The aetiology of adrenocortical carcinomas is generally unknown. There are rare reports of familial incidence. About 10% of adrenal medullary tumours are familial, although in future more may be identified as hereditary. These are usually associated with multiple endocrine neoplasia (MEN) syndrome II and, occasionally, von Hippel-Lindau disease. The RET proto-oncogene was the first to be associated with MEN IIA and familial medullary thyroid cancer and, subsequently, MEN IIB. The ability to identify this oncogene is of considerable importance in prophylaxis of the disease (discussed later). In over 80% of cases exons 10 and 11 are affected, which is useful for screening purposes.

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