Annals of Surgical Oncology

, Volume 13, Issue 1, pp 103–109 | Cite as

Primary Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Individualized Management With Low Recurrence Rates

  • Chen-Hsen Lee
  • Ling-Ming Tseng
  • Jui-Yu Chen
  • Hsin-Yun Hsiao
  • An-Hang Yang
Original Article

Abstract

Background

To evaluate the outcomes in different surgical modalities for primary hyperparathyroidism in multiple endocrine neoplasia type 1 (MEN1) patients, intraoperative findings from a single surgeon were studied to investigate a potentially improved modality of parathyroidectomy (PTx).

Methods

All 22 patients had PTx by a single surgeon in the past 21 years. Three modalities of PTx were used, depending on the operative findings, after all parathyroids and the thymus were identified. If fewer than three glands were enlarged, selective removal of the enlarged glands with or without biopsy of a normal-appearing gland was performed (selective PTx); if all glands were enlarged, either a subtotal PTx leaving a 50-mg remnant in situ or a total PTx with autotransplantation (TPTx + AT) was performed.

Results

There were 7 men and 15 women, aged 22 to 67 years (average, 43 years). Sixteen had familial and six had sporadic MEN1. They underwent 23 operations, including 11 selective PTx, 6 subtotal PTx, and 6 TPTx + AT. On follow-up for 1 to 19 years, only one patient (4.6%) had recurrent hyperparathyroidism 5.5 years after subtotal PTx. Others had either normocalcemia (n = 14; 63.6%) or hypocalcemia (n = 7; 31.8%). Those who had either a subtotal PTx or TPTx + AT had a significantly higher rate of postoperative hypocalcemia than those who had a selective PTx (9.9% vs. 54.5%; P = .032; Fisher’s exact test).

Conclusions

Primary hyperparathyroidism in our MEN1 patients was less aggressive than that reported in the literature. Selective PTx according to the intraoperative findings achieved optimal outcomes.

Keywords

Multiple endocrine neoplasia type 1 Parathyroidectomy Recurrence Hypocalcemia 

References

  1. 1.
    Brandi ML, Gagel RF, Angeli A, et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 2001;86:5658–71CrossRefPubMedGoogle Scholar
  2. 2.
    Marx SJ, Menczel J, Campbell G, et al. Heterogenous size of the parathyroid glands in familial multiple endocrine neoplasia type 1. Clin Endocrinol 1991;35:521–6Google Scholar
  3. 3.
    Malmaeus J, Benson L, Johansson H, et al. Parathyroid surgery in the multiple endocrine neoplasia type I syndrome: choice of surgical procedure. World J Surg 1986;10:668–72CrossRefPubMedGoogle Scholar
  4. 4.
    Berger AC, Alexander HR. Management of hyperparathyroidism in multiple endocrine neoplasia type 1. In: Doherty GM, Skogseid B, (eds). Surgical Endocrinology. Philadelphia: Lippincott Williams & Wilkins, 2001:495–510Google Scholar
  5. 5.
    Rizzoli R, Green J III, Marx SJ. Primary hyperparathyroidism in familial multiple endocrine neoplasia type I. Long term follow-up of serum calcium levels after parathyroidectomy. Am J Med 1985;78:467–74CrossRefPubMedGoogle Scholar
  6. 6.
    Samaan NA, Ouais S, Ordonez NG, et al. Multiple endocrine neoplasia type I. Clinical, laboratory findings, and management in five families. Cancer 1989;64:741–52PubMedGoogle Scholar
  7. 7.
    Wells SA Jr, Farndon JR, Dale JK, et al. Long-term evaluation of patients with primary parathyroid hyperplasia managed by total parathyroidectomy and heterotopic autotransplantation. Ann Surg 1980;192:451–8PubMedGoogle Scholar
  8. 8.
    Burgess JR, David R, Parameswaran V, et al. The outcome of subtotal parathyroidectomy for the treatment of hyperparathyroidism in multiple endocrine neoplasia type 1. Arch Surg 1998;133:126–9PubMedGoogle Scholar
  9. 9.
    Brandi ML, Marx SJ, Aurbach GD, et al. Familial multiple endocrine neoplasia type 1: a new look at pathophysiology. Endocr Rev 1987;8:391–405PubMedCrossRefGoogle Scholar
  10. 10.
    O’Riordain DS, O’Brien T, Grant CS, et al. Surgical management of primary hyperparathyroidism in multiple endocrine neoplasia types 1 and 2. Surgery 1993;114:1031–9PubMedGoogle Scholar
  11. 11.
    Thomposon NW. The surgical management of hyperparathyroidism and endocrine disease of the pancreas in the multiple endocrine neoplasia type 1 patient. J Intern Med 1995;238:269–80Google Scholar
  12. 12.
    Dotzenrath C, Cupisti K, Goretzki PE, et al. Long-term biochemical results after operative treatment of primary hyperparathyroidism associated with multiple endocrine neoplasia type I and IIa: is a more or less extended operation essential? Eur J Surg 2001;167:173–8PubMedGoogle Scholar
  13. 13.
    van Heerden JA, Kent RB III, Sizemore GW, et al. Primary hyperparathyroidism in patients with multiple endocrine neoplasia syndromes. Surgical experience. Arch Surg 1983;118:533–6PubMedGoogle Scholar
  14. 14.
    Hellman P, Skogseid B, Juhlin C, et al. Findings and long-term results of parathyroid surgery in multiple endocrine neoplasia type 1. World J Surg 1992;16:718–23CrossRefPubMedGoogle Scholar
  15. 15.
    Brandi ML, Aurbach GD, Fitzpatrick LA, et al. Parathyroid mitogenic activity in plasma from patients with familial multiple endocrine neoplasia type 1. N Engl J Med 1986;314:1287–93PubMedCrossRefGoogle Scholar
  16. 16.
    Zimering MB, Katsumata N, Sato Y, et al. Increased basic fibroblast growth factor in plasma from multiple endocrine neoplasia type 1: relation to pituitary tumor. J Clin Endocrinol Metab 1993;76:1182–7CrossRefPubMedGoogle Scholar
  17. 17.
    Raisz LG. Regulation by calcium of parathyroid growth and secretion in vitro. Nature 1963;197:1115–6PubMedGoogle Scholar
  18. 18.
    Nichol PF, Starling JR, Mack E, et al. Long-term follow-up of patients with tertiary hyperparathyroidism treated by resection of a single or double adenoma. Ann Surg 2002;235:673–80CrossRefPubMedGoogle Scholar
  19. 19.
    Kivlen MH, Bartlett DL, Libutti SK, et al. Reoperation for hyperparathyroidism in multiple endocrine neoplasia type 1. Surgery 2001;130:991–8CrossRefPubMedGoogle Scholar
  20. 20.
    Lemmens I, van de Ven WJ, Kas K, et al. Identification of the multiple endocrine neoplasia type 1 (MEN1) gene. The European Consortium on MEN1. Hum Mol Genet 1997;6:1177–83CrossRefPubMedGoogle Scholar
  21. 21.
    Chandrasekharappa SC, Guru SC, Manickam P, et al. Positional cloning of the gene for multiple endocrine neoplasia-type 1. Science 1997;276:404–7CrossRefPubMedGoogle Scholar
  22. 22.
    Agarwal SK, Kester MB, Debelenko LV, et al. Germline mutations of the MEN1 gene in family multiple endocrine neoplasia type 1 and related states. Hum Mol Genet 1997;6:1169–75CrossRefPubMedGoogle Scholar
  23. 23.
    Bassett JH, Forbes SA, Pannett AA, et al. Characterization of mutations in patients with multiple endocrine neoplasia type 1. Am J Hum Genet 1998;62:232–44CrossRefPubMedGoogle Scholar
  24. 24.
    Kraimps JL, Duh QY, Demeure M, et al. Hyperparathyroidism in multiple endocrine neoplasia syndrome. Surgery 1992;112:1080–8PubMedGoogle Scholar
  25. 25.
    Proye C, Carnaille B, Quievreux JL, et al. Late outcome of 304 consecutive patients with multiple gland enlargement in primary hyperparathyroidism treated by conservative surgery. World J Surg 1998;22:526–30PubMedGoogle Scholar
  26. 26.
    Teh BT, McArdle J, Parameswaran V, et al. Sporadic primary hyperparathyroidism in the setting of multiple endocrine neoplasia type 1. Arch Surg 1996;131:1230–2PubMedGoogle Scholar
  27. 27.
    Tonelli F, Spini S, Tommasi M, et al. Intraoperative parathormone measurement in patients with multiple endocrine neoplasia type I syndrome and hyperparathyroidism. World J Surg 2000;24:556–62CrossRefPubMedGoogle Scholar
  28. 28.
    Gauger PG, Agarwal G, England BG, et al. Intraoperative parathyroid hormone monitoring fails to detect double parathyroid adenomas: a 2-institution experience. Surgery 2001;130:1005–10CrossRefPubMedGoogle Scholar
  29. 29.
    Miura D, Wada N, Arici C, et al. Does intraoperative quick parathyroid hormone assay improve the results of parathyroidectomy? World J Surg 2002;26:926–30CrossRefPubMedGoogle Scholar
  30. 30.
    Proye CAG, Coropoulos A, Franz C, et al. Usefulness and limits of quick intraoperative measurements of intact(1-84) parathyroid hormone in the surgical management of hyperparathyroidism: sequential measurements in patients with multiple gland disease. Surgery 1991;110:1035–42PubMedGoogle Scholar
  31. 31.
    Herfarth KK, Bartsch D, Doherty GM, et al. Surgical management of hyperparathyroidism in patients with multiple endocrine neoplasia type 2A. Surgery 1996;120:966–74PubMedGoogle Scholar

Copyright information

© The Society of Surgical Oncology, Inc. 2006

Authors and Affiliations

  • Chen-Hsen Lee
    • 1
    • 4
  • Ling-Ming Tseng
    • 1
    • 4
  • Jui-Yu Chen
    • 1
    • 4
  • Hsin-Yun Hsiao
    • 2
    • 4
  • An-Hang Yang
    • 3
    • 4
  1. 1.Department of SurgeryTaipei-Veterans General HospitalTaipeiTaiwan
  2. 2.Department of Insternal MedicineTaipei-Veterans General HospitalTaipeiTaiwan
  3. 3.Department of PathologyTaipei-Veterans General HospitalTaipeiTaiwan
  4. 4.School of MedicineNational Yang-Ming UniveristyTaipeiTaiwan

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