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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 .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, the distinction between benign and malignant parathyroid tumors is difficult to make.[
Risk Factors
The etiology of parathyroid carcinoma is unknown; however, an increased risk of parathyroid cancer has been associated with multiple endocrine neoplasia 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, very 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
Signs and symptoms of the hyperparathyroid state associated with parathyroid cancer that may be found at diagnosis include: [
(Refer to the PDQ summaries on Cancer Pain , Fatigue, Nutrition in Cancer Care [for weight-loss information], and Nausea and Vomiting Related to Cancer Treatment, for information on some of the above symptoms.)
Certain clinical features may help to 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 biphosphonates, gallium, or mithramycin may help control the hypercalcemia.[
Surgery is the only effective therapy for parathyroid carcinoma.[
One analysis of the literature indicated an overall 8% evidence of local recurrence after an en bloc resection compared with a 51% incidence 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 in the range of 2 to 5 years after the initial resection.[
In older studies, distant metastases were reported to occur in 25% of patients, primarily in the lungs but also in the bone and liver.[
References:
The histologic 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 provide large published series of treatment experience or permit the systematic evaluation of combination therapies.[
References:
Treatment options:[
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References:
Metastatic disease can appear shortly after the initial diagnosis and operation or for up to 20 years later.[
Treatment options:[
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
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:[
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
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 PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.
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 PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
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 reviewers for Parathyroid Cancer Treatment are:
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
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 formal evidence ranking system in developing its level-of-evidence designations.
Permission to Use This Summary
PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary]."
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: https://www.cancer.gov/types/parathyroid/hp/parathyroid-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389236]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.
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Based on the strength of the available evidence, treatment options may be described as either "standard" or "under clinical evaluation." These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
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Last Revised: 2020-07-22
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