Subtotal Parathyroidectomy Versus Total Parathyroidectomy with Autotransplantation for Patients with Multiple Endocrine Neoplasia 1 and Primary Hyperparathyroidism

  • Terry C. LairmoreEmail author
Part of the Difficult Decisions in Surgery: An Evidence-Based Approach book series (DDSURGERY)


Hyperparathyroidism in patients with MEN 1 is characterized by multiple gland involvement. Two surgical approaches have been advocated in these patients: total parathyroidectomy with heterotopic autotransplantation of parathyroid tissue grafts into skeletal muscle (TP/AT), or subtotal (3 and ½ gland) parathyroidectomy (SP) leaving a vascularized remnant of parathyroid tissue in situ in the neck. Despite the potential advantages and disadvantages of these two commonly practiced operations, previous retrospective studies have demonstrated similar overall rates of recurrent HPT and permanent postoperative hypoparathyroidism in patients undergoing the two operative procedures. Although one of these approaches is often preferred by individual endocrine surgery centers of excellence, improved outcomes or a clear advantage for either operation has not been previously established. The cumulative evidence from prior retrospective studies and a single randomized controlled trail is presented, and recommendations are made based on the available data. The level of evidence and grade for the strength of these recommendations is provided.


Multiple endocrine neoplasia type 1 Parathyroidectomy Parathyroid transplantation Persistent and recurrent hyperparathyroidism Postoperative hypoparathyroidism 


  1. 1.
    Chandrasekharappa SC, Guru SC, Manickamp P, Olufemi S, Collins FS, Emmert-Buck M, et al. Positional cloning of the gene for multiple endocrine neoplasia-type 1. Science. 1997;276:404–7.CrossRefPubMedGoogle Scholar
  2. 2.
    Doherty GM, Lairmore TC, DeBenedetti MK. Multiple endocrine neoplasia type 1 parathyroid adenoma development over time. World J Surg. 2004;28(11):1139–42.CrossRefPubMedGoogle Scholar
  3. 3.
    Marx S, Spiegel AM, Skarulis MC, Doppman JL, Collins FS, Liotta LA. Multiple endocrine neoplasia type 1: clinical and genetic topics. Ann Intern Med. 1998;129:484–94.CrossRefPubMedGoogle Scholar
  4. 4.
    Lairmore TC, Piersall LD, DeBenedetti MK, Dilley WG, Mutch MG, Whelan AJ, et al. Clinical genetic testing and early surgical intervention in patients with multiple endocrine neoplasia type 1 (MEN 1). Ann Surg. 2004;239(5):637–45. Discussion 45-7.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Lairmore TC, Govednik CM, Quinn CE, Sigmond BR, Lee CY, Jupiter DC. A randomized, prospective trial of operative treatments for hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Surgery. 2014;156(6):1326–34. Discussion 34-5.CrossRefPubMedGoogle Scholar
  6. 6.
    d’Alessandro AF, Montenegro FL, Brandao LG, Lourenco DM Jr, Toledo Sde A, Cordeiro AC. Supernumerary parathyroid glands in hyperparathyroidism associated with multiple endocrine neoplasia type 1. Rev Assoc Med Bras. 2012;58(3):323–7.CrossRefPubMedGoogle Scholar
  7. 7.
    Nilubol N, Weinstein L, Simonds WF, Jensen RT, Phan GQ, Hughes MS, et al. Preoperative localizing studies for initial parathyroidectomy in MEN1 syndrome: is there any benefit? World J Surg. 2012;36(6):1368–74.CrossRefPubMedGoogle Scholar
  8. 8.
    Nilubol N, Weisbrod AB, Weinstein LS, Simonds WF, Jensen RT, Phan GQ, et al. Utility of intraoperative parathyroid hormone monitoring in patients with multiple endocrine neoplasia type 1-associated primary hyperparathyroidism undergoing initial parathyroidectomy. World J Surg. 2013;37(8):1966–72.CrossRefPubMedGoogle Scholar
  9. 9.
    Norman J, Lopez J, Politz D. Abandoning unilateral parathyroidectomy: why we reversed our position after 15,000 parathyroid operations. J Am Coll Surg. 2012;214(3):260–9.CrossRefPubMedGoogle Scholar
  10. 10.
    Fyrsten E, Norlen O, Hessman O, Stalberg P, Hellman P. Long-term surveillance of treated hyperparathyroidism for multiple endocrine neoplasia type 1: recurrence or hypoparathyroidism? World J Surg. 2016;40(3):615–21.CrossRefPubMedGoogle Scholar
  11. 11.
    Hellman P, Skogseid B, Juhlin C, Akerstrom G, Rastad J. Findings and long term results of parathyroid surgery in multiple endocrine neoplasia type 1. World J Surg. 1992;16:718–23.CrossRefPubMedGoogle Scholar
  12. 12.
    Nilubol N, Weinstein LS, Simonds WF, Jensen RT, Marx SJ, Kebebew E. Limited parathyroidectomy in multiple endocrine neoplasia type 1-associated primary hyperparathyroidism: a setup for failure. Ann Surg Oncol. 2016;23(2):416–23.CrossRefPubMedGoogle Scholar
  13. 13.
    Schreinemakers JM, Pieterman CR, Scholten A, Vriens MR, Valk GD, Rinkes IH. The optimal surgical treatment for primary hyperparathyroidism in MEN1 patients: a systematic review. World J Surg. 2011;35(9):1993–2005.CrossRefPubMedGoogle Scholar
  14. 14.
    Versnick M, Popadich A, Sidhu S, Sywak M, Robinson B, Delbridge L. Minimally invasive parathyroidectomy provides a conservative surgical option for multiple endocrine neoplasia type 1-primary hyperparathyroidism. Surgery. 2013;154(1):101–5.CrossRefPubMedGoogle Scholar
  15. 15.
    Edis AJ, van Heerden JA, Scholz DA. Results of subtotal parathyroidectomy for primary chief cell hyperplasia. Surgery. 1979;86(3):462–9.PubMedGoogle Scholar
  16. 16.
    Wells SA Jr, Farndon JR, Dale JK, Leight GS, Dilley WG. Long term evaluation of patients with primary parathyroid hyperplasia managed by total parathyroidectomy and heterotopic autotransplantation. Ann Surg. 1980;192:451–8.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Prinz RA, Gamvros DI, Selly D, Lynn JA. Subtotal parathyroidectomy for primary chief cell hyperplasia in the multiple endocrine neoplasia type I syndrome. Ann Surg. 1981;193:26.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    van Heerden JA, Kent RB, Sizemore GW, Grant CS, ReMine WM. Primary hyperparathyroidism in patients with multiple endocrine neoplasia syndromes: surgical experience. Arch Surg. 1983;118:533–6.CrossRefPubMedGoogle Scholar
  19. 19.
    Malmaeus J, Benson L, Johansson H, Ljunghall S, Rastad J, Akerstrom G, et al. Parathyroid surgery in the multiple endocrine neoplasia type I syndrome: choice of surgical procedure. World J Surg. 1986;10(4):668–72.CrossRefPubMedGoogle Scholar
  20. 20.
    Goretzki PE, Dotzenrath C, Roeher HD. Management of primary hyperparathyroidism caused by multiple gland disease. World J Surg. 1991;15(6):693–7.CrossRefPubMedGoogle Scholar
  21. 21.
    Kraimps JL, Duh Q-Y, Demeure M, Clark OH. Hyperparathyroidism in multiple endocrine neoplasia syndrome. Surgery. 1992;112:1080–8.PubMedGoogle Scholar
  22. 22.
    O’Riordain DS, O’Brien T, Grant CS, Weaver A, Gharib H, van Heerden JA. Surgical management of primary hyperparathyroidism in multiple endocrine neoplasia types 1 and 2. Surgery. 1993;114:1031–9.PubMedGoogle Scholar
  23. 23.
    Hellman P, Skogseid B, Oberg K, Juhlin C, Akerstrom G, Rastad J. Primary and reoperative parathyroid operations in hyperparathyroidism of multiple endocrine neoplasia type 1. Surgery. 1998;124(6):993–9.CrossRefPubMedGoogle Scholar
  24. 24.
    Dotzenrath C, Cupisti K, Goretzki PE, Yang Q, Simon D, Ohmann C, et al. 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. 2001;167(3):173–8.CrossRefPubMedGoogle Scholar
  25. 25.
    Goudet P, Cougard P, Verges B, Murat A, Carnaille B, Calender A, et al. 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. 2001;25(7):886–90.CrossRefPubMedGoogle Scholar
  26. 26.
    Arnalsteen LC, Alesina PF, Quiereux JL, Farrel SG, Patton FN, Carnaille BM, et al. Long-term results of less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1. Surgery. 2002;132(6):1119–24. Discussion 24-5.CrossRefPubMedGoogle Scholar
  27. 27.
    Elaraj DM, Skarulis MC, Libutti SK, Norton JA, Bartlett DL, Pingpank JF, et al. Results of initial operation for hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Surgery. 2003;134(6):858–64. Discussion 64-5.CrossRefPubMedGoogle Scholar
  28. 28.
    Hubbard JG, Sebag F, Maweja S, Henry JF. Subtotal parathyroidectomy as an adequate treatment for primary hyperparathyroidism in multiple endocrine neoplasia type 1. Arch Surg. 2006;141(3):235–9.CrossRefPubMedGoogle Scholar
  29. 29.
    Norton JA, Venzon DJ, Berna MJ, Alexander HR, Fraker DL, Libutti SK, et al. 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. 2008;247(3):501–10.CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Tonelli F, Giudici F, Cavalli T, Brandi ML. Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy. Clinics (Sao Paulo). 2012;67(Suppl 1):155–60.CrossRefGoogle Scholar
  31. 31.
    Tonelli F, Marcucci T, Fratini G, Tommasi MS, Falchetti A, Brandi ML. Is total parathyroidectomy the treatment of choice for hyperparathyroidism in multiple endocrine neoplasia type 1? Ann Surg. 2007;246(6):1075–82.CrossRefPubMedGoogle Scholar
  32. 32.
    Pieterman CR, van Hulsteijn LT, den Heijer M, van der Luijt RB, Bonenkamp JJ, Hermus AR, et al. Primary hyperparathyroidism in MEN1 patients: a cohort study with longterm follow-up on preferred surgical procedure and the relation with genotype. Ann Surg. 2012;255(6):1171–8.CrossRefPubMedGoogle Scholar
  33. 33.
    Jansson S, Tisell LE. Autotransplantation of diseased parathyroid glands into subcutaneous abdominal adipose tissue. Surgery. 1987;101(5):549–56.PubMedGoogle Scholar
  34. 34.
    Cohen MS, Dilley WG, Wells SA Jr, Moley JF, Doherty GM, Sicard GA, et al. Long-term functionality of cryopreserved parathyroid autografts: a 13-year prospective analysis. Surgery. 2005;138(6):1033–40. Discussion 40-1.CrossRefPubMedGoogle Scholar
  35. 35.
    McCulloch P, Taylor I, Sasako M, Lovett B, Griffin D. Randomised trials in surgery: problems and possible solutions. BMJ. 2002;324(7351):1448–51.CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Pollock AV. Surgical evaluation at the crossroads. Br J Surg. 1993;80(8):964–6.CrossRefPubMedGoogle Scholar
  37. 37.
    Solomon MJ, McLeod RS. Should we be performing more randomized controlled trials evaluating surgical operations? Surgery. 1995;118(3):459–67.CrossRefPubMedGoogle Scholar
  38. 38.
    Wells SA Jr. Surgeons and surgical trials-why we must assume a leadership role. Surgery. 2002;132(3):519–20.CrossRefPubMedGoogle Scholar
  39. 39.
    Howick J, Chalmers I, Glasziou P, Greenhalgh T, Heneghan C, Liberati A, Moschetti I, Phillips B, Thornton H. The 2011 Oxford CEBM levels of evidence (introductory document). Oxford: Oxford Centre for Evidence-Based Medicine; 2011. Scholar

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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Surgical Oncology, Department of SurgeryBaylor Scott and White Health, Texas A&M University Health Science CenterTempleUSA

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