Occurs in 1 of every 2 million people.
Accounts for less than 1% of all cases of hyperparathyroidism, and approximately .005% of all cancers.
No gender predilection.
Can be clinically indistinguishable from benign causes of hyperparathyroidism.
Most cases present with symptoms of hyperparathyroidism and include hypercalcemia, end organ damage such as nephrolithiasis, renal failure, decreased bone mineral density and/or fracture, cardiac arrhythmia, and neurocognitive dysfunction.
Incidence has increased in recent years.
Most patients have long-term survival with surgical resection, but multiple surgical procedures and systemic therapy may required for recurrent and or metastatic disease.
Five and 10 year mortality rates are 16% and 33%, respectively.
Patients often present with metastatic disease.
Some lesions smolder and recur locally.
Some lesions are aggressive locally, while others are metastatic.
Primary tumors account for most cases of primary hyperparathyroidism
85-95% of cases of hyperparathyroidism are attributable to benign adenomas.
3% of cases of hyperparathyroidism are due to parathyroid carcinomas.
Do not respond to cytotoxic chemotherapy.
Effects of hypercalcemia may be more pronounced in patients with parathyroid carcinoma than in those with benign disease, with higher calcium levels than 13mg/dL or parathyroid hormone levels more than 2 times normal.