Parathyroid carcinoma

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.

Chronic kidney disease in previous neck or radiation have been associated with an increased incidence.
May occur as part  of a familial syndrome.

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.

Can metastasized to lung, bone, and liver.

Do not respond to cytotoxic chemotherapy.

The use of immunohistochemical panel aids in diagnosis: parafibromin, galectin-3, PGP9.5 and Ki-67.

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.

Cinacalcet can reduce PTH secretion ACL calcium levels and is indicated for the treatment of hypercalcemia in patients with parathyroid cancer.

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