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Parathyroid cancer
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Parathyroid cancer

Parathyroid cancer is a rare malignancy of the parathyroid glands that often causes dangerous elevations in blood calcium levels.

Overview

Parathyroid cancer is a rare condition affecting one of the four parathyroid glands in the neck. These glands secrete parathyroid hormone, which regulates calcium release into the blood. The disease typically affects individuals in their forties or fifties.

Symptoms

Cases are categorized by whether they produce excess hormones:

  • Hormonally functional (90% of cases): Characterized by high levels of parathyroid hormone (hyperparathyroidism) and calcium (hypercalcemia). This can lead to a hypercalcemic crisis, neurological issues, kidney stones, bone pain, and digestive problems.
  • Hormonally nonfunctional (10% of cases): Calcium and hormone levels remain normal. Symptoms are caused by the tumor obstructing nearby neck structures, leading to difficulty swallowing, hoarseness, or shortness of breath.

Causes & Risk Factors

Cancers arise from somatic mutations acquired during a person's lifetime that cause cells to divide uncontrollably. In rare instances, germline mutations inherited from a parent increase risk.

The CDC73 gene is implicated in up to 70 percent of cases. It produces a protein called parafibromin, which acts as a tumor suppressor. When both copies of the CDC73 gene are mutated, cells grow unchecked. Individuals with CDC73 mutations face a higher risk of metastasis and cancer recurrence.

Other risk factors include:

  • Hyperparathyroidism-jaw tumor syndrome
  • Familial isolated hyperparathyroidism
  • Multiple endocrine neoplasia
  • History of chronic kidney failure, thyroid cancer, or neck radiation

Inheritance

Most cases are not inherited. When a germline mutation is present, it is typically inherited in an autosomal dominant pattern. Inheriting the mutation increases the likelihood of developing a tumor, but does not guarantee that cancer will develop.

Frequently Asked Questions

Parathyroid cancer can be curable if detected early and completely removed. The standard surgical procedure involves removing the affected parathyroid gland, the adjacent lobe of the thyroid gland, and any nearby lymph nodes to ensure all cancer cells are excised. Because the remaining parathyroid glands may not produce enough hormone after surgery, patients often require lifelong calcium and vitamin D supplementation to maintain normal blood calcium levels.
Untreated parathyroid cancer can lead to hypercalcemic crisis, a medical emergency caused by dangerously high levels of calcium in the blood. This condition can cause severe dehydration, confusion, coma, and cardiac arrhythmias. Additionally, the excess calcium can lead to the formation of kidney stones and progressive kidney damage, while the bones may become weak and painful due to the constant release of calcium into the bloodstream.
Parathyroid cancer is often difficult to diagnose before surgery because it looks similar to benign adenomas on imaging and blood tests. The definitive diagnosis is usually made after surgical removal when a pathologist examines the tissue under a microscope. Signs that suggest cancer rather than a benign tumor include the tumor invading surrounding tissues, such as the thyroid or muscles, and the presence of cancer cells in the lymph nodes. Blood calcium levels may be significantly higher in cancer cases, but this is not a definitive differentiator on its own.
The CDC73 gene mutation is a tumor suppressor gene, and inheriting a defective copy increases the risk of developing parathyroid cancer, as well as other conditions like fibrous dysplasia and jaw tumors. Individuals with this germline mutation have a higher likelihood of the cancer recurring or spreading to other parts of the body (metastasis) compared to those with sporadic cases. Regular monitoring of calcium levels and neck imaging is essential for these patients to detect recurrence early.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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