title: The optimal surgical treatment for primary hyperparathyroidism in MEN1 patients: a systematic review.
abstract: BACKGROUND
  The optimal surgical approach for patients with primary hyperparathyroidism (pHPT) and multiple endocrine neoplasia 1 (MEN1) is controversial. We sought to determine the optimal type of surgery for pHPT in MEN1.
  
  
  METHODS
  We collected data on clinical presentation, surgery, and follow-up for MEN1 patients with pHPT at the University Medical Center Utrecht and affiliated hospitals between 1967 and 2008. Furthermore, we performed a systematic review of the literature and meta-analysis. Surgical procedures were classified into less than subtotal (<SPTX) versus subtotal (SPTX) and total parathyroidectomy (TPTX).
  
  
  RESULTS
  Fifty-two patients underwent primary surgery for pHPT, of which 29 had <SPTX, 17 SPTX, and 6 TPTX. Recurrent pHPT was most frequent after SPTX (65%) followed by <SPTX (59%). Persistent disease was most frequent after <SPTX (31%). Time to recurrence was 61 months longer after SPTX than after <SPTX. Although recurrent pHPT was not seen after TPTX, permanent hypoparathyroidism developed in 67% of these patients. The meta-analysis showed that after SPTX and TPTX, patients had the lowest risk of persistent and recurrent pHPT. TPTX had the highest risk of permanent hypoparathyroidism. Large noncomparative studies showed a low recurrence rate after SPTX and TPTX.
  
  
  CONCLUSIONS
  We believe that SPTX is the best surgical therapy for pHPT in MEN1. MEN1 patients with pHPT should not be treated with <SPTX because of the unacceptable high rate of recurrent and persistent pHPT. Additionally, a thymectomy should routinely be performed in these patients.
authors.affiliation: Department of Surgery, University Medical Center Utrecht, Heidelberglaan 100, 3584CX, Utrecht, The Netherlands.
authors.forename: Jennifer M J
authors.initials: JM
authors.lastname: Schreinemakers
authors.forename: Carolina R C
authors.initials: CR
authors.lastname: Pieterman
authors.forename: Anouk
authors.initials: A
authors.lastname: Scholten
authors.forename: Menno R
authors.initials: MR
authors.lastname: Vriens
authors.forename: Gerlof D
authors.initials: GD
authors.lastname: Valk
authors.forename: Inne H M Borel
authors.initials: IH
authors.lastname: Rinkes
mesh_terms: D000328:Adult; D015331:Cohort Studies; D015897:Comorbidity; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D049950:Hyperparathyroidism, Primary / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D016105:Parathyroidectomy / Q000009:adverse effects* / Q000379:methods; D011183:Postoperative Complications / Q000453:epidemiology / Q000503:physiopathology; D012086:Reoperation / Q000706:statistics & numerical data; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult