This is a very rare malignant cancer of the cortex of the adrenal gland. A malignant tumour is one that is capable of spreading to other parts of the body causing many problems. It can be functioning (i.e. producing hormones resulting in the different types of conditions described previously) or non-functioning. Approximately 60% of such tumours are functional producing Cushing's Syndrome,feminisation or virilisation. Non functional tumours present often very late with with huge masses which tend to be inoperable. One problem with this tumour is that it is so rare that few people have any great experience of its management. In 30 years of endocrine surgery Mr Lynn was asked to treat less than 50 cases.!
It is a very rare tumour affecting approximately 1 in 1.7 million people. It has been reported in patients of all age ranges, but occurs mainly between the ages of 40 and 50 years. It occurs approximately equally among males and females. The majority of adrenocrtical tumours appear out of the blue (sporadic).A few are associated with well defined syndromes such as multiple endocrine neoplasia type1(MEN1),the Li-Fraument syndrome(p55 mutation) or the Beckwith-Wiedemann (IGFH overexpression).