Incidence
Parathyroid adenomas represent a common endocrine problem, whereas parathyroid carcinomas are very rare tumors. With an estimated incidence of 0.015 per 100,000 population and an estimated prevalence of 0.005% in the United States, parathyroid cancer is one of the rarest of all human cancers.[
Anatomy and Histopathology
Operatively, parathyroid cancers may be distinguished from adenomas by their firm, stony-hard consistency and lobulation; adenomas tend to be soft, round, or oval in shape, and of a reddish-brown color.[
Histopathologically, as with other endocrine neoplasms, it is difficult to make the distinction between benign and malignant parathyroid tumors.[
Risk Factors
The etiology of parathyroid carcinoma is unknown. However, an increased risk of parathyroid cancer has been associated with multiple endocrine neoplasia type 1 and with autosomal dominant familial isolated hyperparathyroidism.[
Clinical Factors
Parathyroid cancer typically runs an indolent, albeit tenacious, course because the tumor has a rather low malignant potential. At initial presentation, few patients with parathyroid carcinoma have metastases either to regional lymph nodes (<5%) or distant sites (<2%).[
Parathyroid cancers are hyperfunctional unlike other endocrine tumors that become less hormonally active when malignant.[
The prevalence of bone disease is much greater in patients with parathyroid carcinoma than it is in patients with parathyroid adenoma, with 70% or fewer patients manifesting symptoms related to calcium absorption with osteoporosis and bone pain.[
Diagnosis
The following signs and symptoms of the hyperparathyroid state associated with parathyroid cancer may be found at diagnosis:[
For more information about some of these symptoms, see
Certain clinical features may help distinguish parathyroid carcinoma from parathyroid adenoma.
Parathyroid carcinoma should be suspected clinically if the patient presents with the following diagnostic features:[
Clinical Treatment and Management
The medical management of hypercalcemia, particularly in patients with unresectable disease or without measurable disease, is critical and must be the initial treatment goal in all patients with HPT. Conventional treatment with intravenous fluids, diuretics, and antiresorptive agents such as bisphosphonates, gallium, or mithramycin may help control the hypercalcemia.[
Surgery is the only effective therapy for parathyroid carcinoma.[
One analysis of the literature indicated a local recurrence rate of 8% after an en bloc resection and 51% after a standard parathyroidectomy.[
Because of the fairly indolent biology of this cancer, the management of recurrent or metastatic disease is primarily surgical. Significant palliation may result from the resection of even very small tumor deposits in the neck, lymph nodes, lungs, or liver.[
Nonsurgical forms of therapy for parathyroid carcinoma generally have poor results.[
Follow-Up and Survivorship
Approximately 40% to 60% of patients experience a postsurgical recurrence, typically within 2 to 5 years after the initial resection.[
In older studies, distant metastases were reported in 25% of patients, primarily in the lungs but also in the bone and liver.[
References:
The histological distinction between benign and malignant parathyroid tumors is difficult to make.[
An aneuploid DNA pattern is more common, and mean nuclear DNA content is greater in carcinomas than in adenomas. When present in a carcinoma, aneuploidy appears to be associated with a poorer prognosis.[
References:
Because of the low incidence of parathyroid carcinoma, an American Joint Committee on Cancer staging system has not yet been formulated and, thus, is not applicable to this malignancy. In addition, neither tumor size nor lymph node status appear to be important prognostic markers for this malignancy.[
Patients are considered to have either localized or metastatic disease.[
Localized Parathyroid Cancer
Localized parathyroid cancer is disease that involves the parathyroid gland with or without invasion of adjacent tissues.
Metastatic Parathyroid Cancer
Metastatic parathyroid cancer is disease that spreads beyond the tissues adjacent to the involved parathyroid gland(s). Parathyroid carcinoma most frequently metastasizes to regional lymph nodes and lungs, and it may involve other distant sites, such as liver, bone, pleura, pericardium, and pancreas.[
References:
The rarity of this tumor does not allow for large published series of treatment experience or permit the systematic evaluation of combination therapies.[
References:
Treatment options for localized parathyroid cancer include the following:[
Current Clinical Trials
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References:
Metastatic disease can appear shortly after the initial diagnosis and operation or for up to 20 years later.[
Treatment options for metastatic parathyroid cancer include the following:[
Current Clinical Trials
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References:
Approximately 40% to 60% of patients experience a postsurgical recurrence, typically between 2 to 5 years after the initial resection.[
Because these tumors are slow-growing, repeated resection of local recurrences and/or distant metastases can result in significant palliation.[
Treatment options for recurrent parathyroid cancer include the following:[
Current Clinical Trials
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References:
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
This summary is written and maintained by the
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of parathyroid cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewer for Parathyroid Cancer Treatment is:
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's
Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a
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The preferred citation for this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Parathyroid Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at:
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Last Revised: 2024-07-05
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