It is rare, with documented cases of less than one thousand since its first discovery in 1904;[2][3][4][5][6][7] and much less common than parathyroid adenoma.

[12] Conditions that appear to result in an increased risk of parathyroid cancer include multiple endocrine neoplasia type 1,[13] autosomal dominant familial isolated hyperparathyroidism[13] and hyperparathyroidism-jaw tumor syndrome[1] (which also is hereditary).

[14] Some authors suggest high levels of HCG as a marker for parathyroid carcinoma in the right context.

If the surgeon suspects carcinoma based on severity or invasion of surrounding tissues by a firm parathyroid tumor, aggressive excision is performed, including the thyroid and surrounding tissues as necessary.

[citation needed] This article incorporates public domain material from Dictionary of Cancer Terms.