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Surgical approach to the patient with familial hyperparathyroidism

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Opinion statement

Familial hyperparathyroidism encompasses the diagnoses of multiple endocrine neoplasia (MEN) type 1, MEN type 2A, and familial isolated primary hyperparathyroidism. All patients should undergo bilateral neck exploration and identification of all four or more parathyroid glands to evaluate for gross abnormalities. MEN-1 patients should have subtotal parathyroidectomy and cervical thymectomy because this operation achieves an appropriate balance between optimizing the potential for cure yet minimizing the risk of permanent hypocalcemia. However, MEN-2A patients may best be treated by selective resection of abnormal parathyroid glands, although some experts recommend a total parathyroidectomy and autotransplantation in the forearm. Familial isolated hyperparathyroidism is a rare disorder, and authors have described success in treatment with subtotal parathyroidectomy or limited adenoma resections. Some patients with familial isolated hyperparathyroidism also have jaw tumors, and members of these families are more likely to have parathyroid carcinoma. Concurrent cryopreservation of parathyroid tissue for all of these disorders is recommended if there is any concern for possible permanent hypoparathyroidism.

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References and Recommended Reading

  1. Kouvaraki MA, Lee JE, Shapiro SE, et al.: Genotypephenotype analysis in multiple endocrine neoplasia type 1. Arch Surg 2002, 137:641–647.

    Article  PubMed  Google Scholar 

  2. Langer P, Wild A, Hall A, et al.: Prevalence of multiple endocrine neoplasia type 1 in young patients with apparently sporadic primary hyperparathyroidism or pancreaticoduodenal endocrine tumours. Br J Surg 2003, 90:1599–1603.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  4. 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, 86:5658–5671. International consensus statement on the treatment of MEN type 1 and 2.

    Article  PubMed  CAS  Google Scholar 

  5. Goudet P, Cougard P, Verges B, et al.: Hyperparathyroidism in multiple endocrine neoplasia type I: surgical trends and results of a 256-patient series from Groupe d’Etude des Néoplasies Endocriniennes Multiples Study Group. World J Surg 2001, 25:886–890. Largest cohort published on MEN-1 patients with hyperparathyroidism.

    Article  PubMed  CAS  Google Scholar 

  6. Arnalsteen LC, Alesina PF, Quiereux JL, et al.: Long-term results of less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1. Surgery 2002, 132:1119–1125.

    Article  PubMed  Google Scholar 

  7. 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, 167:173–178.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  9. Elaraj DM, Skarulis MC, Libutti SK, et al.: Results of initial operation for hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Surgery 2003, 134:858–865.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  12. Sierra M, Gibelin H, Kraimps JL: Familial hyperparathyroidism in multiple endocrine neoplasia syndromes. In Textbook of Endocrine Surgery, edn 2. Edited by Clark OH, Duh QY, Kebebew E. Philadelphia: Elsevier; 2005:493–501.

    Google Scholar 

  13. Gibril F, Chen YJ, Schrump DS, et al.: Prospective study of thymic carcinoids in patients with multiple endocrine neoplasia type 1. J Clin Enocrinol Metab 2003, 88:1066–1081. A prospective analysis of MEN-1 patients and their risk of developing thymic carcinoid.

    Article  CAS  Google Scholar 

  14. Teh BT, Zedenius J, Kytola S, et al.: Thymic carcinoids in multiple endocrine neoplasia type 1. Ann Surg 1998, 228:99–105.

    Article  PubMed  CAS  Google Scholar 

  15. Ferolla P, Falchetti A, Filosso P, et al.: Thymic neuroendocrine carcinoma (carcinoid) in multiple endocrine neoplasia type 1 syndrome: the Italian series. J Clin Endocrinol Metab 2005, 90:2603–2609.

    Article  PubMed  CAS  Google Scholar 

  16. Burgess JR, Giles N, Shepherd JJ: Malignant thymic carcinoid is not prevented by transcervical thymectomy in multiple endocrine neoplasia type 1. Clin Endocrinol 2001, 55:689–693.

    Article  CAS  Google Scholar 

  17. Kraimps JL, Denizot A, Carnaille B, et al.: Primary hyperparathyroidism in multiple endocrine neoplasia type Iia: retrospective French multicentric study. World J Surg 1996, 20:808–813.

    Article  PubMed  CAS  Google Scholar 

  18. Raue F, Kraimps JL, Dralle H, et al.: Primary hyperparathyroidism in multiple endocrine neoplasia type 2A. J Int Med 1995, 238:369–371. Largest cohort on MEN-2A and hyperparathyroidism.

    Article  CAS  Google Scholar 

  19. Herfarth KK, Bartsch F, Doherty GM, et al.: Surgical management of hyperparathyroidism in patients with multiple endocrine neoplasia type 2A. Surgery 1996, 120:966–974.

    Article  PubMed  CAS  Google Scholar 

  20. Decker RA, Geiger JD, Cox CE, et al.: Prophylactic surgery for multiple endocrine neoplasia type Iia after genetic diagnosis: is parathyroid transplantation indicated. World J Surg 1996, 20:814–821.

    Article  PubMed  CAS  Google Scholar 

  21. Warner J, Epstein M, Sweet A, et al.: Genetic testing in familial isolated hyperparathyroidism: unexpected results and their implications. J Med Genet 2004, 41:155–160.

    Article  PubMed  CAS  Google Scholar 

  22. Pannett AAJ, Kennedy AM, Turner JJO, et al.: Multiple endocrine neoplasia type 1 (MEN1) germline mutations in familial isolated primary hyperparathyroidism. Clin Endocrinol 2003, 58:639–646.

    Article  CAS  Google Scholar 

  23. Haven CJ, Wong FK, van Dam EW, et al.: A genotypic and histopathological study of a large Dutch kindred with hyperparathyroidism-jaw tumor syndrome. J Clin Endocrinol Metab 2000, 85:1449–1454.

    Article  PubMed  CAS  Google Scholar 

  24. Cavaco BM, Barros L, Pannett AA, et al.: The hyperparathyroidism-jaw tumour syndrome in a Portuguese kindred. Q J Med 2001, 94:213–222.

    CAS  Google Scholar 

  25. Huang SM: Familial hyperparathyroidism. In Textbook of Endocrine Surgery, edn 2. Edited by Clark OH, Duh QY, Kebebew E. Philadelphia: Elsevier; 2005:493–501.

    Google Scholar 

  26. Szabo E, Hellman P, Lundgren E, et al.: Parathyroidectomy in familial hypercalcemia with clinical characteristics of primary hyperparathyroidism and familial hypocalciuria hypercalcemia. Surgery 2002, 131:257–263.

    Article  PubMed  Google Scholar 

  27. Khan AA, Bilezikian JP, Kung AW, et al.: Alendronate in primary hyperparathyroidism: a double-blind, randomized, placebo-controlled trail. J Clin Endocrinol Metab 2004, 89:3319–3325.

    Article  PubMed  CAS  Google Scholar 

  28. Chow CC, Chan WB, Li JK, et al.: Oral alendronate increases bone mineral density in postmenopausal women with primary hyperparathyroidism. J Clin Endocrinol Metab 2003, 88:581–587.

    Article  PubMed  CAS  Google Scholar 

  29. Parker CR, Blackwell PJ, Fairbairn KJ, Hosking DJ: Alendronate in the treatment of primary hyperparathyroid-related osteoporosis: a 2-year study. J Clin Endocrinol Metab 2002, 87:4482–4489.

    Article  PubMed  CAS  Google Scholar 

  30. Horiuchi T, Onouchi T, Inoue J, et al.: A strategy for the management of elderly women with primary hyperparathyroidism: a comparison of etidronate therapy with parathyroidectomy. Gerontology 2002, 48:103–108.

    Article  PubMed  CAS  Google Scholar 

  31. Carniero DM, Irvin GL, Inabnet WB: Limited versus radical parathyroidectomy in familial isolated primary hyperparathyroidism. Surgery 2002, 132:1050–1055.

    Article  Google Scholar 

  32. Tonelli F, Spini S, Tommasi M, et al.: Intraoperative parathormone measurements in patients with multiple endocrine neoplasia type 1 syndrome and hyperparathyroidism. World J Surg 2000, 24:556–563.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Surgery, Kaiser Permanente Los Angeles Medical Center, 4760 Sunset Boulevard, 90027, Los Angeles, CA, USA

    Philip I. Haigh MD, MSc, FRCSC, FACS

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  1. Lindi H. VanderWalde MD
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  2. Philip I. Haigh MD, MSc, FRCSC, FACS
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VanderWalde, L.H., Haigh, P.I. Surgical approach to the patient with familial hyperparathyroidism. Curr. Treat. Options in Oncol. 7, 326–333 (2006). https://doi.org/10.1007/s11864-006-0042-5

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