The Southern Medical Journal (SMJ) is the official, peer-reviewed journal of the Southern Medical Association. It has a multidisciplinary and inter-professional focus that covers a broad range of topics relevant to physicians and other healthcare specialists.

SMJ // Article

Case Report

Familial Isolated Primary Hyperparathyroidism with Double Adenoma

Authors: Emad Kandil, MD, Haytham H. Alabbas, MD, Ying W. Lum, MD, Anthony P. Tufaro, MD, Dds

Abstract

Familial hyperparathyroidism includes the diagnoses of multiple endocrine neoplasia type 1, type 2A, and familial isolated primary hyperparathyroidism. Familial isolated primary hyperparathyroidism is a rare, distinct form of familial primary hyperparathyroidism, mainly due to four-gland hyperplasia or single-gland adenoma. We describe our success in treating a 24-year-old woman with familial isolated primary hyperparathyroidism with resection of double adenoma, using the guide of intraoperative parathyroid hormone (PTH) monitoring. Familial isolated primary hyperparathyroidism usually presents with four-gland hyperplasia or single-gland adenoma. However, double adenoma should be considered in the differential diagnosis. Using intraoperative parathyroid hormone levels and minimal-access surgery in familial isolated primary hyperparathyroidism may be promising.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1.Cetani F, Pardi E, Ambrogini E, et al. Genetic analyses in familial isolated hyperparathyroidism: implication for clinical assessment and surgical management. Clin Endocrinol (Oxf) 2006;64:146–152.
 
2.Watanabe T, Tsukamoto F, Shimizu T, et al. Familial isolated hyperparathyroidism caused by single adenoma: a distinct entity different from multiple endocrine neoplasia. Endocr J 1998;45:637–646.
 
3.Carling T. Molecular pathology of parathyroid tumors. Trends Endocrinol Metab 2001;12:53–58.
 
4.Marx SJ, Simonds WF, Agarwal SK, et al. Hyperparathyroidism in hereditary syndromes: special expressions and special managements. J Bone Miner Res 2002;17(Suppl 2):N37–N43.
 
5.Simonds WF, James-Newton LA, Agarwal SK, et al. Familial isolated hyperparathyroidism: clinical and genetic characteristics of 36 kindreds. Medicine (Baltimore) 2002;81:1–26.
 
6.Wassif WS, Moniz CF, Friedman E, et al. Familial isolated hyperparathyroidism: a distinct genetic entity with an increased risk of parathyroid cancer. J Clin Endocrinol Metab 1993;77:1485–1489.
 
7.Huang SM, Duh QY, Shaver J, et al. Familial hyperparathyroidism without multiple endocrine neoplasia. World J Surg 1997;21:22–28; discussion 29.
 
8.Campusano C, Oestreicher E, Arteaga E, et al. Familial and isolated primary hyperparathyroidism. Case report [in Spanish]. Rev Med Chil 2001;129:1311–1314.
 
9.Yamashita K, Suzuki S, Yumita W, et al. A case of familial isolated hyperparathyroidism with ectopic parathyroid cancer. Endocr J 2001;48:453–458.
 
10.Szabo E, Hellman P, Lundgren E, et al. Parathyroidectomy in familial hypercalcemia with clinical characteristics of primary hyperparathyroidism and familial hypocalciuric hypercalcemia. Surgery 2002;131:257–263.
 
11.Bergwitz C, Bremer B, Soudah B, et al. Familial isolated parathyroid adenoma in a consanguineous family. J Endocrinol Invest 2001;24:349–355.
 
12.Abe H, Tani T, Naito H, et al. Familial hyperparathyroidism: report of a case. Surg Today 1999;29:63–66.
 
13.Barry MK, van Heerden JA, Grant CS, et al. Is familial hyperparathyroidism a unique disease? Surgery 1997;122:1028–1033.
 
14.Tonelli F, Marcucci T, Fratini G, et al. Is total parathyroidectomy the treatment of choice for hyperparathyroidism in multiple endocrine neoplasia type 1? Ann Surg 2007;246:1075–1082.
 
15.Carneiro DM, Irvin GL III, Inabnet WB. Limited versus radical parathyroidectomy in familial isolated primary hyperparathyroidism. Surgery 2002;132:1050–1054; discussion 1055.
 
16.Carneiro DM, Irvin GL III. Late parathyroid function after successful parathyroidectomy guided by intraoperative hormone assay (QPTH) compared with the standard bilateral neck exploration. Surgery 2000;128:925–929; discussion 935–936.
 
17.Garner SC, Leight GS Jr. Initial experience with intraoperative PTH determinations in the surgical management of 130 consecutive cases of primary hyperparathyroidism. Surgery 1999;126:1132–1137; discussion 1137–1138.
 
18.Carling T, Udelsman R. Parathyroid surgery in familial hyperparathyroid disorders. J Intern Med 2005;257:27–37.
 
19.van Heerden JA, Grant CS. Surgical treatment of primary hyperparathyroidism: an institutional perspective. World J Surg 1991;15:688–692.
 
20.Yao K, Singer FR, Roth SI, et al. Weight of normal parathyroid glands in patients with parathyroid adenomas. J Clin Endocrinol Metab 2004;89:3208–3213.