Diagnosis
Parathyroid cancer
A rare malignancy of the parathyroid glands.
Etiology
Cause [wir]
Unknown
Spontaneous DNA-mutation in a tumor suppressor gene --> Unregulated growth
Congenital genetic mutation: MEN syndrome
Pathophysiology [wir]
The parathyroid chief cells are responsible for producing parathyroid hormone
Most parathyroid carcinomas are functional --> secrete parathyroid hormone (PTH)
The clinical presentation:
Mimic hyperparathyroidism (high PTH level) --> elevated calcium
Palpable neck mass
May invade the trachea, strap muscles, and recurrent laryngeal nerve
Metastases are rare: lungs, bone, mediastinal nodes, liver, and kidney
Risk factors [4gv]
Multiple endocrine neoplasia type 1
Autosomal dominant familial isolated hyperparathyroidism
Radiation exposure
Epidemiology
Incidence per 100.000 [337][s6m][re9][qnf][bpt][9tu]
Symptoms & findings
Symptoms
Confusion, Constipation, Depression, Dysphonia, Fatigue, Hoarseness, Polydipsia, Polyuria, Weakness
Clinical findings
Elevated hCG, Elevated PTH, Hypercalcemia, Pathologic fracture
Anamneses
None listed.
Localized findings
Approach
Blood test: Calcium, Phosphate, PTH, creatinine, hCG [kdm][c42]
Urine: Ca
Neck ultrasound
Parathyroid scintigraphy
PET CT
DNA testing for CDC73 mutations
Bone mineral density
Treatment
Hypercalcemia: [4gv][c42]
Intravenous fluids
Diuretics
Antiresorptive agents: bisphosphonates, gallium, or mithramycin
Calcimimetic agents
Parathyroidectomy [kdm][re9]
Postoperative radiotherapy [rts]
Chemotherapy [qnf]
Differential diagnoses
Familial hypocalciuric hypercalcemia, Hypercalcemia, Hyperparathyroidism, Paraneoplastic syndrome, Parathyroid adenoma, Thyroid cancer, Toxic nodular goiter
References
[1] Byrd C, Kashyap S, Kwartowitz G. Parathyroid Cancer. [Updated 2023 Jul 24]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK519038/
[2] PDQ Adult Treatment Editorial Board. Parathyroid Cancer Treatment: Health Professional Version. 2024 Jul 5: https://www.ncbi.nlm.nih.gov/books/NBK65795/
[3] Schaapveld M, Jorna FH, Aben KK, Haak HR, Plukker JT, Links TP. Incidence and prognosis of parathyroid gland carcinoma: a population-based study in The Netherlands estimating the preoperative diagnosis. Am J Surg. 2011 Nov;202(5):590-7.
[4] van der Zwan JM, Mallone S, van Dijk B, Bielska-Lasota M, Otter R, Foschi R, Baudin E, Links TP; RARECARE WG. Carcinoma of endocrine organs: results of the RARECARE project. Eur J Cancer. 2012 Sep;48(13):1923-31.
[5] Lee PK, Jarosek SL, Virnig BA, Evasovich M, Tuttle TM. Trends in the incidence and treatment of parathyroid cancer in the United States. Cancer. 2007 May 1;109(9):1736-41.
[6] Ullah A, Khan J, Waheed A, Sharma N, Pryor EK, Stumpe TR, Velasquez Zarate L, Cason FD, Kumar S, Misra S, Kavuri S, Mesa H, Roper N, Foroutan S, Karki NR, Del Rivero J, Simonds WF, Karim NA. Parathyroid Carcinoma: Incidence, Survival Analysis, and Management: A Study from the SEER Database and Insights into Future Therapeutic Perspectives. Cancers (Basel). 2022 Mar 10;14(6):1426.
[7] Correa P, Chen VW. Endocrine gland cancer. Cancer. 1995 Jan 1;75(1 Suppl):338-52.
[8] Hundahl SA, Fleming ID, Fremgen AM, Menck HR. Two hundred eighty-six cases of parathyroid carcinoma treated in the U.S. between 1985-1995: a National Cancer Data Base Report. The American College of Surgeons Commission on Cancer and the American Cancer Society. Cancer. 1999 Aug 1;86(3):538-44.
[9] Obara T, Fujimoto Y. Diagnosis and treatment of patients with parathyroid carcinoma: an update and review. World J Surg. 1991 Nov-Dec;15(6):738-44.
[10] Bilezikian JP, Khan AA, Silverberg SJ, Fuleihan GE, Marcocci C, Minisola S, Perrier N, Sitges-Serra A, Thakker RV, Guyatt G, Mannstadt M, Potts JT, Clarke BL, Brandi ML; International Workshop on Primary Hyperparathyroidism. Evaluation and Management of Primary Hyperparathyroidism: Summary Statement and Guidelines from the Fifth International Workshop. J Bone Miner Res. 2022 Nov;37(11):2293-2314.
[11] Apaydın, T., Yavuz, D.G. Seven cases of parathyroid carcinoma and review of the literature. Hormones 20, 189–195 (2021).
[12] http://emedicine.medscape.com/article/280908 (2014-01-02); [Medscape]
[13] Minisola S, Arnold A, Belaya Z, Brandi ML, Clarke BL, Hannan FM, Hofbauer LC, Insogna KL, Lacroix A, Liberman U, Palermo A, Pepe J, Rizzoli R, Wermers R, Thakker RV. Epidemiology, Pathophysiology, and Genetics of Primary Hyperparathyroidism. J Bone Miner Res. 2022 Nov;37(11):2315-2329.