Incidence and Prevalence

The incidence figures have not actually been affected since the introduction of autoanalyzers. As PHPT is no longer rare and is frequently diagnosed biochemically, the relative incidence of carcinomas has fallen because the majority of malignant cases present with symptomatic hypercalcemia (Table 63-1). Centers that have reported a higher incidence in the past may have had a selection bias as a result of being referral centers. The current exceptions are the Japanese centers that report a 10-fold higher incidence rate,6 for which epidemiologic factors may be responsible as well as infrequent screening for hypercalcemia. Familial segregation of parathyroid carcinoma is described in isolated familial hyperparathyroidism (HPT) and in association with multiple endocrine neoplasia (MEN) type l.7"12 In two such independent families, no MEN 1 genetic deletions were found.1112 The hyperparathyroidism-jaw tumor syndrome (HPT-JT), an autosomal dominant disorder, encompasses fibro-osseous lesions of the mandible and maxilla including uniglandular benign and malignant parathyroid neoplasms.

Previous radiation to the neck region associated with parathyroid carcinoma has been reported, although the association of radiation-induced benign tumor formation is more common.13 Concomitant benign and malignant parathyroid tumors can occur.14 Gender and age have not proved to have predictive value because parathyroid carcinoma equally affects both genders and the peak age of diagnosis in the fifth decade of life is only slightly younger than that for patients with benign HPT.41517

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