Skip to Main Content

++

Carcinoma of the parathyroid gland is a rare endocrine malignancy, accounting for only 0.005% of all cancer registrations in the US National Cancer Database (NCDB).1 Using information from the Surveillance, Epidemiology and End Results (SEER) database, the incidence in the United States is estimated to be 5.73 per 10 million population, increasing by 60% from 1988 to 2003.2 Parathyroid cancer accounts for 0.5% to 1% of cases of primary hyperparathyroidism (PHPT) in series from Western nations and 2.8 to 5% of cases in Japan.3–6 It affects males and females equally and is evenly distributed within different socioeconomic and racial groups in the NCBD.1 The mean age of diagnosis of parathyroid cancer is 47 to 56 years, and 73% of cases occur in patients older than age 45 years.1,2

++

Few risk factors have been identified for parathyroid cancer. Hyperparathyroidism–jaw tumor (HPT-JT) syndrome is a familial condition with an autosomal dominant pattern of inheritance caused by an inactivating mutation of the tumor suppressor gene HRPT2. The HRPT2 gene is located on chromosome 1q, codes for the nuclear protein parafibromin, and is thought to be involved in cell growth cycle regulation.7,8 Affected HPT-JT family members develop PHPT secondary to parathyroid adenomas, fibro-osseous tumors of the mandible and maxilla, renal hamartomas, renal stromal cell tumors, and cystic disease of the kidneys.8 Patients with HPT-JT have a markedly increased risk of parathyroid cancer, which is seen in 10% to 17% of patients.7,8 The HRPT2 gene is also involved in the pathogenesis of sporadically occurring cases of parathyroid cancer, with somatic mutation of this gene identified in 67% of parathyroid cancers and germline mutations identified in 20% of patients with parathyroid cancer without a family history of HPT-JT.9

++

Although parathyroid cancer has been reported in patients with other familial forms of hyperparathyroidism (HPT), namely multiple endocrine neoplasia syndrome types 1 and 2A and familial isolated hyperparathyroidism, there is no evidence to associate these conditions with an increased risk of this malignancy. A family history of HPT is present in only 4.7% of individuals with parathyroid cancer.6 A history of previous head and neck irradiation is present in up to 7.4% of patients with parathyroid cancer, and this has also been suggested as a risk factor for this malignancy.10

++

Parathyroid cancer has been reported in patients with chronic renal failure receiving hemodialysis.11 It has been suggested that patients with end-stage renal disease (ESRD) are at increased risk for this malignancy occurring within hyperplastic or adenomatous parathyroid glands because of chronic overstimulation. Such an association has not been proven, and if ESRD was a strong risk factor for parathyroid carcinoma, then the incidence of this malignancy would be expected to be much higher in North America than is presently seen. It is conceivable that differing pathologic criteria for the diagnosis between Japan and North America may account for ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessSurgery Full Site: One-Year Subscription

Connect to the full suite of AccessSurgery content and resources including more than 160 instructional videos, 16,000+ high-quality images, interactive board review, 20+ textbooks, and more.

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessSurgery

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.