[Primary hyperparathyroidism in special situations: multiple endocrine neoplasia syndromes and parathyroid cancer]

Primary hyperparathyroidism (PHP) is a known endocrine disorder. Many years ago, the most frequent forms of clinical presentation were symptomatic renal or skeletal disease with moderate or severe hypercalcemia; however, currently, most patients have few symptoms and mild hypercalcemia. The last NIH Workshop on Asymptomatic PHP developed criteria for the management of this disease. Multiple endocrine neoplasia (MEN) types 1 and 2 are two genetic syndromes caused by different types of molecular abnormalities. PHP is the most common manifestation of MEN-1 and is the last feature to appear in MEN 2A. Parathyroid carcinoma (PC) is a rare neoplasm and an uncommon cause of parathyroid hormone (PTH)-dependent hypercalcemia. In this report, the treatment of PHP in MEN syndrome and PC are reviewed. Special attention is paid to a new class of drugs called "calcimimetics", which are powerful compounds that may be highly useful in the treatment of both conditions.