

Casteràs

et al. 2019

Endocrine Connections

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“…Twenty‐one studies [4, 10, 11, 15–20, 22–29, 31, 32, 34, 36] involving 748 patients evaluated the occurrence of permanent hypoparathyroidism after LSTP and STP. Fifteen patients out of 338 (4.4%) had permanent hypoparathyroidism in the LSTP group, while 82 patients out of 410 (20%) had permanent hypoparathyroidism in the STP group.…”

Section: Resultsmentioning

confidence: 99%

“…Nineteen studies [4,10,11,[15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] involving 696 patients evaluated the persistence of pHPT after LSTP and STP. Seventy-two out of 312 patients (23.1%) had persistent HPT in the LSTP group versus 17 out of 384 (4.4%) in the STP group.…”

Section: Persistent Hyperparathyroidismmentioning

confidence: 99%

Less Than Subtotal Parathyroidectomy for Multiple Endocrine Neoplasia Type 1 Primary Hyperparathyroidism: A Systematic Review and Meta‐Analysis

et al. 2022

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Background Multiple endocrine neoplasia type 1 (MEN1)‐associated primary hyperparathyroidism (pHPT) is classically associated with an asymmetric and asynchronous parathyroid involvement. Subtotal parathyroidectomy (STP), which is currently the recommended surgical treatment, carries a high risk of permanent hypoparathyroidism. The results of less than subtotal parathyroidectomy (LSTP) are conflicting, and its place in this setting is still a matter of debate. The aim of this study was to identify the place of LSTP in the surgical management of patients with MEN‐associated pHPT. Methods A systematic literature review was conducted in accordance with PRISMA and MOOSE guidelines, for studies comparing STP and LSTP for MEN1‐associated pHPT. The results of the two techniques, regarding permanent hypoparathyroidism, persistent hyperparathyroidism and recurrent hyperparathyroidism were computed using pairwise random‐effect meta‐analysis. Results Twenty‐five studies comparing STP and LSTP qualified for inclusion in the quantitative synthesis. In total, 947 patients with MEN1‐associated pHPT were allocated to STP (n = 569) or LSTP (n = 378). LSTP reduces the risk of permanent hypoparathyroidism [odds ratio (OR) 0.29, confidence interval (CI) 95% 0.17–0.49)], but exposes to higher rates of persistent hyperparathyroidism [OR 4.60, 95% CI 2.66–7.97]. Rates of recurrent hyperparathyroidism were not significantly different between the two groups [OR 1.26, CI 95% 0.83–1.91]. Conclusions LSTP should not be abandoned and should be considered as a suitable surgical option for selected patients with MEN1‐associated pHPT. The increased risk of persistent hyperparathyroidism could improve with the emergence of more efficient preoperative localization imaging techniques and a more adequate patients selection.

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“…Controversial data exists on whether recurrence in patients with pHPT and MEN1 is associated with a specific genotype. 16 A recent report by the DutchMEN1 Study Group, found that genotype did affect the chance of recurrence after surgical exploration in 73 patients with pHPT and MEN1. However, the decreased incidence of recurrence was only found in patients with mutations in exons 2, 7, and 9 who had less than three parathyroid glands resected during initial operation.…”

Section: Discussionmentioning

confidence: 97%

Reoperative Surgery in Patients with Multiple Endocrine Neoplasia Type 1 Associated Primary Hyperparathyroidism

et al. 2016

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Although preoperative localizing studies are helpful for guiding reoperative strategy in MEN1 with persistent/recurrent pHPT, additional enlarged glands may be missed by conventional imaging. IOPTH should therefore be employed routinely in this setting. Routine cryopreservation should be considered in all patients. Pancreatic manifestation may be associated with earlier recurrence or persistent disease.

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Multiple endokrine Neoplasie Typ 1

Cited by 17 publications

References 29 publications

MEN1-associated primary hyperparathyroidism in the Spanish Registry: clinical characterictics and surgical outcomes

MEN1-associated primary hyperparathyroidism in the Spanish Registry: clinical characterictics and surgical outcomes

Less Than Subtotal Parathyroidectomy for Multiple Endocrine Neoplasia Type 1 Primary Hyperparathyroidism: A Systematic Review and Meta‐Analysis

Reoperative Surgery in Patients with Multiple Endocrine Neoplasia Type 1 Associated Primary Hyperparathyroidism

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