DNA TESTING FOR MORE SOPHISTICATED CLINICAL MANAGEMENT OF MULTIPLE ENDOCRINE NEOPLASMA TYPE 2 (MEN 2)
DNA testing to predict cancer predisposition currently plays an
important role in the clinical management of hereditary cancer.
MEN 2 syndromes are autosomal dominant inherited cancer
syndromes, and DNA testing is now useful in clinical practice of
these diseases. MEN 2 syndromes are subclassified into MEN 2A,
MEN 2B, and familial medullary thyroid carcinoma (FMTC).
MEN 2A is characterized by the development of medullary thyroid
carcinoma (MTC), pheochromocytoma and hyperparathyroidism
(HPT). MEN 2B is also associated with MTC and
pheochromocytoma but has developmental abnormalities such as
marfanoid habitus, mucosal neuromas and ganglioneuromatosis.
In FMTC, MTC is the only disease feature. Recently, the RET
proto-oncogene has been identified as the susceptibility gene for
MEN 2 (1-3). The gene encodes a receptor tyrosine kinase and its
physiological ligands are glial-cell-line-derived neurotropic factor
(GDNF) . . . [Full Text of this Article]