Persistent Primary Hyperparathyroidism Secondary to an Ectopic Mediastinal Adenoma in a Young Adult

A Case Report

Authors

DOI:

https://doi.org/10.15605/jafes.038.02.16

Keywords:

hyperparathyroidism, reoperation, hypercalcemia, ectopic parathyroid adenoma

Abstract

Primary hyperparathyroidism commonly affects elderly women. When present in the young population, it is usually asymptomatic, most frequently due to a parathyroid adenoma and the definitive management is surgical excision.
Uncommonly, 5-10% of patients fail to achieve long-term cure after initial parathyroidectomy and 6-16% of them is due to
an ectopic parathyroid adenoma that will require focused diagnostic and surgical approaches.

We report a 21-year-old male who had bilateral thigh pain. Work-up revealed bilateral femoral fractures, brown tumors on the arms and multiple lytic lesions on the skull. Serum studies showed hypercalcemia (1.83 mmol/L), elevated parathyroid hormone [(PTH) 2025.10 pg/mL], elevated alkaline phosphatase (830 U/L), normal phosphorus (0.92 mmol/L) and low vitamin D levels (18.50 ng/mL). Bone densitometry showed osteoporotic findings. Sestamibi scan showed uptake on the left superior mediastinal region consistent with an ectopic parathyroid adenoma. Vitamin D supplementation was started pre-operatively. Patient underwent parathyroidectomy with neck exploration; however, the pathologic adenoma was not visualized and PTH levels remained elevated post-operatively. Chest computed tomography with intravenous contrast was performed revealing a mediastinal location of the adenoma. A repeat parathyroidectomy was done, with successful identification of the adenoma resulting in a significant drop in PTH and calcium levels. Patient experienced hungry bone syndrome post-operatively and was managed with calcium and magnesium supplementation. A high index of suspicion for an ectopic adenoma is warranted for patients presenting with hypercalcemia and secondary osteoporosis if there is persistent PTH elevation after initial surgical intervention. Adequate follow-up and monitoring is also needed starting immediately in the post-operative period to manage possible complications such as hungry bone syndrome.

Downloads

Download data is not yet available.

Author Biographies

Karl Homer, Chinese General Hospital and Medical Center, Manila, Philippines

Section of Endocrinology, Diabetes and Metabolism

Rebecca Alba, Chinese General Hospital and Medical Center, Manila, Philippines

Section of Endocrinology, Diabetes  and Metabolism

References

Kanis JA, Oden A, Johnell O, Jonsson B, de Laet C, Dawson A. The burden of osteoporotic fractures: A method for setting intervention thresholds. Osteoporos Int. 2001;12(5):417-27. https://pubmed.ncbi.nlm.nih.gov/ 11444092. https://doi.org/10.1007/s001980170112.

Mazotas IG, Yen TWF, Doffek K, et al. Persistent/recurrent primary hyperparathyroidism: Does the number of abnormal glands play a role? J Surg Res. 2020;246:335-41. https://pubmed.ncbi.nlm.nih.gov/31635835. https://doi.org/10.1016/j.jss.2019.08.007.

Smith G, Prinz RA. Chapter 9. Persistent and recurrent hyperparathyroidism. McGraw-Hill Manual: Endocrine Surgery; 2010. https://accesssurgery.mhmedical.com/content.aspx?bookid=

&sectionid=40161242.

Roy M, Mazeh H, Chen H, Sippel RS. Incidence and localization of ectopic parathyroid adenomas in previously unexplored patients. World J Surg. 2013;37(1):102-6. https://pubmed.ncbi.nlm.nih.gov/ 22968537. https://doi.org/10.1007/s00268-012-1773-z.

Bilezikian JP. Primary hyperparathyroidism. J Clin Endocrinol Metab. 2018;103(11):3993-4004. https://pubmed.ncbi.nlm.nih.gov/ 30060226. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6182311. https://doi.org/10.1210/jc.2018-01225.

Lou I, Schneider DF, Sippel RS, Chen H, Elfenbein DM. The changing pattern of diagnosing primary hyperparathyroidism in young patients. Am J Surg. 2017;213(1):146-50. https://pubmed.ncbi.nlm.nih.gov/ 27392754. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5154878.

https://doi.org/10.1016/j.amjsurg.2016.03.019.

Walls J, Ratcliffe WA, Howell A, Bundred NJ. Parathyroid hormone and parathyroid hormone-related protein in the investigation of hypercalcaemia in two hospital populations. Clin Endocrinol (Oxf). 1994;41(4):407-13. https://pubmed.ncbi.nlm.nih.gov/7955450. https://doi.org/10.1111/j.1365-2265.1994.tb02569.x.

Walker MD, Bilezikian JP. Primary hyperparathyroidism. [Updated

Apr 19]. In: Feingold KR, Anawalt B, Blackman MR, et al. (eds).

Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000.

https://www.ncbi.nlm.nih.gov/books/NBK278923/.

Chang BA, Sharma A, Anderson DW. Ectopic parathyroid adenoma in the soft palate: A case report. J of Otolaryngol Head Neck Surg. 2016;45(1):53. https://pubmed.ncbi.nlm.nih.gov/ 27756384. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5069995. https://doi.org/10.1186/s40463-016-0165-z.

Yen TW, Wang TS, Doffek KM, Krzywda EA, Wilson SD. Reoperative

parathyroidectomy: An algorithm for imaging and monitoring of intraoperative parathyroid hormone levels that results in a successful focused approach. Surgery. 2008;144(4):611-9. https://pubmed.ncbi.nlm.nih.gov/18847646.

https://doi.org/10.1016/j.surg.2008.06.017.

Zarebczan B, Chen H. Influence of surgical volume on operative failures for hyperparathyroidism. Adv Surg. 2011;45:237-48.

Taubman ML, Goldfarb M, Lew JI. Role of SPECT and SPECT/CT in the surgical treatment of primary hyperparathyroidism. Int J Mol Imaging. 2011;2011:141593. https://pubmed.ncbi.nlm.nih.gov/ 21776381. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3139123. https://doi.org/10.1155/2011/141593.

Jaskowiak NT, Sugg SL, Helke J, Koka MR, Kaplan EL. Pitfalls of intraoperative quick parathyroid hormone monitoring and gamma probe localization in surgery for primary hyperparathyroidism. Arch Surg. 2002;137(6):659–69. https://pubmed.ncbi.nlm.nih.gov/ 12049536. https://doi.org/10.1001/archsurg.137.6.659.

Naik AH, Wani MA, Wani KA, Laway BA, Malik AA, Shah ZA. Intraoperative parathyroid hormone monitoring in guiding adequate parathyroidectomy. Indian J Endocrinol Metabol. 2018;22(3):410-6. https://pubmed.ncbi.nlm.nih.gov/30090736. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6063190. https://doi.org/10.4103/ijem.IJEM_678_17.

Li J, Vasilyeva E, Hiebert J, Britton H, Walker B, Wiseman SM. Limited clinical utility of intraoperative frozen section during parathyroidectomy for treatment of primary hyperparathyroidism. Am J Surg. 2019;217(5):893-8. https://pubmed.ncbi.nlm.nih.gov/30771863. https://doi.org/10.1016/j.amjsurg.2019.01.032.

Bollerslev J, Rejnmark L, Zahn A, et al. European expert consensus on

practical management of specific aspects of parathyroid disorders in adults and in pregnancy: Recommendations of the ESE educational program of parathyroid disorders. Eur J Endocrinol. 2022;186(2):R33-63. https://pubmed.ncbi.nlm.nih.gov/34863037. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8789028. https://doi.org/10.1530/EJE-21-1044.

Anwar F, Abraham J, Nakshabandi A, Lee E. Treatment of hypocalcemia in hungry bone syndrome: A case report. Int J Surg Case Rep. 2018;51:335-9. https://pubmed.ncbi.nlm.nih.gov/30245357. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6153392. https://doi.org/10.1016/j.ijscr.2018.08.011.

Cartwright C, Anastasopoulou C. Hungry bone syndrome. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022. https://www.ncbi.nlm.nih.gov/books/NBK549880/.

Ho LY, Wong PN, Sin HK, et al. Risk factors and clinical course of the hungry bone syndrome after total parathyroidectomy in dialysis patients with secondary hyperparathyroidism. BMC Nephrol. 2017;18(1):12. https://pubmed.ncbi.nlm.nih.gov/28073343. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5223390. https://doi.org/10.1186/s12882-016-0421-5.

Downloads

Published

2023-07-15

How to Cite

Karl Homer, & Alba, R. (2023). Persistent Primary Hyperparathyroidism Secondary to an Ectopic Mediastinal Adenoma in a Young Adult: A Case Report. Journal of the ASEAN Federation of Endocrine Societies, 38(2), 145–148. https://doi.org/10.15605/jafes.038.02.16

Issue

Section

Case Reports