World Journal of Endocrine Surgery

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VOLUME 2 , ISSUE 3 ( September-December, 2010 ) > List of Articles


Severe Hypercalcemia due to Primary Hyperparathyroidism with MEN 2A

Geoffrey B Thompson, Benzon M Dy, Bianca Vazquez, Peter J Tebben, Seema Kumar

Citation Information : Thompson GB, Dy BM, Vazquez B, Tebben PJ, Kumar S. Severe Hypercalcemia due to Primary Hyperparathyroidism with MEN 2A. World J Endoc Surg 2010; 2 (3):131-133.

DOI: 10.5005/jp-journals-10002-1037

Published Online: 01-12-2010

Copyright Statement:  Copyright © 2010; The Author(s).



Severe hypercalcemia due to primary hyperparathyroidism (PHPT) is rare in the setting of MEN 2A.

Materials and methods

Two patients with MEN 2A and severe hypercalcemia were identified recently. Their clinical presentation, evaluation, surgical management and outcomes are reviewed.


Two patients with MEN 2A were identified with severe hypercalcemia secondary to a parathyroid adenoma. Calcium levels were elevated to 12.7 mg/dL and 15.1 mg/dL, respectively (normal range = 8.9-10.1 mg/dL). In each case, a single parathyroid adenoma was identified and surgically excised with normalization of parathyroid and calcium levels postoperatively. Clinical manifestations at the time of diagnosis included constipation, polyuria, hypercalciuria, and decreased bone mineral density.


Severe elevation of serum calcium is a rare presentation of PHPT in MEN2A. The differential diagnosis should include parathyroid adenoma, hyperplasia and parathyroid carcinoma. Early surgical management is essential in the treatment of hyperparathyroidism with severe hypercalcemia to prevent further complications.

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  1. Current perspectives on the diagnosis and management of patients with multiple endocrine neoplasia type 2 syndromes. Endocrino Metab Clin North Am 1994;23(1):215-28.
  2. Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2a. Nature 1993;363(6428):458-60.
  3. Guidelines for diagnosis and therapy of MEN type1 and type 2. J Clin Endocrinol Metab 2001;86(12):5658-71.
  4. Parathyroid surgery in familial hyperparathyroid disorders. J Intern Med 2005;257(1):27-37.
  5. Clinical manifestations of primary hyperparathyroidism before and after parathyroidectomy: A case-control study. Ann Surg 1995;222(3):402-14.
  6. The influence of surgery on the risk of death in patients with primary hyperparathyroidism. World J Surg 1991;15(3):399-407.
  7. Primary hyperparathyroidism in multiple endocrine neoplasia type IIa: Retrospective French multicentric study. Groupe d'Etude des Tumeurs á Calcitonine (GETC, French Calcitonin Tumors Study Group), French Association of Endocrine Surgeons. World J Surg 1996;20(7):808-12.
  8. Primary hyperparathyroidism in multiple endocrine neoplasia type 2A. J Intern Med 1995;238(4):369-73.
  9. Surgical management of primary hyperparathyroidism in multiple endocrine neoplasia types 1 and 2. Surgery 1993;114(6): 1031-37.
  10. The NIH criteria for parathyroidectomy in asymptomatic primary hyperparathyroidism: Are they too limited? Ann Surg 2004;239(4):528-35.
  11. Primary hyperparathyroidism: Can parathyroid carcinoma be anticipated on clinical and biochemical grounds? Report of nine cases and review of the literature. Ann Surg Oncol 2005;12(7):526-32.
  12. Biletikian JP. Parathyroid carcinoma. J Bone Miner Res 2008;23(12):1869-80.
  13. Clark OH. Parathyroid carcinoma versus parathyroid adenoma in patients with profound hypercalcemia. Surgery 1987;101(6):649-60.
  14. Reoperative surgery for primary hyperparathyroidism. Br J Surg 2009;96(7):699-701.
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