Skip to main content
SpringerLink
Log in

The Optimal Surgical Treatment for Primary Hyperparathyroidism in MEN1 Patients: A Systematic Review

  • Published:
World Journal of Surgery Aims and scope Submit manuscript

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.

Conclusion

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Pieterman CR, Schreinemakers JM, Koppeschaar HP et al (2009) Multiple endocrine neoplasia type 1 (MEN1): its manifestations and effect of genetic screening on clinical outcome. Clin Endocrinol (Oxf) 70(4):575–581

    Article  CAS  Google Scholar 

  2. Carty SE, Helm AK, Amico JA et al (1998) The variable penetrance and spectrum of manifestations of multiple endocrine neoplasia type 1. Surgery 124:1106–1113 discussion 1113–1114

    Article  PubMed  CAS  Google Scholar 

  3. Akerstrom G, Juhlin C, Skogseid B (1994) Surgical treatment of multiple endocrine neoplasia type 1 (MEN I) associated parathyroid hyperplasia. Acta Chir Austriaca 26:30–32

    Article  Google Scholar 

  4. Malone JP, Srivastava A, Khardori R (2004) Hyperparathyroidism and multiple endocrine neoplasia. Otolaryngol Clin North Am 37:715–736, viii

    Google Scholar 

  5. Doherty GM, Lairmore TC, DeBenedetti MK (2004) Multiple endocrine neoplasia type 1 parathyroid adenoma development over time. World J Surg 28:1139–1142

    Article  PubMed  Google Scholar 

  6. Lambert LA, Shapiro SE, Lee JE et al (2005) Surgical treatment of hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Arch Surg 140:374–382

    Article  PubMed  Google Scholar 

  7. Norton JA, Venzon DJ, Berna MJ et al (2008) Prospective study of surgery for primary hyperparathyroidism (HPT) in multiple endocrine neoplasia-type 1 and Zollinger-Ellison syndrome: long-term outcome of a more virulent form of HPT. Ann Surg 247:501–510

    Article  PubMed  Google Scholar 

  8. Burgess JR, David R, Parameswaran V et al (1998) The outcome of subtotal parathyroidectomy for the treatment of hyperparathyroidism in multiple endocrine neoplasia type 1. Arch Surg 133:126–129

    Article  PubMed  CAS  Google Scholar 

  9. Tonelli F, Marcucci T, Fratini G et al (2007) Is total parathyroidectomy the treatment of choice for hyperparathyroidism in multiple endocrine neoplasia type 1? Ann Surg 246:1075–1082

    Article  PubMed  Google Scholar 

  10. Arnalsteen LC, Alesina PF, Quiereux JL et al (2002) Long-term results of less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1. Surgery 132:1119–1124 discussion 1124-1115

    Article  PubMed  Google Scholar 

  11. Cougard P, Proye C (1994) Hyperparathyroidism and multiple endocrine neoplasia type I (MEN I). Acta Chir Austriaca 26:32–35

    Google Scholar 

  12. Kivlen MH, Bartlett DL, Libutti SK et al (2001) Reoperation for hyperparathyroidism in multiple endocrine neoplasia type 1. Surgery 130:991–998

    Article  PubMed  CAS  Google Scholar 

  13. Hellman P, Skogseid B, Oberg K et al (1998) Primary and reoperative parathyroid operations in hyperparathyroidism of multiple endocrine neoplasia type 1. Surgery 124:993–999

    Article  PubMed  CAS  Google Scholar 

  14. Hubbard JG, Sebag F, Maweja S et al (2006) Subtotal parathyroidectomy as an adequate treatment for primary hyperparathyroidism in multiple endocrine neoplasia type 1. Arch Surg 141:235–239

    Article  PubMed  Google Scholar 

  15. Elaraj DM, Skarulis MC, Libutti SK et al (2003) Results of initial operation for hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Surgery 134:858–864 discussion 864-855

    Article  PubMed  Google Scholar 

  16. Harach HR, Jasani B (1992) Parathyroid hyperplasia in multiple endocrine neoplasia type 1: a pathological and immunohistochemical reappraisal. Histopathology 20:305–313

    Article  PubMed  CAS  Google Scholar 

  17. Dotzenrath C, Cupisti K, Goretzki PE et al (2001) Long-term biochemical results after operative treatment of primary hyperparathyroidism associated with multiple endocrine neoplasia types I and IIa: is a more or less extended operation essential? Eur J Surg 167:173–178

    Article  PubMed  CAS  Google Scholar 

  18. Hubbard JG, Sebag F, Maweja S et al (2002) Primary hyperparathyroidism in MEN 1–how radical should surgery be? Langenbecks Arch Surg 386:553–557

    Article  PubMed  Google Scholar 

  19. Malmaeus J, Benson L, Johansson H (1986) Parathyroid surgery in the multiple endocrine neoplasia type I syndrome: Choice of surgical procedure. World J Surg 10:668–672

    Article  PubMed  CAS  Google Scholar 

  20. Rizzoli R, Green J 3rd, Marx SJ (1985) Primary hyperparathyroidism in familial multiple endocrine neoplasia type I. Long-term follow-up of serum calcium levels after parathyroidectomy. Am J Med 78:467–474

    Article  PubMed  CAS  Google Scholar 

  21. Dralle H, Scheumann GFW (1994) How to handle the parathyroid glands in multiple endocrine neoplasia type I (MEN I) and type II (MEN II)? Surgical approach to uniglandular versus multiglandular disease in hereditary primary hyperparathyroidism. Acta Chir Austriaca 26:35–38

    Google Scholar 

  22. Lee CH, Tseng LM, Chen JY et al (2006) Primary hyperparathyroidism in multiple endocrine neoplasia type 1: individualized management with low recurrence rates. Ann Surg Oncol 13:103–109

    Article  PubMed  Google Scholar 

  23. Jansson S, Tisell LE (1994) Total parathyroidectomy and parathyroid transplantation into subcutaneous fat tissue in the treatment of hyperparathyroidism in multiple endocrine neoplasia type I (MEN I). Acta Chir Austriaca 26:23–26

    Article  Google Scholar 

  24. Langer P, Wild A, Schilling T et al (2004) Multiple endocrine neoplasia type 1. Surgical therapy of primary hyperparathyroidism. Chirurg 75:900–906

    Article  PubMed  CAS  Google Scholar 

  25. O’Riordain DS, O’Brien T, Grant CS et al (1993) Surgical management of primary hyperparathyroidism in multiple endocrine neoplasia types 1 and 2. Surgery 114:1031–1037 discussion 1037–1039

    PubMed  Google Scholar 

  26. Bonjer HJ (2003) Technique of parathyroidectomy. In: Clark OH (ed) Textbook of Endocrine Surgery. Elsevier Saunders, Philadelphia

    Google Scholar 

  27. Kraimps JL, Duh QY, Demeure M et al (1992) Hyperparathyroidism in multiple endocrine neoplasia syndrome. Surgery 112:1080–1086 discussion 1086–1088

    PubMed  CAS  Google Scholar 

  28. Gauger PG, Thompson NW (2001) Early surgical intervention and strategy in patients with multiple endocrine neoplasia type I. Best Pract Res Clin Endocrinol Metab 15:213–223

    Article  PubMed  CAS  Google Scholar 

  29. Goudet P, Cougard P, Verges B et al (2001) Hyperparathyroidism in multiple endocrine neoplasia type I: surgical trends and results of a 256-patient series from Groupe D’etude des Neoplasies Endocriniennes Multiples Study Group. World J Surg 25:886–890

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

We thank Prof. Lips for reading the manuscript. This work was supported by the Michael van Vloten Fund of the Dutch Surgical Society and the Dutch Cancer Society.

Author information

Authors and Affiliations

  1. Department of Surgery, University Medical Center Utrecht, Heidelberglaan 100, 3584CX, Utrecht, The Netherlands

    Jennifer M. J. Schreinemakers, Anouk Scholten, Menno R. Vriens & Inne H. M. Borel Rinkes

  2. Department of Endocrinology, University Medical Center Utrecht, Utrecht, The Netherlands

    Carolina R. C. Pieterman & Gerlof D. Valk

  3. Department of Surgery, Amphia Hospital, Breda, The Netherlands

    Jennifer M. J. Schreinemakers

Authors
  1. Jennifer M. J. Schreinemakers
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Carolina R. C. Pieterman
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Anouk Scholten
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Menno R. Vriens
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Gerlof D. Valk
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Inne H. M. Borel Rinkes
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Inne H. M. Borel Rinkes.

Additional information

G. D. Valk and I. H. M. Borel Rinkes contributed equally to this work.

Appendices

Appendix A: Predefined search terms

MEN1

“MEN 1” OR “MEN1” OR “MENI” OR “MEN I” OR “multiple endocrine neoplasia type 1” OR “multiple endocrine neoplasia syndrome type 1”.

Primary hyperparathyroidism

“Hyperparathyroidism, Primary”[Mesh] OR “Parathyroid Neoplasms”[Mesh] OR “primary hyperparathyroidism” OR “hyperparathyroidism” OR “HPT” OR “parathyroid adenoma*” OR “parathyroid hyperplasia”.

Surgical procedure

“Parathyroidectomy”[Mesh] OR “parathyroid surgery” OR “parathyroidectomy” OR “total parathyroidectomy” OR “subtotal parathyroidectomy” OR “conventional neck exploration” OR “unilateral neck exploration” OR “minimally invasive adenomectomy”.

Appendix B: Summary of the outcomes of primary surgical treatment of pHPT in MEN1 patients in the literature

See Table 4.

Table 4  
Full size table

Appendix C1: Comparison of <SPTX versus SPTX/TPTX on persistent pHPT

<SPTX fewer than 3 parathyroid glands resected, SPTX 3–3½ parathyroid glands resected, TPTX total parathyroidectomy with autotransplantation, SPTX and TPTX are analyzed together

After <SPTX, patients are more likely to develop persistent pHPT than after SPTX/TPTX (OR = 4.97, 95% CI = 2.11–11.71, P = 0.0002)

Appendix C2: Comparison of SPTX versus TPTX for persistent pHPT

SPTX 3–3½ parathyroid glands resected, TPTX total parathyroidectomy with autotransplantation

After SPTX, patients do not have a significantly higher risk of developing persistent pHPT than after TPTX (OR = 2.37, 95% CI = 0.54–10.44, P = 0.25)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Schreinemakers, J.M.J., Pieterman, C.R.C., Scholten, A. et al. The Optimal Surgical Treatment for Primary Hyperparathyroidism in MEN1 Patients: A Systematic Review. World J Surg 35, 1993–2005 (2011). https://doi.org/10.1007/s00268-011-1068-9

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00268-011-1068-9

Keywords

Search

Navigation