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Parathyroid Carcinoma

  • Author: Lawrence Kim, MD; Chief Editor: Jules E Harris, MD, FACP, FRCPC  more...
 
Updated: Dec 01, 2015
 

Background

Parathyroid carcinoma is a rare malignancy of the parathyroid glands. These tumors usually secrete parathyroid hormone, thereby producing hyperparathyroidism, which is usually severe. Parathyroid carcinoma may be suspected, but it usually cannot be confirmed prior to operation. This complicates the treatment strategy.[1]

Photomicrograph of parathyroid carcinoma showing t Photomicrograph of parathyroid carcinoma showing typical fibrotic septae. Histologic diagnosis can be difficult.
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Pathophysiology

Parathyroid carcinoma causes hyperparathyroidism (for a more complete discussion see Hyperparathyroidism) in all but a few rare cases of nonfunctional parathyroid carcinoma. Hyperparathyroidism is caused by overproduction of parathyroid hormone. In most cases, hyperparathyroidism is caused by a single benign adenoma (approximately 85%). The vast majority of the remaining cases are caused by parathyroid hyperplasia or multiple adenomas. In less than 1% of cases of hyperparathyroidism, the etiology is parathyroid carcinoma.

The hyperparathyroidism seen in parathyroid carcinoma is usually severe, with high serum calcium levels, severe bone disease, and renal stones. Rarely, parathyroid cancer can be nonfunctional, that is, it may cause a tumor but not produce parathyroid hormone and the sequelae of hyperparathyroidism. Overproduction of authentic parathyroid hormone from nonparathyroid sources is extremely rare but has been reported. Modern assays for parathyroid hormone measure the intact, authentic molecule and do not detect similar molecules produced by other tumors. Therefore, an elevated parathyroid hormone level is virtually always parathyroid in origin.

Untreated, parathyroid carcinoma leads to severe symptoms of hyperparathyroidism and may cause renal stones, nephrocalcinosis, pathologic fractures, and neuropsychiatric symptoms. The diagnosis is often not made prior to parathyroidectomy. This results in inadequate tumor excision and a propensity to local recurrence. Regional lymph node and distant metastases are usually not present at initial diagnosis. Recurrences may be treated by local excision or ablative treatments such as radiofrequency ablation. Death is usually caused by medically refractory hypercalcemia and seldom tumor burden alone.

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Epidemiology

Frequency

United States

Parathyroid cancer is a rare disease, occurring with an annual incidence of approximately 1.25 cases per 10,000,000 persons.[2] It occurs in less than 1% of all cases of hyperparathyroidism.[3, 4]

International

A large European study also demonstrated the rarity of parathyroid carcinoma with an estimated incidence of 2 cases per 10,000,000 persons/year.[5]

Mortality/Morbidity

See the list below:

  • Survival has varied widely in the literature, with 5-year survival ranging from 20-85% and 10-year survival from approximately 15-80%. [6, 7, 8, 9, 10, 11, 12, 13, 2] Data from the US National Cancer Data Base showed a 5-year survival of 88.5% and a 10-year survival of 49.1%. [14]
  • A significant number of patients continue to die of recurrent disease many years after the initial treatment.
  • Permanent cure is achieved in the minority of patients.

Race

No known racial predilection exists.

Sex

Parathyroid cancer occurs equally in males and females.[14] Males fare slightly worse in prognosis.[13, 15]

Age

Parathyroid cancer usually occurs in patients older than 30 years. After that, no predominant age association has been noted.

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Contributor Information and Disclosures
Author

Lawrence Kim, MD Professor, Department of Surgery, Division of Surgical Oncology, University of North Carolina at Chapel Hill School of Medicine

Lawrence Kim, MD is a member of the following medical societies: Association for Academic Surgery, Association of VA Surgeons, International Association of Endocrine Surgeons, Society of Surgical Oncology, Society of University Surgeons, American Association of Clinical Endocrinologists, American Association of Endocrine Surgeons, American College of Surgeons, Southwestern Surgical Congress

Disclosure: Received income in an amount equal to or greater than $250 from: Affinergy, LLC.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Jules E Harris, MD, FACP, FRCPC Clinical Professor of Medicine, Section of Hematology/Oncology, University of Arizona College of Medicine, Arizona Cancer Center

Jules E Harris, MD, FACP, FRCPC is a member of the following medical societies: American Association for the Advancement of Science, American Society of Hematology, Central Society for Clinical and Translational Research, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Additional Contributors

Sanjiv S Agarwala, MD Chief of Oncology and Hematology, St Luke's Cancer Center, St Luke's Hospital and Health Network; Professor, Temple University Shool of Medicine

Sanjiv S Agarwala, MD is a member of the following medical societies: American Association for Cancer Research, American Head and Neck Society, European Society for Medical Oncology, American Society of Clinical Oncology, Eastern Cooperative Oncology Group

Disclosure: Received honoraria from BMS for speaking and teaching; Received consulting fee from Novartis for consulting; Received consulting fee from Merck for consulting.

Acknowledgements

Wendy Hu, MD Consulting Staff, Department of Hematology/Oncology and Bone Marrow Transplantation, Huntington Memorial Medical Center

Wendy Hu, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Blood and Marrow Transplantation, American Society of Hematology, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

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Photomicrograph of parathyroid carcinoma showing typical fibrotic septae. Histologic diagnosis can be difficult.
 
 
 
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